Case of massive Splenomegaly-A report

Dr Armin Rashidi, at the University of Minnesota, Minneapolis, MN and colleagues have reported a case of massive Splenomegaly. The case has appeared in the New England Journal of Medicine.

Massive splenomegaly is defined as weight of spleen >1,000 g or largest dimension >20 cm. It is usually reported in myeloproliferative disorders, lymphoma, leukemia, visceral leishmaniasis, tropical malaria and extrahepatic (noncirrhotic) portal hypertension. Splenomegaly is a common finding in a wide-spectrum of diseases. In a retrospective study, evaluating massive splenomegaly in 2,056 patients who presented to a large university medical center from 1913 to 1995, 31% had a hematologic disorder (lymphoma, leukemia), 17% had hepatic disease (noncirrhotic portal hypertension)and 8% had infectious disease (visceral leishmaniasis, tropical malaria). Massive splenomegaly is a rarely described feature of hepatic cirrhosis.

A 35-year-old man presented to the emergency department with a 3-week history of fatigue and painful distention of the left side of his abdomen. He had a history of hepatosplenic T-cell lymphoma and had completed treatment 9 months earlier. Physical examination revealed massive enlargement of the liver and spleen, with the spleen crossing the midline and its lower margin extending into the pelvis.

A computed tomographic scan of the abdomen confirmed hepatomegaly and splenomegaly (with the spleen measuring 36 cm in its greatest dimension). The white-cell count was 17,400 per cubic millimetre (reference range, 4000 to 11,000), the hemoglobin level 3.9 g per deciliter (reference range, 13.3 to 17.7), and the platelet count 10,000 per cubic millimetre (reference range, 150,000 to 450,000).

A blood smear showed atypical mononuclear cells, intermediate to large in size, with irregular nuclear contours, moderately fine chromatin, prominent nucleoli, and some cytoplasmic blebs. Relapsed hepatosplenic T-cell lymphoma was confirmed on flow cytometry. Other causes of massive splenomegaly include chronic myeloid leukemia, myelofibrosis, hairy-cell leukemia, malaria, β-thalassemia major, and visceral leishmaniasis. Treatment with immune checkpoint blockade and opioid analgesics was initiated, but the patient died consequent to disease progression 10 weeks after presentation.