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CASE 205

CASE 205. Denyo Adjoa Zakhia, MD Kristin Hunt Karner, MD Henry Ford Hospital, Detroit, MI. 61 year-old Caucasian male Cough, night sweats, low-grade fever Complete blood count Hemoglobin – 9.4 gm/dl White blood cell count – 71.1 K/uL Platelet count – 135 K/uL Peripheral blood smear

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CASE 205

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  1. CASE 205 Denyo Adjoa Zakhia, MD Kristin Hunt Karner, MDHenry Ford Hospital, Detroit, MI

  2. 61 year-old Caucasian male • Cough, night sweats, low-grade fever • Complete blood count • Hemoglobin – 9.4 gm/dl • White blood cell count – 71.1 K/uL • Platelet count – 135 K/uL • Peripheral blood smear • Leukocytosis with 79% blasts CLINICAL HISTORY

  3. BONE MARROW ASPIRATE Cellularity ->95% Blasts-87% BONE BIOPSY H&E CD1a

  4. FLOW CYTOMETRY cyto CD3

  5. FLOW CYTOMETRY SUMMARY

  6. CHROMOSOMAL ANALYSIS OF BONE MARROW ASPIRATE 46,XY,del(16)(q13)[6]/46,XY[14] FISH ANALYSIS No genetic abnormalities involving: MYC(8q24) BCL6(3q27) IGH-BCL2(14:18) API2-MALT1(11:18) BCR-ABL(9:22) MLL (11q23) Inv 16

  7. WHAT IS THE DIAGNOSIS? EGIL WHO

  8. EGIL T-lineage: Score 3.5 Myeloid: Score 2.5 • Mixed phenotype acute leukemia, favor T/myeloid

  9. WHO table (criteria) • Acute leukemia of T-cell Lineage with • aberrant myeloid differentiation

  10. INITIAL PROPOSED DIAGNOSIS • Mixed phenotype acute leukemia, favor T/myeloid • Acute leukemia of T-cell Lineage with aberrant myeloid differentiation PANEL CONSENSUS DIAGNOSIS EARLY T-CELL PRECURSOR ALL with MYELOID ANTIGEN EXPRESSION

  11. NEED FOR A CONSENSUS IN THE CLASSIFICATION SYSTEM • Provide appropriate treatment • Provide appropriate prognostication

  12. Hyper CVAD therapy Matched unrelated donor stem cell transplant Complications DVT New diagnosis of Hepatitis C infection Clostridium difficile colitis Reactivation of EBV and BK virus Subdural hemorrhage Patient in remission at day 116+ s/p SCT Treatment and Outcome

  13. IMMUNOPHENOTYPE (Adapted from WHO 2008) CD7 CD1a CD2/CD5 CD3 Cytoplasmic | Surface CD4+ CD8+ CD4/CD8 Double + TdT T-ALL Mature Early

  14. Comprises 85-90% of all lymphoblastic lymphoma • Adolescents > Younger children • 15% pediatric ALL • 25% adult ALL • Presentation: • Mediastinal mass with pleura effusions • Bone marrow involvement if present <20% • High peripheral blood blast counts COMMON DIAGNOSTIC FEATURES OF EARLY T-CELL PRECURSOR ALL (ETP-ALL)

  15. CLINICAL SIGNIFICANCE OF ETP-ALL • Clinical, laboratory and biologic characteristics not useful in prognostication • HIGH RISK • INDUCTION FAILURE • EARLY RELAPSE • CNS RELAPSE • Children treated as high risk • 75% 5 yr-event free survival rate (similar to pre B-ALL/LBL)

  16. DOES MYELOID ANTIGEN EXPRESSION OFFER ANY CLINICAL SIGNIFICANCE? Asnafi et al CD13 and CD33 most frequently expressed myeloid-associated antigens 15% of 91 cases reviewed MPO – negative or very rarely <3%

  17. Uckun et al • Not an adverse prognostic factor in childhood ALL

  18. Evaluated c-kit expression in 295 ALL patients and in 977 AML patients • T- ALL, 5.4 % expressed c-kit • In AML 84% expressed c-kit • Concluded • High c-kit expression is an independent predictor of • Lower complete response in ALL • Higher complete response in AML • Not an independent predictor of disease free survival in ALL or AML • T-ALL may benefit from AML type therapy

  19. Improved survival rates • Normal karyotype • T(10:14)(q24:q11) HOX11:TCRα • Overexpression of HOX11 gene

  20. THANK YOUQUESTIONS???

  21. REFERENCES • Goldberg JM, Silverman LB, Levy DE, et al. Childhood T-cell acute lymphoblastic leukemia: the Dana-Farber Cancer Institute acute lymphoblastic leukemia consortium experience. J ClinOncol. 2003;21:3616-3622. • Onciu M, Lai R, Vega F, et al. Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets. Am J ClinPathol. 2002;117:252-258. • Brunning R, Borowitz M, Matutes E, et al. Precursor B-cell and T-cell neoplasms. In: Jaffe ES, Harris NL, Stein H, et al, eds. Pathology and Genetics of Tumors of Hematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001:109-117. World Health Organization Classification of Tumors. • Thalhammer-Scherrer R, Mitterbauer G, Simonitsch I, et al. The immunophenotype of 325 adult acute leukemias’: relationship to morphologic and molecular classification and proposal for a minimal screening program highly predictive for lineage discrimination. Am J ClinPathol. 2002;117:380-389. • Asnafi V, Beldjord K, Libura M, et al. Age-related phenotypic and oncogenic differences in T-cell acute lymphoblastic leukemias may reflect thymic atrophy. Blood. 2004;104:4173-4180. • Asnafi V, Buzyn A, Thomas X, et al. Impact of TCR status and genotype on outcome in adult T-cell acute lymphoblastic leukemia: a LALA-94 study. Blood. 2005;105:3072-3078. • Uckun FM, Sather HN, Gaynon PS, et al. Clinical feature and treatment outcome of children with myeloid antigen positive acute lymphoblastic leukemia: a report from the Children’s Cancer Group. Blood. 1997;90:28-35. • Tsao AS, Kantarjian H, Thomas D, et al. C-kit receptor expression in acute leukemias: association with patient and disease characteristics and with outcome. Leuk Res. 2004;28:373-378. • Ferrando AA, Neuberg DS, Dodge RK, et al. Prognostic importance of TLX1 (HOX11) oncogene expression in adults with T-cell acute lymphoblastic leukaemia. Lancet. 2004;363:535-536. • Schneider NR, Carroll AJ, Shuster JJ, et al. New recurring cytogenetic abnormalities and association of blast cell karyotypes with prognosis in childhood T-cell acute lymphoblastic leukemia: a Pediatric Oncology Group report of 343 cases. Blood. 2000;96:2543-2549. • X Han, CE Bueso-Ramos. Precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma and acute biphenotypicleukemias. Am J of clinpathol 2007;127:528-544. • Bene MC, Bernier RO, Casasnovas G, et al. The reliability and specificity of c-kit for the diagnosis of acute myeloid Leukemias and Undifferentiated leukemias. Blood 1998;15:596-599.

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