Hepatosplenic T-cell lymphoma with coexistent hepatitis B infection: a rare clinicopathologic entity.
BACKGROUND
Hepatosplenic T-cell lymphoma (HSTL) is a rare extranodal and systemic lymphoma derived from cytotoxic T cells usually of γδ T cell receptor type. It is characterized by primary extranodal disease with typical sinusoidal infiltration of liver, spleen, and bone marrow by medium-sized lymphoid cells.


METHODS
A 29-year-old man, with no significant prior medical history, presented with fever and massive splenomegaly. A diagnosis of HSTL was established by histologic examination and immunohistochemistry. Staging workup demonstrated bone marrow involvement by lymphoma. In addition, the patient was found to have hepatitis B infection. The association of these 2 entities has been described rarely.


CONCLUSIONS
Hepatosplenic T-cell lymphoma is a distinct T cell lymphoma associated with an aggressive clinical course, a poor response to conventional treatment, and an exceedingly high mortality rate. An association of HSTL with hepatitis B as seen in the present case is exceedingly rare, with few cases reported in the literature.
National Institute of Pathology (ICMR), New Delhi - India.
Fouzia
F
Siraj
National Institute of Pathology (ICMR), New Delhi - India.
Varsha
V
Dalal
JLNM Hospital, Srinagar, Kashmir - India.
Afaq A
AA
Khan
Department of Pathology, AIIMS, New Delhi - India.
Deepali
D
Jain
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D017809:Fatal Outcome; D006509:Hepatitis B / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D016130:Immunophenotyping; D008113:Liver Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D013160:Splenic Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D014750:Vincristine / Q000627:therapeutic use
D000998:Antiviral Agents; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone