KAREN ESTRELLAPEDS PGY-204/28/2011

CASE CONFERENCE

16y/o F comes to ER for abdominal pain for 5 days

and “rash”

HPIAbd pain: 5 days, > in flanks, better when

sittingNo V, N, DNormal stoolNo dysuria, no vaginal d/cWent to nurse @ school who noted “hard

abdomen”Rash: small red-purple dot in left breast,

later spreading, not painful, not itchy

ROSEpistaxisGums bleeding“more tired and weak”Nocturnal sweatingFrequent burpingNo wt loss

PEVS: T 97.8, HR 106, RR 18 BP 95/62, O2 100%

Wt: 46.9kgHEENT: swollen nasal turbinates w/ min

bleedingCV: wnlLungs: wnlAbdomen: + BS, NT, ND, + hard , protuberant

upper quadrants, difficult to assess HSM, dull to percusion, no adenopathies

Ext: wnlSkin: + petechiae in medial lower quadrant of L

breast

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Differential Dx

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LabsImaging

Labs:CBC: 88.8<5.3/16.2>21, RBC 2.00Coags: 12.1/1.2/27.6138 100 7 125 3 25 1.5

3724 27 0.7 6.2 72P: 2.6UA: turbid, prot: TR, urobilinogen: 5Monospot: negEBV: + IgG

LabsManual differential:

Segm: 3%, Bands: 1%, Lymphs: 89%atypical Lymph: 3%Blasts: 3%RBC morphology: 2+ anysocytosis,

hypocromasiaWBC: 2+ smudge cellsPlatelets: markedly decrease

Uric Acid: 10.5LDH: 3323

ImagingCXR: normalAbd Xray:

Dx:Pre-B cell ALL

ALL(Acute Lymphoblastic

Leukemia)

Definition:Malignant disease of BM in which early

lymphoid precursors proliferate and replace normal hemotopoietic cells. Anemia, neutropenia, trombocytopeniaProlieration in other organs: lymph nodes,

spleen, liverMore common cancer in children

2.8 to 10 cases/100000Peak 2-5y/o> blacks> boysInmunosuppresed sd: Down, ataxia-

telangiectasia, neurofibromatosis type 1

SymptomsAnemia (>75%)Neutropenia (50%), leukocytosis (20%)Thrombocytopenia (75%)

Associated with HSMFeverBone pain: aseptic osteonecrosisEarly satietyHyperuricemiaHeadache: CNS involvementLymphadenopathy > 1.5cmBoys: testicular enlargementSOB: mediastinal mass

WorkupLABORATORYCBCBlood smearCoags: exclude DICCMP: LFTUric acidLDHIf febrile: cx

IMAGINGCXRCT SCANEKG

WorkupBM biopsy

ALL: >20% lymphoblasts (WHO) in BM and/or peripheral blood

Morphology:FAB classification on L1, L2, L3 is no longer valid.

Immunophenotype:Based on CD markers identified by flow

cytometry70-80% are preB cell: +CD10, CD19, CD2015% are preTcell: +CD2, 3, 4, 5, 7, 8

Cytogenetics: hyperdiplody-trisomy 4, 10 (>50): goodHypodyploidy (<46): poor prognosisTranslocations: t (9;22), t (1;19), t(4;11), and

t(8;14)

PrognosisCure rate: 80%

With a 5y/o survival in high-risk pts of 65-75%

With a 75-80% of recent dx who are incorporated on clinical trials

Treatment1. Induction2. Consolidation3. Maintenance4. CNS

1. InductionPlace pt on remission : >90%Drugs:

Vincristine: qwk for 3wksCorticosteroids: qdAsparaginase: qdDoxorrubicin

Success:Minimal residual disease (MRD) at 2wks and

4 wks (<5% lymphoblasts)Main complication: tumor lysis sd

Tumor Lysis SyndromeRapid leukemic cell lysis after chemotherapy

can cause overproduction and overexcretion of uric acid. The precipitation of uric acid in the tubules can lead to oliguric or anuric renal failure.

Risk factors:Age >10 yearsSplenomegalyMediastinal massInitial white blood cell count >20,000/microL

Labs:HyperK, hyperP, hypocalcemia, hyperuricemia

20-25% : + relapseBMCNSBoys: testes

In general: if relapse occurs in <18mo of dx, 5yr prognosis is <21%

2. Consolidation (intensification)Soon after MRD.

Goals: Prevent leukemic regrowth, reduce residual tumor burden, prevent drug-resistance in the remaining leukemic cells.

4-6monthsOccasional “pulse therapy”

24-36moDrugs:

6-mercaptopurine + steroids: qdMetotrexate: qwk

Higher risk of infections: prophylactic Bactrim

3. Maintenance

4. CNSIntrathecal chemotherapy:

Drugs:CytarabineMetotrexatehydrocortisone

Long term OutcomesCNS: cognitive impairment, stroke,

coordination and fine motor, sensory deficitsDecrease linear growthObesityCardiotoxicityHyperglycemiaInfertilityIncrease frequency for secondary

malignanciesBrain tumorAML

In our patientBONE MARROW: Histology:Hypercellular marrow (> 90%) diffusely infiltrated

by blasts. few scattered megakaryocyteslimited hematopoyesisa reticulin stain shows mild to moderate diffuse

increase in reticulin fibers.

Flow cytometry:87%: pre B lymphoblasts that express CD9, CD10,

CD19, CD22, CD79A, HLA-DR, CD45 and TdT NEG: CD20, CD34

7% mature lymphocytes3% grabulocytes

Cytogenetic: 46, XX

CHAM course:HD1Fever on HD1 : started on Cefepime for 5 daysUA: + yeast: fluconazoleAbd: normal appearing kidneys and bladder

HD2:BM bx, PICC, induction therapys/p platelets and PRBC transfusion

Treatment: -vincristine, doxorrubicin, leukovorin, metotrexate,

d/c home with f/u