Hemophagocytic syndrome associated with nasal T-cell lymphoma: Report of an autopsy case.

Abstract

A 59-year-old man was admitted because of fever and nasal obstruction. On admission, bilateral nasal cavities and the palate were replaced by necrotic tissue. Physical examination revealed cervical lymphadenopathy, hepatosplenomegaly and edema but no icterus. Nasal mucosa and cervical lymph node were biopsied and they showed diffuse proliferation of large tumor cells with convoluted nuclei admixing with macrophages. Tumor cells, studied immunohistochemically, were positive for Leu4, MT1, but negative for SmIg, B1, B2, MB1, OKM1, and the tumor was diagnosed as T-cell lymphoma.During the course, pancytopenia rapidly progressed and the bone marrow aspiration showed hypoplastic marrow with increased large histiocytic cells but no apparent tumor cells. Clinical and laboratory features revealed disseminated intravascular coagulation (DIC). Chemotherapy with the CHOP regimen was initiated but he died of extensive hemorrhagic pneumonia and bronchopneumonia. Autopsy showed lymphoma cell infiltration in pharynx, tonsils, liver, spleen and lymph nodes of neck and carina. Activation of mature histiocytes with marked erythrophagocytosis was characteristic and seen throughout the mononuclear phagocytic system, i.e. splenic red pulp, hepatic sinusoids, lymph node sinuses and bone marrow.This case would be clinically classified in so-called lethal midline granuloma, but histologically diagnosed as nasal T-cell lymphoma complicated by hemophagocytic syndrome and DIC. Erythrophagocytic histiocytosis was considered to be the reactive process to the opportunistic infection associated with the T-cell malignancy.