Lymphoma hepatosplenic T-cell

Lymphoma appears to be increased in patients with untreated inflammatory bowel disease. Anti-TNF agents may further increase the risk of lymphoma in this population, although the relative risk is uncertain. An increased number of cases of hepatosplenic T-cell lymphoma, a rare but usually fetal disease, have been noted in children and young adults, virtually all of whom have been on combined therapy with immunomodulators, anti-TNF agents, or corticosteroids. 

T/NK (natural killer) cell lymphomas are much less common in Western countries and much less understood than the B cell lymphomas. Thus, the discussion of these entities will be much briefer. In addition, a significant number of the T/NK lymphomas, such as enteropathy-type T cell lymphoma, hepatosplenic T cell lymphoma, subcutaneous panniculitis-like T cell lymphoma, blastic NK cell lymphoma, T cell granular lymphocytic leukemia, and adult T cell leukemia/ lymphoma, will not be discussed in this review. The reader is advised to consult the recent World Health Organization Classification of Tumours (J2). 

C26. Coles, C. B., Krenacs, L., Stetler-Stevenson, M., Greiner, T. C., Raffeld, M., Kingma, D. W., Abruzzo, L., Frantz, C., Kaviani, M., and Jaffe, E. S., Hepatosplenic T-cell lymphoma A distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood 88, 4265—4274 (1996). 

Rare postmarketing cases of hepatosplenic T cell lymphoma reported. This rare type of T cell lymphoma has a very aggressive disease course and is usually fatal within 2 years of diagnosis... 

Hematologic Leukopenia, neutropenia, pancytopenia, thrombocytopenia, hepatosplenic T-cell lymphoma... 

Cooke CB, Krenacs L, Stetler-Stevenson M, et al. Hepatosplenic T-cell lymphoma a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood. 1996 88(ll) 4265-4274. 

Extranodal NK/T-cell lymphoma, nasal type Enteropathy-type T-cell lymphoma Hepatosplenic gamma-delta T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides/sezary syndrome... 

Salhany, K.E., Feldman, M., Kahn, MX, Peritt, D., Schretzmair, R.D., Wilson, D.M., DiPaola, R.S., Click, A.D., Kant, XA., Nowell, P.C., Kamoun, M. Hepatosplenic y5 T-cell lymphoma ultrastructural immunophenotypic, and functional evidence for cytotoxic T-lympho-cyte differentiation. Hum. Pathol. 1997 28 674— 685... 

Wu H, Wasik MA, Przybylski G, et al. Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lympho-proliferative disorder in renal transplant recipients. Am J Clin Pathol. 2000 113(4) 487-496. 

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