Agressive Lymphoma without Lymphadenopathy

Abstract

Hepatosplenic T cell lymphomas are among a rare breed of aggressive non Hodgkin tumors that usually comprise of immature cytotoxic T cells. They most commonly involve young adults who generally present with constitutional symptoms of fever, weight loss and night sweats. Diagnosis can be challenging in the absence of lymphadenopathy and paucity of bone marrow findings. The discovery of neoplastic cells in peripheral blood is often a finding late in the clinical course. Demonstation of sinusoidal infiltration of T cells on either liver or splenic biopsy is ususally diagnostic. These cells are positive for clonal rearrangement of the γ gene of the T cell receptor which is a hallmark of this disease. Prognosis remains poor and most patients die within two years of diagnosis. We report a case of a patient who presented with unexplained fever and hepatosplenomegaly and after extensive workup was diagnosed with hepatosplenic T cell lymphoma.A 42 year old male with no significant past medical history presented with complains of abdominal pain, fever, malaise and 30lb weight loss over a period of six weeks. Pain was described as intermittent episodes of bilateral flank fullness associated with nausea and vomiting. He had traveled to Mexico 8 weeks earlier and according to him his symptoms began soon after his trip. He denied any headaches, cough, shortness of breath,. No dysuria, and no diarrhea. No smoking alcoholism or illicit drug use was reported. No sick or new sexual contacts. Family history was noncontributory. Vital signs were significant for temperature of 101 F. Physical examination revealed presence of marked hepatosplenomegaly. There was no lympadenopathy as well as no murmurs or rubs. Labs showed pancytopenia with blood counts of 2.7, 9.8 and 101 for WBC, Hb and platelets respectively. Additionally, alkaline phosphatase was elevated while rest of the liver functions was normal. Preliminary diagnosis of infectious versus malignant etiology was made. Peripheral smear did not show any malignant cells. Infectious workup returned negative for HIV, hepatitis, histoplasma, brucella, leptospira, coccidioidomycosis, syphilis, leishmaniasis as well as malaria and dengue fever. A bone marrow biopsy was performed for further evaluation and showed moderately hypercellular bone marrow with erythroid and megakaryocytic hyperplasia but no evidence of malignancy or granulomatous process. Meanwhile patient remained persistently febrile without any growth on blood cultues. An echo was normal with no evidence of endocarditis. Tuberculin test was non reactive. Given the results of the bone marrow biopsy and hepatosplenomegaly without any lymphadenopathy it was thought that a localized infiltrative process of he liver or spleen was likely. Patient thus underwent a liver biopsy disclosing intra sinusoidal collections of atypical CD3, CD7, TIA-1 and CD56 positive lymphocytes also bearing T cell receptor clonal rearrangement consistent with a diagnosis of hepatosplenic T-cell lymphoma which was further classified as gammadelta type.. He was subsequently started on ECHOP regimen and is currently in remission on a three month followup Hepatosplenic lymphoma, though is a rare tumor, should be considered in patients with unexplained hepatosplenomegaly without lymphadenopathy and normal peripheral smear. Liver biopsy is diagnostic most of the time.