kul kel hematologi sem v.ppt
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Indriani SilviaPatologi Klinik
FK UNSWAGATI18 Oktober 2013
BLOK 254
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Kelainan pada hematologiJenis kelainan hematologi
Gambaran kelainan hematologi padapemeriksaan laboratorium
Kelainan onkologiJenis pemeriksaan onkologi
Indikasi dan tujuan pemeriksaanInterpretasi hasil pemeriksaan
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Transports oxygen, called oxyhemoglobin,when it gives up its oxygen it isdeoxyhemoglobin.
Also binds and transports carbon dioxide,carbaminohemoglobin.
Makes up ± 97 % of RBC ± 250 million Hbmolecules per RBC
Men: 14-18 mg/dl blood
Women: 12-16 mg/dl blood
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Types of Hemoglobin (Hb)
Hb A
• 96% of adult Hb (α2 β2)
Hb A2• 3% of adult Hb (α2 σ2)
Hb F
• 1 % of adult Hb (α2 γ2)
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• Liver – contains phagocytic cells known as
Kupffer cells that act as a filter for damaged or
aged cells in a manner similar to, but less
efficient than the phagocytic cells in thespleen. If the bone marrow cannot keep up with the physiologic
demand for blood cells, the liver may resume the
production of blood cells that it began during fetal life
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Bloodgroup
Antigen Antibody Donor to Recipientfrom
A A Anti-B A A, O
B B Anti-A B B, O
AB AB Neither AB A, B, AB, O
O Neither Anti-A/Anti-B
O, A, B, AB O
Universal donor "O"Universal recipient "AB"
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Acute Hemolytic Reaction Symptoms
• Fever, chills and fever, the feeling of heat along thevein in which the blood is being transfused
• Pain in the lumbar region• Constricting pain in the chest, tachycardia, hypo-
tension• Hemoglobinemia with subsequent hemoglo-binuria
and hyperbilirubin-emia.
"feeling of impending doom"
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Acute Hemolytic Reaction
Causes
• Human error!
Transfused red cells react with circulating antibody in therecipient with resultant intravascular hemolysis
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Acute Hemolytic Reaction
Frequency
• Rare
Prevention
• Proper identification of patients, pretransfusion blood
samples and blood components at the time of
transfusion
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Delayed Hemolytic ReactionFalling hematocrit
due to extravascular destruction of the transfused redblood cells)
Positive direct antiglobulin (Coombs) test (DAT)
Occurs about 4-8 days after blood transfusion
Patients may manifest fever and leukocytosis
Appearing to have an occult infection.
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Febrile Transfusion Reaction
Fever or chill fever
• temperature rise of 1.5 F or 1.0 C from the baseline
Cytokines and antibodies to leukocyte antigensreacting with leukocytes or leukocyte fragments
1 in 8 transfusions
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Allergic – urticaria
Laryngeal edema and bronchospasm
1% of recipients
• If coupled with another sign, such as fever, evaluationfor a hemolytic reaction may be indicated.
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Allergic – Anaphylaxis
Anaphylactic or anaphylactoid
• Respiratory involvement with dyspnea or stridor
Cardiovascular instability
• hypotension, tachycardia, loss of consciousness,
cardiac arrhythmia, shock and cardiac arrest
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Volume Overload Transfusion-related volume overload
Infuse smaller volumes more slowly
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Bacterial Contamination
Hypotension, shock, fever and chills, nausea and
vomiting, and respiratory distress
Gram stain and blood culture
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Follow protocol for transfusion reactions
implemented by the institution
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Stop the transfusion immediately!
Disconnect the intravenous line from theneedle.
Seek medical attention immediately. If thepatient is suffering cardiopulmonary collapse,and medical attention is not immediately
available, press for ―Code"
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Check to ensure that the patient name and
registration number on the blood bag label
exactly with information on the patient's
identification
Do not discard the unit of blood that has been
discontinued because it may be necessary for
the investigation of the transfusion reaction.
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Reaction Type Treatment - Adult Pediatric Follow-up
Acute
Hemolytic
Reactions
Diuretic therapy: Initially, give 40-80
mg Furosemide (Lasix)
intravenously. This dose can be
repeated once. Lack of response
to furosemide in 2-3 hours
indicates the presence of acute
renal failure.
Pediatric dose: 1-2
mg/kg/dose.
May repeat once
at 2-4 mg/kg.
Treat shock and disseminated
intravascular coagulation with
appropriate measures if and when
they appear.
Water loading: The patient should be
hydrated to maintain urinary
output of at least 100 mL/hr until
urine is free of hemoglobin.
Infuse a loading dose of 0.9% sodium
chloride or 5% dextrose in 0.45%
sodium chloride. Chart hourly
urine output. Maintain the urine
output by administeringintravenous fluid at 100 mL/hour
until the urine is free of
hemoglobin. If the patient's
urinary output does not increase,
with this hydration any additional
fluids should be infused with
caution.
Pediatric patients
should receive a
smaller loading
volume of fluid
in proportion to
their body
surface area.
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Delayed
Hemolytic
Transfusion
Reactions
Specific treatment generally is not
necessary
Supplemental transfusion of blood lacking
the antigen corresponding to the
offending antibody may be necessary
to compensate for the transfused
cells that have been removed fromthe circulation.
Reaction Type Treatment - Adult Pediatric Follow-up
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Reaction Type Treatment - Adult Pediatric Follow-up
Allergic
Transfusion
Reactions
Antihistamines(e.g., Benadryl). Give
50-100 mg orally or intravenously. If
urticaria develops slowly,
antihistamines may be given orally.
Pediatric dose: 1-2
mg/kg
intramuscularly or
intravenously for 25-
50 mg per average
dose.
Routine use of Benadryl as premedication
for all transfusions, regardless of a history
of allergic reactions, is discouraged.
Aminophylline for wheezing, at a dose
of 125-250 mg intravenously slowly
over a period of about five minutes
Pediatric dose: 3
mg/kg/dose in
intravenous drip over
of 20 minutes.
Epinephrine for severe, acute reactions
including laryngeal edema or
bronchospasm Give 0.1-0.5 mg (0.1-0.5
mL of a 1:1000 solution)
subcutaneously. Subcutaneous dose
may be repeated at 10-15 minute
intervals. The total subcutaneous dose
in a 24-hour period, with rare
exception, should not exceed 5 mg.
Pediatric dose: 0.03
mL/M2 (0.03 mg/M2
of a 1:1000 solution)
given subcutaneously.
A single pediatric
dose should not
exceed 0.3 mg.
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Febrile
Transfusion
Reactions
Premedicate the patient with
acetaminophen or other
antipyretic agents when previous
reactions have been extremely
bothersome. Pediatric dose: 10
mg/kg to a maximum of 600 mg.
Aspirin will adversely affect the patient's
platelet function, so non-aspirin
antipyretic agents are preferable.
Severe shaking
Chills
(rigors) can be controlled by the
sedative effect of Benadryl or
Demerol (25-50 mg given
intramuscularly or intravenously
Note: Demerol may cause acute
respiratory arrest. An opiate
antagonist (Narcan) should be
immediately available.
Sepsis Due to
Bacterial
Contaminationof Donor Blood
Treatment of septic shock includes:
terminating the suspected
transfusion immediately, cardio-vascular and respiratory support,
blood culture of the patient, and
administration of broad spectrum
antibiotics including anti-
pseudomonas coverage if the
blood component involved is Red
Blood Cells.
Reaction Type Treatment - Adult Pediatric Follow-up
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Abnormally low number of RBC or Hb levels
Reduced oxygen carrying capacity
Causes
Blood loss Increased rate of red cell destruction
• Hemolytic anemia
Deficient or impaired red cell production
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Risk factors Poor diet Intestinal disorders Menstruation Pregnancy Chronic conditions Family history
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―NOT A DISEASE‖ but a symptom
• Dependent on severity, speed of development, age,
health status and compensatory mechanisms
• Associated with impaired O2 transport, alteration inRBC structure or with chronic illness
• Not expressed until 50% of RBC mass is lost
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Signs and symptoms The main symptom of most types of anemia is
fatigue• Weakness
• Pale skin• Tachycardia• Shortness of breath• Chest pain• Dizziness
• Cognitive problems• Numbness or coldness in your extremities• Headache
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Iron Deficiency Anemia Most common form of anemia
• Affects about one in five women• Half of pregnant women and 3 percent of men in the
United States. The cause is a shortage of the element iron
• Nutritional imbalance• Slow, chronic bleeding disorders• Inability to recycle plasma iron
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Vitamin Deficiency Anemias Folate and vitamin B-12 deficiency Intestinal disorder that affects the absorption of
nutrients Fall into a group of anemias called megaloblastic
anemias, in which the bone marrow produceslarge, abnormal red blood cells.
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Anemia of Chronic Disease Interfere with the production of red blood cells,
resulting in chronic anemia Kidney failure also can be a cause of anemia
• The kidneys produce a hormone called erythropoietin,which stimulates your bone marrow to produce redblood cells. A shortage of erythropoietin, which can result from kidney failure
or be a side effect of chemotherapy, can result in a shortage ofred blood cells.
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Aplastic Anemia Life-threatening anemia caused by a decrease in
the bone marrow's ability to produce all threetypes of blood cells — red blood cells, whiteblood cells and platelets
Cause of aplastic anemia is unknown• autoimmune disease
• Chemotherapy
• Radiation therapy• Environmental toxins
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Anemias associated with bone marrow diseaseLeukemia and myelodysplasia, can cause
anemia by affecting blood production in the bonemarrow
Effects vary from a mild alteration in blood
production to a complete, life-threateningshutdown of the blood-making process Myelodysplasia is a pre-leukemic condition
that can cause anemia.Other cancers of the blood or bone marrow, such as
multiple myeloma, myeloproliferative disorders orlymphoma, can cause anemia.
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Hemolytic Anemias Red blood cells are destroyed faster than bone
marrow can replace them. Autoimmune disorders can produce antibodies to
red blood cells, destroying them prematurely• Hemolytic anemias may cause yellowing of the skin
(jaundice) and an enlarged spleen.
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Hereditary Spherocytosis
Mutations in the ankyrin molecule with a
secondary deficiency of spectrin along the cell
membrane• Reduced red cell stability
Does not affect oxygen carrying capacity
Splenic sequestration
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Sickle cell anemia Defective form of hemoglobin that forces red
blood cells to assume an abnormal crescent(sickle) shape.
• Mutation for the gene coding for the β-globulin chain Valine is substituted for glutamic acid HbS
Red cells die prematurely, resulting in a chronicshortage of red blood cells.
• Block blood flow through small blood vessels in thebody, producing other, often painful, symptoms.
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Single base pair mutation results in a
single amino acid change.
Under low oxygen, Hgb becomes insoluble
forming long polymersThis leads to membrane changes
(―sickling‖) and vasoocclusion
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Deoxygenation of SS erythrocytes leads tointracellular hemoglobin polymerization, loss ofdeformability and changes in cell morphology.
OXY-STATE DEOXY-STATE
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α- Thalassemia
Common in Asians
Deletion of glubulin chain loci
4 possible degrees of α thalassemia:• Silent carrier, loss of a single α globulin gene
• α thalassemia trait, loss of a pair of globulin gene
• HbH disease, only a single gene is present
• Hydrops fetalis, deletion of all α globulin
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The only treatments are stem cell
transplant and simple transfusion.
Chelation therapy to avoid iron overload
has to be started early.
TALASSEMIA
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An acquired disorder of the bone marrow that
causes the overproduction of all three blood cell
lines
• white blood cells, red blood cells, and platelets It is a rare disease that occurs more frequently in
men than women, and rarely in patients under 40
years old.
causes is unknown
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Usually develops slowly, and most patients are
asymtomatic
• abnormal bone marrow cells proliferate uncontrollably
leading to acute myelogenous leukemia Patients have an increased tendency to form
blood clots that can result in strokes or heart
attacks
• Some patients may experience abnormal bleedingbecause their platelets are abnormal
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Symptoms Headache Dizziness Pruritus Fullness in the left upper abdomen Erythema (face) Shortness of breath Orthopnea Symptoms of phlebitis
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Collectively known as White Blood Cells (WBC)
Formed elements of the blood with organellesand a nucleus but lack hemoglobin
Protect the body against microorganisms andremove dead cells and debris from the body
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Per µl blood Per µl of blood
Total WBC count 5,000 – 10,000
Neutrophils 50 - 70% 2,000 – 7,000
Lymphocytes 20 - 40% 1,000–
4,000Monocytes 1 – 6% 50 – 600
Eosinophils 1 – 5% 50 – 500
Basophils 0 – 2% 0 - 100
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Leukopenia
• Decreased peripheral white cell count due to decrease
numbers of any specific types of leukocytes
Leukocytosis• Non –neoplastic elevation of WBC count
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Neutropenia
Reduction in the number of granulocytes
(<1500/µl)
Increased risk of infection• Reduced phagocytosis response
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Neutropenia
Decreased or defective granulopoiesis
• Aplastic anemia
• Anti-neoplastic agents• Other drugs: chloramphenicol, sulfonamides,
chlorpromazine
Accelerated removal or destruction
• Aggressive and chronic infections
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Manifestation of Neutropenia
Infections
Signs and Symptoms Malaise, chills, fever
Ulcerative necrotizing lesions of the mouth, skin
vagina and GI tract
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Reactive Leukocytosis
Increase number of WBC
Common reaction due to a variety of
inflammatory states caused by microbial or non-microbial stimuli
Usually non-specific
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Causes of Leukocytosis
Polymorphonuclear leukocytosis Acute bacterial infections
Eosinophilic leukocytosis Allergic disorders
Monocytosis Chronic infections
Lymphocytosis Chronic immunologic disease
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Leukemoid reaction – this is an extreme neutrophilia with a WBCcount > 30 x 109/L
Many bands, metamyelocytes, and myelocytes are seen
Occasional promyelocytes and myeloblasts may be seen.
This condition resembles a chronic myelocytic leukemia (CML), butcan be differentiated from CML based on the fact that in leukemoidreactions:
There is no Philadelphia chromosome
The condition is transient
There is an increased leukocyte alkaline phosphatase score (moreon this later)
Leukemoid reactions may be seen in tuberculosis, chronicinfections, malignant tumors, etc.
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1. Leukemia – neoiplasms of the hematopoietic
stem cells
2. Malignant lymphomas – cohesive tumor
lesions; neoplastic lymphocytes3. Plasma cell dyscrasias – arising from the
bones; localized disseminated proliferation of
antibody forming cells
4. Histocytoses – proliferative lesions ofhistiocytes
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Leukemia
Malignant neoplasm of the hematopietic stem
cells
BM replaced by unregulated, proliferating,immature neoplastic cells blood leukemia
enter spleen, lymph nodes
Most common cancer in the paediatric age
Leading cause of death in children between 3and 14 years old
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Classification of Leukemia A. According to cell type and state of cell maturity
• Lymphocytic – immature lymphocytes and theirprogenators
• Myelocytic – pluripotent myeloid stem cells andinterferes with maturation of all granulocytes, RBCand platelets
B. Acute or Chronic
• Acute – immature cells (blast)• Chronic – well differentiated leukocytes
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Congenital• Pelger-Huet anomaly
Bilobed and occasional unsegmented neutrophils
Autosomal recessive disorder
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• Neutrophil hyper-segmentation Rare autosomal dominant condition
Neutrophil function is essentially normal
• May-Hegglin anomaly
Neutrophils contain basophilic inclusions of RNA Occasionally there is associated leucopenia
Thrombocytopenia and giant platelet are frequent
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• Alder’s anomaly Granulocytes, monocytes and lymphocytes contain granules which stain
purple with Romanowsky stain
Granules contain mucopolysaccharides
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• Chediak-Higashi syndrome Autosomal recessive disorder
Giant granules in granulocytes, monocytes and lymphocytes
Partial occulocutaneous albinism
Depressed migration and degranulation Recurrent pyogenic infections
Lymphoproliferative syndrome may develop
Treatment is BMT
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Acquired Toxic granulation
Dohle bodies
Pelger cells
Hypersegmented neutrophils
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Leucocyte adhesion deficiencyChronic granulomatous disease
Chediak-Higashi syndrome
Primary immunodeficiency Severe combined immunodeficiency
Common variable immunodeficiency
Isolated IgA deficiency
T-cell immunodeficiency
Thymic aplasia (Di George syndrome)
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Block in the differentiation of leukemic cells with
prolonged genration time clonal expansion of
the transformed stem cells + failure of maturation
accumulation of leukemic blast
suppressnormal hematopoietic stem cells
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Features
Sudden onset (3 months)
Depressed marrow function
Bone pain and tenderness Generalized lymphadenophaty
Splenomegaly, hepatomegaly
CNS: headache, vomiting
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Most common leukemia in children (80%)
Treatable and potentially curable
Classified according to lymphocytes and state
of maturation1. Early B cell
2. Pre-B cell
3. Mature B cell
4. Early T cell5. Mature T cell
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Acute Non-lymphocytic Leukemia (ANLL)
Most common in adults; >50% 60years old
70% of adults will enter remission with induction
chemo• 25-35% of those in remission will have a 5 year
survival rate
BM transplant
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Treatment
Selective radiation
Chemotherapy
1. Induction2. Intensification
3. Maintenance and consolidation
Bone marrow transplant
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Insidious onset
Incidental findings during routine exam
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Proliferation and accumulation of mature
lymphocytes which are immunologically
incompetent
• B cell line (US)• T cell line (Asia)
Hairy cell leukemia
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15% of all leukemias
Chromosomal abnormality (Ph1)
Mostly B cell disease
• Leukocytosis• Splenomegaly
• Hepatomegaly
• Lympadenopathy
Bone marrow transplant 5 year survival for 50-75% of patients
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Two distinct phases
Chronic
• Last about 3-4 years
•Near end
accelerated phase: fever, night sweats,malaise
Acute
• 2-4 months
• Poor prognosis, palliative management
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Primary solid tumors of the lymphoid system
Cancers involving lymphocytes during
maturation or storage in the bone marrow
Third most common malignacy in children
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Hodgkin’s Lymphoma
Disorders primarily involving the lymphoid
tissues
Anatomical spread Morphological presence of Reed-Sternberg cells
60-90% cure rate
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Manifestations of Hodgkin’s
A symptoms
• Painless progressive enlargement of a single or group
of nodes (neck)
• May spread continuously through out the lymphatic
system
B symptoms
• Fever, night sweat, weight loss
• Fatigue, anemia
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Treatment for Hodgkin’s
Radiation
Chemotherapy
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Non-Hodgkin’s Lymphoma
Involves lymphoid tissue and may spread to
various tissues
Mostly B cell (80%) Cause may be viral or genetic
• EBV
• Immunosuppresed patients
AIDS After organ transplant
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Treatment
Early stage radiation
Late stage chemo and radiation
BM transplant
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Transfuse for any severe anemia with
physiologic compromise.
Decide early whether transfusion will be
rare or part of therapy. Avoid long-term complications by working
with your blood bank and using chelation
theraoy.
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