MARGOT S. KRUSKALL, M.D.; SIGMUND A. WEITZMAN, M.D.; THOMAS P. STOSSEL, M.D.; NANCY HARRIS, M.D.; STEPHEN H. ROBINSON, M.D.
A relation between lymphoma and autoimmune neutropenia, unlike autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, has not previously been well documented. We report a patient with a disorder presenting as autoimmune agranulocytosis, splenomegaly, and infiltration of the hepatic sinusoids by lymphocytes. Antineutrophil antibodies were present. Over a 2½-year period, the illness progressed to an aggressive, poorly differentiated lymphocytic lymphoma with terminal liver failure and fibrosis. Peripheral blood lymphocyte markers identified the tumor as a proliferation of T-cells of the helper class. A review of previous literature disclosed other reports of similar patients who had neutropenia, a lymphoproliferative illness, and hepatic disease. Our case is representative of a previously unrecognized syndrome characterized by autoimmune neutropenia in the setting of a lymphoproliferative disorder of T cells, with a predilection for liver involvement.
MARGOT S. KRUSKALL, SIGMUND A. WEITZMAN, THOMAS P. STOSSEL, NANCY HARRIS, STEPHEN H. ROBINSON. Lymphoma with Autoimmune Neutropenia and Hepatic Sinusoidal Infiltration: A Syndrome. Ann Intern Med. 1982;97:202–205. doi: 10.7326/0003-4819-97-2-202
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Published: Ann Intern Med. 1982;97(2):202-205.
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