acute b lymphoblastic leukemia

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Acute B Lymphoblastic Leukemia (B-ALL)

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Definition

Acute lymphoblastic leukemia (ALL) is a clonal expansion of the lymphoid blasts in bone

marrow, blood or other tissues. ALL can be either T or B lineage (see T ALL) . Pre-B ALL is the

proliferation of the blasts of the B lineage.

Sample Cases

Clickhere for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.

Epidemiology

The incidence of acute leukemia is approximately 4 cases per 100,000 per year. 30% of these are

ALL. ALL is generally seen in children 75% of cases are usually in children under 6 years of

age. There are approximately 3200 cases of ALL in the United States per year; 80-85% of theseare precursor B-ALL, the others areprecursor T-ALL. The overall cure rate in children is 85%,

and about 50% of adults have long-term disease-free survival.

Possible causes

Similar to AML, possible factors that have been associated with ALL are viruses, radiation,

cytotoxic chemotherapy and benzene exposure. Exposure to the atomic bomb of WWII increased

Case Name(click on case name to

open)

Comments Size

ALL-1

pre-B All

5 Mb

ALL-2

pre-B ALL with myeloid Antigens

7 Mb

ALL-3

pre B ALL

2.86 Mb

case 22 pre B ALLThis case was kindly provided by the ASCP Press. It is part ofFlow Cytometry in Clinical Diagnosisby

John Carey, Phil McCoy and David Keren.

7.54 MB

ALL case pre B ALL case submitted by UTMC. Compare withNormal PB. 2.8 MB
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the incidence of all leukemias including ALL. There is also evidence that may suggest a genetic

factor in some cases.

Morphology

Lymphoblasts (T or B) vary in size from similar to that of a mature lymphocyte to the size of aneutrophil. These blasts have scant to moderate basophillic cytoplasm with occassional

azurophillic granules (T ALL). The nuclear chromatin can be fine to clumped, with occasionally

prominant nucleoli.

Typical morphology of B lymphoblasts

WHO classification

The recent WHO International panel on ALL recommends that the FAB morphologic

classification (L1, L2, L3) be abandoned, since this classification has no clinical or prognostic

relevance. It instead advocates the use of the immunophenotypic classification. There are 2 mainimmunologic types: pre-B cell andpre-T cell. The mature B-cell ALL (L3) is now classified as

Burkitt leukemia/lymphoma. Subtyping helps determine the prognosis and most appropriate

treatment in treating ALL.

Immunophenotyping

Blasts in pre B ALL can be initially identified using a SSC vs CD45 plot. These blasts have low

SSC (many times smaller than normal lymphocytes) and dim to negative CD45. This differs from

blasts in AML where the SSC is generally higher.
http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Mature_B-Cell_Neoplasms_(general)/Burkitt's_Lymphoma_(BL)http://clinicalflow.com/Cases/Case_List/Acute_Myelogenous_Leukemia_(AML)_not_otherwise_categorized_-_General_Informationhttp://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Acute_Lymphoblastic_Leukemia_(ALL)_-_General_Information/Acute_T_Lymphoblastic_Leukemia%2F%2FLymphoma_(T_ALL)http://clinicalflow.com/Cases/Case_List/Mature_B-Cell_Neoplasms_(general)/Burkitt's_Lymphoma_(BL)http://clinicalflow.com/Cases/Case_List/Acute_Myelogenous_Leukemia_(AML)_not_otherwise_categorized_-_General_Information


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Example peripheral blood with CD45 negative Blymphoblasts and characteristically small (low SSC)

Example bone marrow with CD45 dim B lymphobla

Once the blasts are identified and gated, the following markers are useful in classification of pre

B ALL. Included are marking prevalence:

Marker Prevalence

CD10 89%

CD13 5%CD19 100%

CD20 24%

CD22 69%

CD33 31%

CD34 76%

CD45 (bright) 2%

CD45 (moderate) 33%

CD45 (dim) 36%

CD45 (negative) 29%

CD56 36%

CD79a 88%

CD117 0%

cytoplasmic IgM 22%

HLA Dr 98%


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TdT 91%

Example Dot plots in pre B ALL

pre B ALL cellscharacteristically coexpress

CD10 and CD19. In addition,

CD20 and CD23 (other B cellmarkers) are not expressed.

Expression of CD34 and TdTindicates immaturity and is

characteristic in pre B ALL.

cytoplasmic expression ofCD79 confirms B cell lineage.

CD38 is an expression ofimmaturity. The ploidy as seen

by the DNA Histogram,

indicates a hypodiploidy(DI=0.53) indicating poor

prognosis.

The phenotype of the blasts is an idependent prognostic parameter. B-ALL is subdivided into:

early pre B-ALL: TdT+, CD19+, CD10-

common ALL: CD19+, CD10+/CALLA+

pre B ALL: CD10+/-, CD19+, HLA Dr+, cytoplasmic IgM+


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mature B ALL: CD10+, CD19+, CD20+, CD22+, surface IgM+

Other relevant tests

Genetics: Some cytogenetic subtypes have a worse prognosis than others. These include:

A translocation between chromosomes 9 and 22, known as the Philadelphia chromosome,

occurs in about 20% of adult and 5% in pediatric cases of ALL.

A translocation between chromosomes 4 and 11 occurs in about 4% of cases and is most

common in infants under 12 months.

Not all translocations of chromosomes carry a poorer prognosis. Some translocations arerelatively favorable. For example, Hyperdiploidy (>50 chromosomes) is a good

prognostic factor.

Flow Diagnosis

To diagnose B ALL: CD45 downregulated, CD19+, TdT+, CD10+, surface light chainsnegative.

References

1. World Health Organization Classification of Tumors. Tumors of Haematopoetic and Lymphoid

Tissue. Jaffe, E., Harriss, N., Stein, H., Vardiman, J. IARC Press 2001

2. Flow Cytometry in Neoplastic Hematology-Morphologic-Immunophenotypic Correlation.Gorczyca W., Informa Healthcare 2007

3.2006 Bethesda International Consensus Recommendations on Immunophenotypic Analysis ofHematolymphoid Neoplasia by Flow Cytometry. Cytometry Part B, 2007

4. Clinical Flow Cytometric Analysis of Hematolymphoid Cells; Approved Guideline - SecondEdition H43-A2 Clinical and Laboratory Standards Institute (CLSI) 2007

Contributors to this page

Teri (34 edits)

David Novo (12 edits)

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Tanya Tolmachoff(6 edits) Tanya (1 edits)

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ALL UTMC (1).fey

Pre-B cell ALL submitted by UTMC.2.77 MB23:04, 10 Apr 2009 Tanya TolmachoffActions

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pre-B ALL4.97 MB19:30, 4 Apr 2008 David Novo Actions

B-ALL2.feypre-B ALL with myeloid Antigens

7.08 MB19:30, 4 Apr 2008 David Novo Actions

B-ALL3.fey

pre-B ALL2.86 MB19:30, 4 Apr 2008 David Novo Actions

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Acute lymphoblastic leukemia
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From Wikipedia, the free encyclopedia

Acute lymphoblastic leukemia (ALL)Classification and external resources

ICD-10 C91.0

ICD-9 204.0

ICD-O: M9835/3

DiseasesDB 195

eMedicine med/3146ped/2587

MeSH D054198

Acute lymphoblastic leukemias (ALL), is a form ofleukemia, orcancer of the white blood

cells characterized by excess lymphoblasts.

Malignant, immature white blood cellscontinuously multiply and are overproduced in thebonemarrow. ALL causes damage and death by crowding out normal cells in the bone marrow, and

by spreading (infiltrating) to other organs. ALL is most common in childhood with a peak

incidence at 25 years of age, and another peak in old age. The overall cure rate in children isabout 80%, and about 45%-60% of adults have long-term disease-free survival. [1]

Acute refers to the relatively short time course of the disease (being fatal in as little as a few

weeks if left untreated) to differentiate it from the very different disease ofChronic Lymphocytic

Leukemia which has a potential time course of many years. It is interchangeably referred to asLymphocytic or Lymphoblastic. This refers to the cells that are involved, which if they were

normal would be referred to aslymphocytes but are seen in this disease in a relatively immature(also termed 'blast') state.

Symptoms The signs and symptoms of ALL are variable but follow from bone marrowreplacement and/or organ infiltration.

Generalized weakness and fatigue

Anemia
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Frequent or unexplained [fever] and [infection]

Weight loss and/or loss of appetite

Excessive and unexplained bruising

Bone pain, joint pain (caused by the spread of "blast" cells to the surface of the bone or

into the joint from the marrow cavity)

Breathlessness Enlarged lymph nodes,liver and/or spleen

Pitting edema (swelling) in the lower limbs and/or abdomen

Petechia, which are tiny red spots or lines in the skin due to lowplatelet levels

The signs and symptoms of ALL result from the lack of normal and healthy blood cells becausethey are crowded out by malignant and immature leukocytes (white blood cells). Therefore,

people with ALL experience symptoms from malfunctioning of their erythrocytes (red blood

cells), leukocytes, and platelets. Laboratory tests which might show abnormalities include bloodcount tests, renal function tests, electrolyte tests and liver enzyme tests.

Diagnosis

Diagnosing ALL begins with a medical history,physical examination,complete blood count, and

blood smears. Because the symptoms are so general, many other diseases with similar symptomsmust be excluded. Typically, the higher the white blood cell count, the worse the prognosis.[2]

Blast cells are seen onblood smearin majority of cases (blast cells are precursors (stem cells) to

all immune cell lines). Abone marrow biopsy is conclusive proof of ALL.[3] A lumbar puncture

(also known as a spinal tap) will tell if the spinal column andbrainhas been invaded.

Pathological examination, cytogenetics(particularly the presence ofPhiladelphia chromosome)andimmunophenotyping, establish whether the Myeloblastic (neutrophils, eosinophils or

basophils) or Lymphoblastic (B lymphocytes orT lymphocytes) cells are the problem. RNAtesting can establish how aggressive the disease is; different mutations have been associated with

shorter or longer survival. Immunohistochemical testing may reveal TdT or CALLA antigens on

the surface of leukemic cells. TdT is a protein expressed early in the development of pre-T and

pre-B cells while CALLA is an antigen found in 80% of ALL cases and also in the "blast crisis"ofCML.

Medical imaging (such as ultrasoundorCT scanning) can find invasion of otherorgans

commonly the lung, liver, spleen, lymph nodes, brain, kidneys and reproductive organs.[4]

[edit] Pathophysiology

In general, cancer is caused by damage to DNA that leads to uncontrolled cellular growth andspread throughout the body, either by increasing chemical signals that cause growth, or

interrupting chemical signals that control growth. Damage can be caused through the formation

offusion genes, as well as the dysregulation of aproto-oncogene via juxtaposition of it to the
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promoter of another gene, e.g. theT-cell receptorgene. This damage may be caused by

environmental factors such as chemicals, drugs or radiation.

ALL is associated with exposure toradiationand chemicals in animals and humans. Theassociation of radiation and leukemia in humans has been clearly established in studies of

victims of the Chernobyl nuclear reactor andatom bombs in Hiroshima andNagasaki. Inanimals, exposure tobenzeneand other chemicals can cause leukemia. Epidemiological studies

have associated leukemia with workplace exposure to chemicals, but these studies are not asconclusive. Some evidence suggests that secondary leukemia can develop in individuals who are

treated for other cancers with radiation and chemotherapy as a result of that treatment. [5]

[edit] Cytogenetics

Cytogenetic translocations associated with specific molecular genetic abnormalities in ALL

Cytogenetic

translocation

Molecular genetic

abnormality%

cryptic t(12;21) TEL-AML1 fusion[6]25.4%[

7]

t(1;19)(q23;p13) E2A-PBX (PBX1) fusion[8] 4.8%[7]

t(9;22)(q34;q11) BCR-ABL fusion(P185)[9] 1.6%[7]

t(4;11)(q21;q23) MLL-AF4 fusion[10] 1.6%[7]

t(8;14)(q24;q32) IGH-MYC fusion[11]

t(11;14)(p13;q11) TCR-RBTN2 fusion [12]

12:21 is the most common translocation and portends a good prognosis. 4:11 is the most

common in children under 12 months and portends a poor prognosis.

[edit] Prognosis

The survival rate has improved from zero four decades ago, to 20-75 percent currently, largelydue to clinical trials on new chemotherapeutic agents and improvements in stem cell

transplantation(SCT) technology.

Five-year survival rates evaluate older, not current, treatments. New drugs, and matching

treatment to the genetic characteristics of the blast cells, may improve those rates. The prognosisfor ALL differs between individuals depending on a variety of factors:

Sex: females tend to fare better than males.
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Ethnicity: Caucasians are more likely to develop acute leukemia than African-Americans, Asians and Hispanics and tend to have a better prognosis thannon-Caucasians.

Age at diagnosis: children between 110 years of age are most likely todevelop ALL and to be cured of it. Cases in older patients are more likely toresult from chromosomal abnormalities (e.g. the Philadelphia chromosome)

that make treatment more difficult and prognoses poorer. White blood cell count at diagnosis of less than 50,000/l Cancer spread into the Central nervous system (brain or spinal cord) has

worse outcomes. Morphological, immunological, and genetic subtypes Patient's response to initial treatment Genetic disorders such as Down's Syndrome

Cytogenetics, the study of characteristic large changes in the chromosomes ofcancer cells, is animportant predictor of outcome.[13]

Some cytogenetic subtypes have a worse prognosis than others. These include:

A translocation between chromosomes 9 and 22, known as the Philadelphiachromosome, occurs in about 20% of adult and 5% in pediatric cases of ALL.

A translocation between chromosomes 4 and 11 occurs in about 4% of casesand is most common in infants under 12 months.

Not all translocations of chromosomes carry a poorer prognosis. Sometranslocations are relatively favorable. For example, Hyperdiploidy (>50chromosomes) is a good prognostic factor.

Genome-wide copy number changes can be assessed by conventionalcytogenetics or virtual karyotyping. SNP array virtual karyotyping can detectcopy number changes and LOH status, while arrayCGH can detect only copy

number changes. Copy neutral LOH (acquired uniparental disomy) has beenreported at key loci in ALL, such as CDKN2A gene, which have prognosticsignificance.[14][15][16] SNP array virtual karyotyping can readily detect copyneutral LOH. Array CGH, FISH, and conventional cytogenetics cannot detectcopy neutral LOH.

Cytogenetic changeRisk

category

Philadelphia chromosomePoor

prognosis

t(4;11)(q21;q23)Poorprognosis

t(8;14)(q24.1;q32)Poor

prognosis
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Complex karyotype (more than four

abnormalities)

Poor

prognosis

Low hypodiploidy or near triploidyPoor

prognosis

High hyperdiploidy (specifically, trisomy

4, 10, 17)

Good

prognosis

del(9p)Good

prognosis

Correlation of prognosis with bone marrow cytogenetic finding in acute lymphoblastic

leukemia

Prognosi

sCytogenetic findings

Favorable Hyperdiploidy > 50 ; t (12;21)

Intermedia

te

Hyperdiploidy 47 -50; Normal(diploidy); del (6q);

Rearrangements of 8q24

Unfavorabl

e

Hypodiploidy-near haploidy; Near tetraploidy; del (17p); t

(9;22); t (11q23)

Unclassified ALL is considered to have an intermediate prognosis.[17]

[edit] Classification

As ALL is not a solid tumour, the TNM notation as used in solid cancers is of little use.

[edit] The FAB classification

Subtyping of the various forms of ALL used to be done according to the French-American-

British (FAB) classification,[18] which was used for all acute leukemias (including acute

myelogenous leukemia, AML).

ALL-L1: small uniform cells ALL-L2: large varied cells ALL-L3: large varied cells with vacuoles (bubble-like features)

Each subtype is then further classified by determining the surface markers of the abnormallymphocytes, called immunophenotyping. There are 2 main immunologic types: pre-B cell and
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pre-T cell. The mature B-cell ALL (L3) is now classified as Burkitt's lymphoma/leukemia.

Subtyping helps determine the prognosis and most appropriate treatment in treating ALL.

[edit] WHO proposed classification of acute lymphoblastic

leukemia

The recent WHO International panel on ALL recommends that the FAB classification be

abandoned, since the morphological classification has no clinical or prognostic relevance. It

instead advocates the use of the immunophenotypic classification mentioned below.

1- Acute lymphoblastic leukemia/lymphoma Synonyms:Former Fab L1/L2

i. Precursor B acute lymphoblastic leukemia/lymphoma. Cytogeneticsubtypes:[19]

o t(12;21)(p12,q22) TEL/AML-1o t(1;19)(q23;p13) PBX/E2A

o t(9;22)(q34;q11) ABL/BCRo T(V,11)(V;q23) V/MLL

ii. Precursor T acute lymphoblastic leukemia/lymphoma

2- Burkitt's leukemia/lymphoma Synonyms:Former FAB L3

3- Biphenotypic acute leukemia

[edit] Variant Features of ALL

1- Acute lymphoblastic leukemia with cytoplasmic granules 2- Aplastic presentation of ALL 3- Acute lymphoblastic leukemia with eosinophilia 4- Relapse of lymphoblastic leukemia 5- Secondary ALL

[edit] Immunophenotyping in the diagnosis and classification

of ALL

The use of a TdT assay and a panel of monoclonal antibodies (MoAbs) to T cell and B cellassociated antigens will identify almost all cases of ALL.

Immunophenotypic categories of acute lymphoblastic leukemia (ALL)

TypesFAB

Class

Td

t

T cell associate

antigen

B cell associate

antigen

c

Ig

s

Ig
http://en.wikipedia.org/wiki/Burkitt's_lymphomahttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=7http://en.wikipedia.org/wiki/Precursor_B_acute_lymphoblastic_leukemia/lymphomahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-urlAdvances_in_Acute_Lymphoblastic_Leukemia_.7C_Clinical_Laboratory_Science_.7C_Find_Articles_at_BNET.com-18http://en.wikipedia.org/wiki/Precursor_T_acute_lymphoblastic_leukemia/lymphomahttp://en.wikipedia.org/wiki/Burkitt's_leukemiahttp://en.wikipedia.org/wiki/Biphenotypic_acute_leukemiahttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=8http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=9http://en.wikipedia.org/wiki/Burkitt's_lymphomahttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=7http://en.wikipedia.org/wiki/Precursor_B_acute_lymphoblastic_leukemia/lymphomahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-urlAdvances_in_Acute_Lymphoblastic_Leukemia_.7C_Clinical_Laboratory_Science_.7C_Find_Articles_at_BNET.com-18http://en.wikipedia.org/wiki/Precursor_T_acute_lymphoblastic_leukemia/lymphomahttp://en.wikipedia.org/wiki/Burkitt's_leukemiahttp://en.wikipedia.org/wiki/Biphenotypic_acute_leukemiahttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=8http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=9


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Precursor

BL1,L2 + - +

-/

+-

Precursor

TL1,L2 + + - - -

B-cell L3 - - + - +

[edit] Treatment

The earlier acute lymphocytic leukemia is detected, the more effective the treatment. The aim isto induce a lasting remission, defined as the absence of detectable cancer cells in the body

(usually less than 5% blast cells on the bone marrow).

Treatment for acute leukemia can includechemotherapy,steroids, radiation therapy, intensivecombined treatments (includingbone marroworstem celltransplants), and growth factors.[20]

[edit] Chemotherapy

Chemotherapy is the initial treatment of choice. Most ALL patients will receive a combination ofdifferent treatments. There are no surgical options, due to the body-wide distribution of the

malignant cells. In general, cytotoxic chemotherapy for ALL combines multiple antileukemic

drugs in various combinations. Chemotherapy for ALL consists of three phases: remission

induction, intensification, and maintenance therapy.

Phase Description Agents

Remission induction The aim of remission induction is

to rapidly kill most tumor cells

and get the patient into

remission. This is defined as the

presence of less than 5%

leukemic blasts in the bone

marrow, normal blood cells and

absence of tumor cells from

blood, and absence of other signs

and symptoms of the disease.

CNS prophylaxis should beginduring this phase of treatment

and continue during the

consolidation/intensification

period. The rationale is based on

the presence of CNS involvement

in 10%-40% of adult patients at

Combination of

Prednisolone or

dexamethasone,

vincristine, asparaginase

(better tolerance in

pediatric patients), and

daunorubicin (used in

Adult ALL) is used to

induce remission.
http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=10http://en.wikipedia.org/wiki/Remission_(medicine)http://en.wikipedia.org/wiki/Remission_(medicine)http://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Steroidhttp://en.wikipedia.org/wiki/Steroidhttp://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/wiki/Stem_cellhttp://en.wikipedia.org/wiki/Stem_cellhttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-19http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=11http://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Malignant_cellshttp://en.wikipedia.org/wiki/Prednisolonehttp://en.wikipedia.org/wiki/Dexamethasonehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Asparaginasehttp://en.wikipedia.org/wiki/Daunorubicinhttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=10http://en.wikipedia.org/wiki/Remission_(medicine)http://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Steroidhttp://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/wiki/Stem_cellhttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-19http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=11http://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Malignant_cellshttp://en.wikipedia.org/wiki/Prednisolonehttp://en.wikipedia.org/wiki/Dexamethasonehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Asparaginasehttp://en.wikipedia.org/wiki/Daunorubicin


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diagnosis.

Consolidation/Intensific

ation

Intensification uses high doses of

intravenous multidrug

chemotherapy to further reducetumor burden. Since ALL cells

sometimes penetrate the Central

Nervous System (CNS), most

protocols include delivery of

chemotherapy into the CNS fluid

(termed intrathecal

chemotherapy). Some centers

deliver the drug through

Ommaya reservoir (a device

surgically placed under the scalpand used to deliver drugs to the

CNS fluid and to extract CNS fluid

for various tests). Other centers

would perform multiple lumbar

punctures as needed for testing

and treatment delivery.

Typical intensification

protocols use vincristine,

cyclophosphamide,

cytarabine, daunorubicin,etoposide, thioguanine or

mercaptopurine given as

blocks in different

combinations. For CNS

protection, intrathecal

methotrexate or

cytarabine is usually used

combined with or without

cranio-spinal irradiation

(the use of radiation

therapy to the head and

spine). Central nervous

system relapse is treated

with intrathecal

administration of

hydrocortisone,

methotrexate, and

cytarabine.

Maintenance therapy

The aim of maintenance therapy

is to kill any residual cell that was

not killed by remission induction,

and intensification regimens.

Although such cells are few, they

will cause relapse if not

eradicated.

For this purpose, daily oral

mercaptopurine, once

weekly oral methotrexate,

once monthly 5-day

course of intravenous

vincristine and oral

corticosteroids are usually

used. The length of

maintenance therapy is 3

years for boys, 2 years for

girls and adults.[21]

As the chemotherapy regimens can be intensive and protracted (often about 2 years in case of theGMALL UKALL, HyperCVAD or CALGB protocols; for ALL about 3 years, 2 months for

males on COG protocols; 2 years, 2 months for females- longer for males as testicles are a

potential reservoir), many patients have anintravenous catheter inserted into a large vein (termed

acentral venous catheteror a Hickman line), or a Portacath, a cone-shaped port with a siliconenose that is surgically planted under the skin, usually near the collar bone, and the most effective

product available, due to low infection risks and the long-term viability of a portacath.
http://en.wikipedia.org/wiki/CNShttp://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/wiki/Ommaya_reservoirhttp://en.wikipedia.org/wiki/Cyclophosphamidehttp://en.wikipedia.org/wiki/Cytarabinehttp://en.wikipedia.org/wiki/Etoposidehttp://en.wikipedia.org/wiki/Thioguaninehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Hydrocortisonehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Methotrexatehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-20http://en.wikipedia.org/wiki/Chemotherapy_regimenshttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Hickman_linehttp://en.wikipedia.org/wiki/Portacathhttp://en.wikipedia.org/wiki/CNShttp://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/w/index.php?title=Intrathecal_chemotherapy&action=edit&redlink=1http://en.wikipedia.org/wiki/Ommaya_reservoirhttp://en.wikipedia.org/wiki/Cyclophosphamidehttp://en.wikipedia.org/wiki/Cytarabinehttp://en.wikipedia.org/wiki/Etoposidehttp://en.wikipedia.org/wiki/Thioguaninehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Hydrocortisonehttp://en.wikipedia.org/wiki/Mercaptopurinehttp://en.wikipedia.org/wiki/Methotrexatehttp://en.wikipedia.org/wiki/Vincristinehttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-20http://en.wikipedia.org/wiki/Chemotherapy_regimenshttp://en.wikipedia.org/wiki/Intravenoushttp://en.wikipedia.org/wiki/Central_venous_catheterhttp://en.wikipedia.org/wiki/Hickman_linehttp://en.wikipedia.org/wiki/Portacath


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[edit] Radiation therapy

Radiation therapy (or radiotherapy) is used on painful bony areas, in high disease burdens, or aspart of the preparations for abone marrow transplant (total body irradiation). Radiation in the

form of whole brain radiation is also used for central nervous system prophylaxis, to prevent

recurrence of leukemia in the brain. Whole brain prophylaxis radiation used to be a commonmethod in treatment of childrens ALL. Recent studies showed that CNS chemotherapy provided

results as favorable but with less developmental side effects. As a result, the use of whole brain

radiation has been more limited. Most specialists in adult leukemia have abandoned the use o

[edit] Epidemiology

In the US, the incidence of ALL is roughly 6000 new cases per year (as of 2009),[22]orapproximately 1 in 50,000. ALL accounts for approximately 70 percent of all childhood

leukemia cases (ages 0 to 19 years), making it the most common type of childhood cancer.[22] It

has a peak incident rate of 25 years old, decreasing in incidence with increasing age before

increasing again at around 50 years old. ALL is slightly more common in males than females.There is an increased incidence in people with Down Syndrome, Fanconi anemia,Bloom

syndrome,Ataxia telangiectasia,X-linked agammaglobulinemiaandSevere combined

immunodeficiency.

[edit] Additional images

acute lymphoblastic leukemia (ALL), peripheral blood of a child, Pappenheim stain,magnification x100

bone marrow smear (large magnification) from a patient with acute lymphoblastic

leukemia
http://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=12http://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=13http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Down_Syndromehttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=14http://en.wikipedia.org/wiki/File:ALL-KM-2.jpghttp://en.wikipedia.org/wiki/File:ALL_-_Peripherial_Blood_-_Diagnosis_-_01.jpghttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=12http://en.wikipedia.org/wiki/Radiation_therapyhttp://en.wikipedia.org/wiki/Bone_marrow_transplanthttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=13http://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Childhood_leukemiahttp://en.wikipedia.org/wiki/Acute_lymphoblastic_leukemia#cite_note-acs-21http://en.wikipedia.org/wiki/Down_Syndromehttp://en.wikipedia.org/wiki/Fanconi_anemiahttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Bloom_syndromehttp://en.wikipedia.org/wiki/Ataxia_telangiectasiahttp://en.wikipedia.org/wiki/X-linked_agammaglobulinemiahttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/wiki/Severe_combined_immunodeficiencyhttp://en.wikipedia.org/w/index.php?title=Acute_lymphoblastic_leukemia&action=edit&section=14


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bone marrow smear from a patient with acute lymphoblastic leukemia

LeukemiaNote: This section has health/medical information. It was not written by a health care

professional. The medical references are:

Childhood Leukemias, edited by Ching-Hon Pui, 1999, Cambridge UniversityPress

NCI web site sections on the different types of childhood leukemias, accessed2007

Childhood Leukemia: A Guide for Families, Friends, and Caregivers, 4th ed.,by Nancy Keene, 2010.

On this site: Warning Signs of Childhood Cancer: Leukemia

Leukemia is a cancer of the bone marrow, the spongy center of the bones that makes blood cells.In leukemia, abnormal white blood cells divide out of control and crowd out the normal cells in

the bloodstream. The abnormal white blood cells are not mature, and therefore cannot carry out

their infection-fighting function in the blood. These cells crowd out healthy white blood cells, as
http://www.cancer.gov/http://www.ped-onc.org/diseases/SOCC.html#leukemiahttp://en.wikipedia.org/wiki/File:ALL-KM-3.jpghttp://www.cancer.gov/http://www.ped-onc.org/diseases/SOCC.html#leukemia


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well as the red blood cells which carry oxygen to the body and the platelets which cause the

blood to clot.

What are the different types of childhood leukemia?

The most common type of leukemia in children is acute lymphocytic (or lymphoblastic)leukemia orALL, which is further characterized as pre-B, B, or T-cell ALL. Childhood acute

myeloid leukemia orAML is less common. "Acute" means that the diseases progress rapidly.

The chronic forms of these leukemias, CLL and CML respectively, are seen almost solely in

adults. In general, acute leukemias are most prevalent in children and are therefore often referredto as "childhood leukemias".

ALL: acute lymphocytic leukemia (pre-B, B, or T-cell). ALL NCI PDQ AML: acute myeloid leukemia AML NCI PDQ

About 5% of childhood leukemias are distinct types of chronic myeloid leukemias. Juvenile

myelomonocytic leukemia (JMML,NCI PDQ) occurs primarily in children aged 2 or under.Acute promyelocytic leukemia (APL,NCI PDQ) is a distinct subtype of AML. A good startingpoint for research into these and other less common childhood leukemias is on the cancer.gov

myeloid leukemias page.

A rare type of leukemia in children is Anaplastic Large Cell Lymphoblastic Leukemia, or

ALCL. Follow this link for more information on pediatric ALCL.

Types of white blood cells

White blood cells - the blood cells that grow out of control in leukemia - are the cells that fight

infection. Blood contains three types of cells:

red blood cells (RBCs, or erythrocytes): these cells carry oxygen to all partsof your body and give the blood its red color

platelets (thrombocytes): these cells cause your blood to clot when youbleed

white blood cells (WBCs, or leukocytes): these cells defend your body frominfections

All blood cells originate in the bone marrow. In fact, they all develop from one special type of

cell, called a stem cell.

White blood cells come in several types, including:

granulocytes: fight bacteria by surrounding them and "eating" them. monocytes: fight germs, but aren't as specific as granulocytes. B-lymphocytes: these cells attach antibodies on germs (or anything they

don't think belongs) with antibodies, which in turn signal other WBCs to getthe tagged germ.
http://www.cancer.gov/cancertopics/pdq/treatment/childALL/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page10http://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page7http://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.ped-onc.org/diseases/ALCL.htmlhttp://www.cancer.gov/cancertopics/pdq/treatment/childALL/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page10http://www.cancer.gov/cancertopics/pdq/treatment/childAML/HealthProfessional/page7http://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.cancer.gov/cancertopics/pdq/treatment/childAML/healthprofessionalhttp://www.ped-onc.org/diseases/ALCL.html


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T-lymphocytes: these cells signal orders to other WBCs to come to a germ,and they make those other WBCs stay at the battle sight.

In acute lymphocytic leukemias, the B- or T-lymphocytes are growing out of control. In acutemyelogenous leukemias, the granulocytes are growing out of control.

lymphocytic (ALL): uncontrolled growth of B- or T-lymphocytes myelogenous (AML) (granulocytic): uncontrolled growth of granulocytes

In all of the leukemias, immature white cells crowd out the good cells. Since they crowd out thered blood cells, a person with leukemia is anemic, without enough red blood cells to carry the

necessary oxygen or energy to the body. That's why fatigue is a sign of leukemia. The leukemia

cells also crowd out the platelets, so if a person with leukemia is cut, the bleeding does not stopas readily. They also bruise easier. Since the blasts are immature, non-functioning infection

fighting cells, a person with leukemia is easily susceptible to infection.

If you are interested in more information on blood cells, follow the links below for in-depth,

technical information.

University of Virginia's site on blood cells - a good tutorial from the Universityof Virginia.

University of Washington REAL classification of leukemia cells, flow cytometrypanels, diagnosis of acute leukemia, tdt, descriptions of many diagnostictests used in leukemia treatment. From the University of Washington,Department of Laboratory Medicine, Hematopathology Laboratory.

Treatment for childhood leukemias

ALL. The primary treatment for newly diagnosed ALL is combination chemotherapy. Radiationand bone marrow transplantation may be used in some cases. Treatment begins with an intense

treatment called "induction" with a combination of several chemotherapy drugs, usually cytosinearabinoside, vincristine, prednisone, L-asparaginase, and daunorubicin. The goal of induction is

to kill most of the leukemia cells; most patients do not have any leukemic cells in the bone

marrow at the end of induction. (At least, not detectable in a light-microscopical examination ofstained bone marrow smear.) The next phase is called "consolidation" in which a different

combination of drugs is administered, usually methotrexate, cyclophosphamide, cytosine

arabinoside, mercaptopurine, and prednisone. "Maintenance" follows, in which thechemotherapy is lessened to a few of the drugs administered less frequently. Maintenance is

generally well tolerated by the patient. Often a period of maintenance is followed by another

cycle of induction-consolidation, called "re-intensification". Total therapy lasts from two to threeyears. Detailed information on this web site:

ALL clinical trials page and ALL main page on this ped-onc site

AML. In general, newly diagnosed AML is initially treated more aggressively than is ALL.Intensive chemotherapy followed by bone marrow transplantation is becoming the first treatment

chosen, especially when a suitable donor is available. After the intensive chemotherapy and/or
http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.ped-onc.org/diseases/ALLclintrial.htmlhttp://www.ped-onc.org/diseases/ALL.htmlhttp://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.ped-onc.org/diseases/ALLclintrial.htmlhttp://www.ped-onc.org/diseases/ALL.html


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bone marrow transplant, children with AML do not go on maintenance; studies have shown that

AML children in remission have had as much chemotherapy as their bodies can tolerate, and

additional maintenance chemotherapy does not benefit them.

Chronic myeloid leukemias. As in AML, intensive chemotherapy and/or BMT are generally

employed. Currently (2005), imatinib mesylate (Gleevec) is being studied in clinical trials.Chronic leukemias have three clinical phases: chronic, accelerated, and blast crisis. Prognosis

depends on the clinical phase of the disease.

Relapsed leukemia. Relapse, or recurrence of leukemia, can occur anytime during therapy or

after completion of treatment. Generally, it is more difficult to cure a child after relapse of the

leukemia; relapse during or soon after the completion of treatment is considered less favorable

than relapse a year or several years after treatment. Treatment depends on the site of relapse,whether it is in the bone marrow, central nervous system, testes, or other locations. Aggressive

chemotherapy and radiation treatment, often followed bone marrow transplantation, are used to

treat relapse of childhood leukemia.

New Treatments

What's on the horizon for leukemia treatment? The following organization talks about new

treatments:

Research updates from the Leukemia and Lymphoma Society

The big news (early 2000s) for the treatment of CML (and Ph+ ALL) is STI-571. Brian Druker

(Oregon Health Sciences University in Portland) is the Leukemia and Lymphoma Society doctor

prominent in this research.

Gleevec web site

ALL: New ideas for treatmentbys for ALL are also listed on the Clinical Trials for ALL page.

In 2004, ara G entered the treatment plans for T-cell ALL. Clofarabine (2005) shows promise forrefractory (relapsed) ALL. More information in an essay on your author's private web site:

from sea to ara c

Statistics

Leukemia accounts for approximately 35% of all childhood cancers Approximately 1 in 1000 children will be diagnosed with leukemia by the age

of 19 It is more common in children under the age of 10 The five year survival rate for children diagnosed with leukemia and

subsequently treated is approximately 70% 2500 cases of leukemia are diagnosed per year in the US
http://www.lls.org/http://www.gleevec.com/http://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.pfeist.net/ALL/arac/sea2arac7.htmlhttp://www.lls.org/http://www.gleevec.com/http://www.ped-onc.org/diseases/ALLclintrial.html#newtreatmentshttp://www.pfeist.net/ALL/arac/sea2arac7.html


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Ped-Onc Resources for Leukemia

The following ped-onc resource lists have appropriate sections for parents of children with

leukemia:

childhood cancer e-mail lists - the ACOR ALL-kids list and the generalchildhood cancer list, ped-onc, are both appropriate

support organizations - Leukemia and Lymphoma books and printed materials - leukemias and lymphomas (and don't miss the

book for children, I'm Still Me) young people with leukemia - personal home pages Kidz With Leukemia. Computer program for kids. Read my in-depth review for

details.

Links to More Information

The following web sites provide good, general information on lymphomas cancers and theirtreatment.

General Information on Leukemia and Leukemia Research

Leukemia Education Series on the Leukemia and Lymphoma site. A variety ofinformation.

Childhood Leukemia Center. This site is the online version ofChildhoodLeukemia by Nancy Keene (listed above under books).

Cancer.gov site. Excellent. MedLine Plus. Links to a lot of useful information.

The Leukemia and Lymphoma Society. This US society web site providesgood basic information on leukemias. They have booklets to download as pdffiles (or to send for), disease descriptions, treatments, patient services, etc.Especially recommended: NewsLine articles

Leukaemia and Lymphoma Research. Information on leukemia from thisBritish society. There are a lot of "goodies" on this site, links to reviews aswell as good basic information.

Leukemia Links: Granny Barb and Art's. This classic site provides useful andinformative information with great links.

St. Jude's Research Hospital page on Leukemia. This page has information onleukemia and the latest research at St. Jude's.

ALL-KIDS web site, the reference site for the ALL-kids mail list.

JMML Foundation. Excellent resource, specific for JMML, JuvenileMyelomonocytic Leukemia. Founded by parents, covers all aspects of JMML. AML: Caylee's Hope. Good resources for AML, another parent site. Children's Cancer Web. Links and descriptions of leukemia. Excellent. Medicine Online. Information on leukemia and links to more information.

Although this is a dot-com site, it has some good links, and may acquire moregood links in the future. I like the atlas of acute leukemia and the leukemialinks.
http://www.ped-onc.org/cfissues/maillist.htmlhttp://www.ped-onc.org/cfissues/maillist.html#leukemialistshttp://www.ped-onc.org/resources/supportorg.html#leuklymphsupporghttp://www.ped-onc.org/cfissues/books.html#leukemiahttp://www.ped-onc.org/cfissues/books.html#stillmehttp://www.ped-onc.org/hp/leukpages.htmlhttp://www.kidzwithleukemia.com/http://www.ped-onc.org/cfissues/kidz.htmlhttp://www.lls.org/resourcecenter/freeeducationmaterials/#ALLhttp://www.childhoodcancerguides.org/leukemia.htmlhttp://www.cancer.gov/cancerinfo/types/childhoodcancershttp://www.nlm.nih.gov/medlineplus/leukemiachildhood.htmlhttp://www.lls.org/http://www.lls.org/#/resourcecenter/enewsletters/http://www.beatbloodcancers.org/http://www.acor.org/leukemia/http://www.stjude.org/leukemiahttp://www.stjude.org/leukemiahttp://www.all-kids.org/http://www.jmmlfoundation.org/http://www.cayleeshope.com/http://www.cancerindex.org/ccw/guide2l.htmhttp://www.meds.com/leukemia/leukemia.htmlhttp://www.ped-onc.org/cfissues/maillist.htmlhttp://www.ped-onc.org/cfissues/maillist.html#leukemialistshttp://www.ped-onc.org/resources/supportorg.html#leuklymphsupporghttp://www.ped-onc.org/cfissues/books.html#leukemiahttp://www.ped-onc.org/cfissues/books.html#stillmehttp://www.ped-onc.org/hp/leukpages.htmlhttp://www.kidzwithleukemia.com/http://www.ped-onc.org/cfissues/kidz.htmlhttp://www.lls.org/resourcecenter/freeeducationmaterials/#ALLhttp://www.childhoodcancerguides.org/leukemia.htmlhttp://www.cancer.gov/cancerinfo/types/childhoodcancershttp://www.nlm.nih.gov/medlineplus/leukemiachildhood.htmlhttp://www.lls.org/http://www.lls.org/#/resourcecenter/enewsletters/http://www.beatbloodcancers.org/http://www.acor.org/leukemia/http://www.stjude.org/leukemiahttp://www.all-kids.org/http://www.jmmlfoundation.org/http://www.cayleeshope.com/http://www.cancerindex.org/ccw/guide2l.htmhttp://www.meds.com/leukemia/leukemia.html


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National Children's Leukemia Foundation. Provides a range of services, fromhelp in finding donors to referrals to fundraising.

Technical Information

University of Virginia's site on blood cells - a good tutorial from the Universityof Virginia. University of Washington REAL classification of leukemia cells, flow cytometry

panels, diagnosis of acute leukemia, tdt, descriptions of many diagnostictests used in leukemia treatment. From the University of Washington,Department of Laboratory Medicine, Hematopathology Laboratory.

ASCO online, links to abstracts on pediatric lymphoma and leukemia. ASH: American Society of Hematology. Links to the annual meetings and

abstracts and to journals. A good way to follow the latest research inleukemia.

Acute Lymphoblastic Leukemia (ALL)

Acute lymphoblastic leukemia (ALL) is a fast-growing cancer of the white blood cells.

Lymphocytes are a type of white blood cell that the body uses to fight infections. In ALL, thebone marrow makes lots of unformed cells called blasts that normally would develop into

lymphocytes. However, the blasts are abnormal. They do not develop and cannot fight infections.
http://www.leukemiafoundation.org/http://www.leukemiafoundation.org/http://www.leukemiafoundation.org/http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.asco.org/ASCOv2/Meetings/Abstractshttp://www.hematology.org/http://www.leukemiafoundation.org/http://www.med-ed.virginia.edu/courses/path/innes/wcd/http://depts.washington.edu/labweb/Divisions/Hema/Flow.htmhttp://www.asco.org/ASCOv2/Meetings/Abstractshttp://www.hematology.org/


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The number of abnormal cells (or leukemia cells) grows quickly. They crowd out the normal red

blood cells, white blood cells and platelets the body needs.

Acute lymphoblastic leukemia symptoms and diagnosis

There are about 4,000 new cases of ALL in the United States each year. It appears

most often in children younger than age 10. ALL is the most common leukemia in

children. However, it can appear in people of any age about one-third of cases

are adults. Acute lymphoblastic leukemia may also be called acute lymphocytic

leukemia or acute lymphoid leukemia.

Signs and symptoms

The symptoms a person with ALL has depend on how many normal blood cells he or

she has. Symptoms also depend on how many leukemia cells there are and where

they collect in the body.

Red blood cells carry oxygen throughout the body. Low numbers of red bloodcells can lead to anemia -- feeling tired or weak, being short of breath andlooking pale.

White blood cells fight infections. Low numbers of white blood cells can leadto fever and frequent infections that are hard to treat.

Platelets control bleeding. Low numbers of platelets can lead to cuts that healslowly, easy bruising or bleeding and tiny red spots under the skin(petechiae).

High numbers of leukemia cells can cause pain in the bones or joints, lack ofappetite, headache or vomiting. These symptoms are less common.

Diagnosis

ALL is diagnosed when blood and bone marrow samples show a large number of

abnormal lymphocyte blasts. To find out the type of ALL and how well it might

respond to treatment, doctors test samples taken from the blood and bone marrow

to learn:

The size and number of leukemia cells. The type of lymphocyte affected the leukemia cells can begin from one of

two types of lymphocytes, B cells or T cells. What changes appear in the chromosomes of the leukemia cells. This is

called cytogenetics.

Doctors also use a test called a lumbar puncture (or spinal tap) to find out whether there areleukemia cells in the fluid around the brain and spinal cord.

Based on these tests, doctors may categorize ALL into one of the following types:


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Early pre-B ALL Common ALL Pre-B-cell ALL Mature B-cell ALL (Burkitt leukemia) Pre-T-cell ALL Mature T-cell ALL

The type of ALL is one of several factors doctors use to plan treatment.

reatment options for acute lymphoblastic leukemia

ALL can get worse quickly, so doctors usually begin treatment right away. To plan

the treatment, doctors look at a patient's risk factors (also called prognostic

factors). Risk factors are patient and disease traits that clinical research studies

have linked to better or poorer outcomes from treatment. Examples of risk factors

are a patient's age and the type of ALL he or she has. For more details, see Risk

Factors for Planning ALL Treatment.

For a patient with ALL, the treatment plan may include:

Chemotherapy drugs that destroy cancer cells or stop them from growing(discussed further below). Some form ofchemotherapy will be part of thetreatment plan for all patients with ALL.

Radiation therapy most patients do not receive radiation therapy.However, children who have signs of disease in the central nervous system(brain and spinal cord) or have a high risk of the disease spreading to thisarea may receive radiation therapy to the brain.

Bone marrow or cord blood transplant (also called a BMT) a transplant

(discussed further below) offers some patients the best chance for a long-term remission of their disease. Because transplants can have serious risks,this treatment is used for patients who are less likely to reach a long-termremission with chemotherapy alone.

Chemotherapy for acute lymphoblastic leukemia

There are three phases of chemotherapy treatment for ALL: induction, consolidation

and maintenance. Many patients also receive treatment called intrathecal

chemotherapy to prevent leukemia from spreading to the central nervous system.

Induction chemotherapy

Most patients with ALL are given induction chemotherapy. The goal of induction

therapy is to bring the disease into remission. Remission is when the patient's blood

counts return to normal and bone marrow samples show no sign of disease.

Induction therapy achieves a remission in more than 95% of children and in about

75% to 89% of adults. [1, 2] Induction therapy is usually very intense and lasts

about one month. After induction chemotherapy, the next step may be a transplant

or consolidation chemotherapy, depending on the treatment plan.
http://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Chemotherapy/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Radiation/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Lrn_about_Disease/ALL/Risk_Factors_for_Planning_ALL_/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Chemotherapy/index.htmlhttp://www.marrow.org/PATIENT/Undrstnd_Disease_Treat/Undrstnd_Treat_Opt/Lrn_Other_Treatment/Radiation/index.html


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When a patient is diagnosed with ALL, he or she may have 100 billion leukemia

cells. If induction therapy destroys at least 99% of these cells, the patient is in

remission. However, that could still leave 100 million leukemia cells in the body. If

these cells are not destroyed, they can grow and multiply and cause a relapse of

the disease.

Consolidation therapy

Consolidation therapy, the second phase of chemotherapy, is also intense. It lasts

about four to eight months. The goal of consolidation therapy is to reduce the

number of disease cells left in the body. The drugs and doses used during

consolidation therapy depend on the patient's risk factors.

Maintenance therapy

If a patient stays in remission after induction and consolidation therapy,

maintenance therapy begins. The goal is to destroy any disease cells that remain sothat the leukemia is completely gone. Maintenance therapy is less intense than the

other two phases. It may last two to three years.

Intrathecal chemotherapy

During all three phases of chemotherapy treatment, many patients receive extra chemotherapy to

destroy leukemia cells that may have spread to the central nervous system (the brain and spinal

cord). This chemotherapy is injected right into the spinal fluid using a lumbar puncture (spinaltap) or an Omaya reservoir (a device placed under the scalp). It is called intrathecal

chemotherapy.

Children with ALL who have a high risk of the disease spreading to the central nervous system

may receive higher or more frequent doses of intrathecal chemotherapy. Some of these childrenmay also be given radiation therapy to the brain. However, radiation to the brain can cause

problems with growth and mental development in children, so doctors try to avoid this treatment.

Chemotherapy success rates for ALL

One way to measure the success of a treatment is tracking how many patients

survive five years or more after treatment.

For children, the overall survival rate after chemotherapy is nearly 80%. [3]This includes children with all levels of risk factors. Survival rates are muchlower for children with high-risk disease, while children with low-risk diseasehave even higher rates of survival.

For adults, the overall survival rate after chemotherapy is about 40%. [3] Thisincludes adults with all levels of risk factors. For adults with high-risk disease,survival rates are much lower, while survival rates are higher for some adultswith low-risk disease.


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Relapse

Induction therapy brings about a remission in most patients, but over time some

patients relapse. A relapse is when the disease returns after a remission. Patients

who relapse after chemotherapy may be treated with different chemotherapy drugs

and/or more intense doses. Patients who relapse soon after remission or while they

are receiving chemotherapy have high-risk disease. For these patients,

chemotherapy is less likely to achieve a long-term remission, but a bone marrow or

cord blood transplant may be effective.

Bone marrow or cord blood transplant for acute

lymphoblastic leukemia

For some patients, a bone marrow or cord blood transplant may offer the best

chance for a long-term remission. A transplant is a strong treatment with risks of

serious side effects, so it is not used for all patients with ALL. Transplants are usedwhen chemotherapy alone is unlikely to provide a long-term remission.

Allo

acute b lymphoblastic leukemia

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