A case of peripheral T-cell lymphoma presenting with acute liver failure

Abstract

A 68-year-old woman was evaluated by her primary physician for swelling and pain in the right neck. Treatment with antibiotics failed to achieve any improvement. Two weeks later, she was hospitalized to the gastroenterology service because of liver dysfunction and pneumonia. Disseminated intravascular coagulation (DIC) was diagnosed, and protease inhibitor and steroid pulse therapy were started. She was transferred to our department for further evaluation the following day. Bone marrow examination revealed hemophagocytosis and infiltration of CD3-positive cells. Multiple masses were identified in the liver. Her prothrombin time was 35.7% of the standard value 17 days from disease onset, despite improvement of DIC. She was diagnosed with acute liver failure based on the Japanese diagnostic criteria. Her general condition worsened quickly, which prevented use of chemotherapy, and she died after a total course of 19 days. Autopsy revealed atypical lymphocytes in the liver. The diagnosis was peripheral T-cell lymphoma.

HTLV-1 associated acute adult T-cell lymphoma/leukemia presenting as acute liver failure in Micronesian

“…Hepatically, ATLL cells generally localize to the periportal region, resulting in massive hepatic ischemia and eventual widespread necrosis of the hepatocytes (suspected secondary to the Schwartzman phenomena) [64] . ATLL (versus non-Hodgkin's lymphoma) liver infiltration portends a poorer prognosis, possibly secondary to the risk of sepsis-induced hepatopathy, disseminated intravascular coagulation, or hemophagocytosis syndrome—complications not present in our patient [63,65–67] …”

“…[64] ATLL (versus non-Hodgkin's lymphoma) liver infiltration portends a poorer prognosis, possibly secondary to the risk of sepsis-induced hepatopathy, disseminated intravascular coagulation, or hemophagocytosis syndrome-complications not present in our patient. [63,[65][66][67] Overall, our case highlights how in situations where etiology of liver failure remains enigmatic, the clinical team should consider a peripheral blood smear to probe possibility of a hematologic malignancy (Fig. 2).…”