Case report: an autopsy study of a rare T-cell lymphoma clinically misdiagnosed as fulminant hepatic disease.
We describe a case of a 73-year-old male with a rare T-cell lymphoma that presented deceptively as progressive hepatic failure with fever, weight loss, pancytopenia, mental confusion, splenomegaly, and no lymphadenopathy. An alcoholic history supported the diagnosis of cirrhosis, but a liver biopsy was not performed. A bone marrow biopsy was considered unremarkable. Death occurred after a course of four months. Postmortem examination showed hepatic, splenic, lymph node, and marrow infiltration by characteristically sparse, isolated, bizarre, medium-to-large sized neoplastic cells with extensive hepatic centrilobular necrosis, steatosis, and predominant splenic involvement. Immunohistochemical markers indicated a T-cell lymphoma consistent with either an alpha/beta peripheral T-cell lymphoma or a gamma/delta lymphoma. Definitive immunotyping was not available. However, the pathologic features are most consistent with a gamma/delta T-cell lymphoma. This case is an example of a rare, rapidly progressive lymphoma, which is a recognized clinical entity, easily missed, and treatable. Its diagnostic consideration must be explicitly communicated to pathologists, because the isolated or sparse tumor cells in a lymph node, liver, or bone marrow biopsy may easily be mistaken for variants of megakaryocytes or histiocytes.
Schwartz
R A
RA
Department of Pathology, University of Connecticut School of Medicine, USA.
Rezuke
W N
WN
Cartun
R W
RW
Hasson
J
J
D000368:Aged; D001344:Autopsy; D001853:Bone Marrow / Q000473:pathology; D003951:Diagnostic Errors; D017809:Fatal Outcome; D006501:Hepatic Encephalopathy / Q000209:etiology; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis*; D017093:Liver Failure / Q000209:etiology*; D016399:Lymphoma, T-Cell / Q000150:complications / Q000175:diagnosis*; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000175:diagnosis*; D008297:Male; D013154:Spleen / Q000473:pathology