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Hepatosplenic Gamma Delta T Cell Lymphoma

Background. Hepatosplenic gamma/delta T-cell lymphoma is a neoplasm of mature gamma/delta T-cells with sinusoidal infiltration of spleen, liver, and bone marrowFirst proposed as a distinct entity among peripheral T-cell lymphomas in 1990. Recognized since 1994 in the Revised European American Lymphoma (REAL) classification .

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Hepatosplenic Gamma Delta T Cell Lymphoma

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    1. Hepatosplenic Gamma Delta T Cell Lymphoma Andrew Avery AM Report 07/23/08

    2. Background Hepatosplenic gamma/delta T-cell lymphoma is a neoplasm of mature gamma/delta T-cells with sinusoidal infiltration of spleen, liver, and bone marrow First proposed as a distinct entity among peripheral T-cell lymphomas in 1990. Recognized since 1994 in the Revised European American Lymphoma (REAL) classification

    3. Epidemiology Rare, but given recent characterization, its frequency is not known Previous cases were likely classified as T-cell chronic lymphocytic leukemia/ prolymphocytic leukemia or PTCL, unspecified Most common in adolescents and young adults Predominantly affects males

    4. Clinical Features B-symptoms (i.e. fevers, weight loss, night sweats, fatigue, pruritis) Massive HS w/ or w/o abdominal pain/fullness Moderate anemia Marked thrombocytopenia Features of Hemophagocytic Syndrome in some patients (i.e. low fibrin, hyperferritinemia, hypertriglyceridemia)

    5. Morphology Produces a sinusoidal infiltrate in liver, spleen, and bone marrow, of medium-sized lymphoid cells with round nuclei, moderately condensed chromatin, and moderately abundant, pale cytoplasm Mitotic activity is generally low The white pulp is atrophic. Erythrophagocytosis may be prominent in splenic and bone marrow sinuses Peripheral blood involvement is occasionally seen

    6. Histopathology of the bone marrow in hepatosplenic gamma delta T-cell lymphoma Hypercellular marrow composed of medium-sized atypical lymphs

    7. Hemophagocytosis

    8. Immunophenotype Tumor cells are CD2+ and CD3+, CD5-, CD4-, CD7+, CD8-, CD16+, CD56 +/- Expresses the gamma/delta T-cell receptor (in contrast to the alpha/beta T-cell receptor expressed on PTCL)

    9. Genetic Features The TCR gamma and delta genes are rearranged The TCR beta gene may be rearranged or germline The tumor cells are EBV negative Isochromosome 7q and trisomy 8 have been reported in many cases

    10. Diagnostic criteria for hemophagocytic lymphohistiocytosis Major criteria: (1) Fever: Peak temperature >38.5şC for seven or more days (2) Splenomegaly (3) Cytopenia involving two or more cell lines (4) Hypertriglyceridemia or hypofibrinogenemia: (5) Hemophagocytosis: Demonstrated in bone marrow, spleen, or lymph node. No evidence for malignancy. Alternative criteria:(a) Low or absent natural killer(b) Serum ferritin level >500 ľg/L (c) Soluble CD25 (sIL-2 receptor) >2400 U/mL. The diagnosis of HLH requires the presence of all five major criteria. Either criterion (a) or a combination of criteria (b) and (c) may substitute for one of the major criteria. Henter JI, et al. Semin Oncol 1991; 18:29.

    11. Treatment and Prognosis First line therapy is with CHOP-based chemotherapy There is anecdotal evidence regarding the successful use of allogeneic and autologous stem cell transplantation

    12. Treatment and Prognosis Per one study by Belhadj, K. et al. in the journal Blood, 14/21 pts responded to induction, using either CHOP or cytarabine based chemo (CR-9, PR-5). Of these, 10 relapsed and died b/w 10 & 44 months, 2 died from early cerebral toxoplasmosis, and 2 were in complete remission at 42 and 52 months from diagnosis. Median survival time was 16 months Prognosis considered to be 1-2 years

    13. References 1. Belhadj, K. et al. Blood 2003;102:4261-4269 2. Freedman, AS, and NL Harris. Clinical and pathologic features of mature peripheral T and NK cell lymphomas . In: UpToDate, Freedman, AS (Ed), UpToDate, Waltham, MA, 2008.

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