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CASE CONFERENCE

CASE CONFERENCE. KAREN ESTRELLA PEDS PGY-2 04/28/2011. 16y/o F comes to ER for abdominal pain for 5 days and “rash”. HPI. Abd pain: 5 days, > in flanks, better when sitting No V, N, D Normal stool No dysuria, no vaginal d/c Went to nurse @ school who noted “hard abdomen”

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CASE CONFERENCE

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  1. CASE CONFERENCE KAREN ESTRELLA PEDS PGY-2 04/28/2011

  2. 16y/o F comes to ER for abdominal pain for 5 days and “rash”

  3. HPI • Abd pain: 5 days, > in flanks, better when sitting • No V, N, D • Normal stool • No dysuria, no vaginal d/c • Went to nurse @ school who noted “hard abdomen” • Rash: small red-purple dot in left breast, later spreading, not painful, not itchy

  4. ROS • Epistaxis • Gums bleeding • “more tired and weak” • Nocturnal sweating • Frequent burping • No wt loss

  5. PE • VS: T 97.8, HR 106, RR 18 BP 95/62, O2 100% Wt: 46.9kg • HEENT: swollen nasal turbinates w/ min bleeding • CV: wnl • Lungs: wnl • Abdomen: + BS, NT, ND, + hard , protuberant upper quadrants, difficult to assess HSM, dull to percusion, no adenopathies • Ext: wnl • Skin: + petechiae in medial lower quadrant of L breast

  6. Differential Dx ?

  7. LabsImaging ?

  8. Labs: • CBC: 88.8<5.3/16.2>21, RBC 2.00 • Coags: 12.1/1.2/27.6 138 100 7 125 3 25 1.5 372 4 27 0.7 6.2 72 • P: 2.6 • UA: turbid, prot: TR, urobilinogen: 5 • Monospot: neg • EBV: + IgG

  9. Labs • Manual differential: • Segm: 3%, Bands: 1%, Lymphs: 89% • atypical Lymph: 3% • Blasts: 3% • RBC morphology: 2+ anysocytosis, hypocromasia • WBC: 2+ smudge cells • Platelets: markedly decrease • Uric Acid: 10.5 • LDH: 3323

  10. Imaging • CXR: normal • Abd Xray:

  11. Dx:Pre-B cell ALL

  12. ALL(Acute Lymphoblastic Leukemia)

  13. Definition: • Malignant disease of BM in which early lymphoid precursors proliferate and replace normal hemotopoietic cells. • Anemia, neutropenia, trombocytopenia • Prolieration in other organs: lymph nodes, spleen, liver • More common cancer in children • 2.8 to 10 cases/100000 • Peak 2-5y/o • > blacks • > boys • Inmunosuppresed sd: Down, ataxia-telangiectasia, neurofibromatosis type 1

  14. Symptoms • Anemia (>75%) • Neutropenia (50%), leukocytosis (20%) • Thrombocytopenia (75%) • Associated with HSM • Fever • Bone pain: aseptic osteonecrosis • Early satiety • Hyperuricemia • Headache: CNS involvement • Lymphadenopathy > 1.5cm • Boys: testicular enlargement • SOB: mediastinal mass

  15. Workup LABORATORY • CBC • Blood smear • Coags: exclude DIC • CMP: LFT • Uric acid • LDH • If febrile: cx IMAGING • CXR • CT SCAN • EKG

  16. Workup • BM biopsy • ALL: >20% lymphoblasts (WHO) in BM and/or peripheral blood

  17. Morphology: • FAB classification on L1, L2, L3 is no longer valid. • Immunophenotype: • Based on CD markers identified by flow cytometry • 70-80% are preB cell: +CD10, CD19, CD20 • 15% are preTcell: +CD2, 3, 4, 5, 7, 8 • Cytogenetics: • hyperdiplody-trisomy 4, 10 (>50): good • Hypodyploidy (<46): poor prognosis • Translocations: t (9;22), t (1;19), t(4;11), and t(8;14)

  18. Prognosis • Cure 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

  19. Treatment • Induction • Consolidation • Maintenance • CNS

  20. 1. Induction • Place pt on remission : >90% • Drugs: • Vincristine: qwk for 3wks • Corticosteroids: qd • Asparaginase: qd • Doxorrubicin • Success: • Minimal residual disease (MRD) at 2wks and 4 wks (<5% lymphoblasts) • Main complication: tumor lysis sd

  21. Tumor Lysis Syndrome • Rapid 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 years • Splenomegaly • Mediastinal mass • Initial white blood cell count >20,000/microL • Labs: • HyperK, hyperP, hypocalcemia, hyperuricemia

  22. 20-25% : + relapse • BM • CNS • Boys: testes • In general: if relapse occurs in <18mo of dx, 5yr prognosis is <21%

  23. 2. Consolidation (intensification) • Soon after MRD. • Goals: Prevent leukemic regrowth, reduce residual tumor burden, prevent drug-resistance in the remaining leukemic cells. • 4-6months • Occasional “pulse therapy” 3. Maintenance • 24-36mo • Drugs: • 6-mercaptopurine + steroids: qd • Metotrexate: qwk • Higher risk of infections: prophylactic Bactrim

  24. 4. CNS • Intrathecal chemotherapy: • Drugs: • Cytarabine • Metotrexate • hydrocortisone

  25. Long term Outcomes • CNS: cognitive impairment, stroke, coordination and fine motor, sensory deficits • Decrease linear growth • Obesity • Cardiotoxicity • Hyperglycemia • Infertility • Increase frequency for secondary malignancies • Brain tumor • AML

  26. In our patient BONE MARROW: Histology: • Hypercellular marrow (> 90%) diffusely infiltrated by blasts. • few scattered megakaryocytes • limited hematopoyesis • a 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 lymphocytes • 3% grabulocytes Cytogenetic: 46, XX

  27. CHAM course: HD1 • Fever on HD1 : started on Cefepime for 5 days • UA: + yeast: fluconazole • Abd: normal appearing kidneys and bladder HD2: • BM bx, PICC, induction therapy • s/p platelets and PRBC transfusion Treatment: • -vincristine, doxorrubicin, leukovorin, metotrexate, d/c home with f/u

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