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The Spleen in Myelofibrosis With Myeloid Metaplasia
Mayo Clin Proc, January 2003, Vol 78
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The Spleen in Myelofibrosis With Myeloid Metaplasia AYALEW TEFFERI, MD,* AND DAVID M. NAGORNEY, MD† *Divsion of Hematology and Internal Medicine and †Division of Gastroenterologic and General Surgery, Mayo Clinic College of Medicine, Rochester, Minn
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he differential diagnosis of massive splenomegaly, operationally defined as a palpable spleen that extends below the transumbilical line, includes myelofibrosis with myeloid metaplasia (MMM),1 chronic myeloid leukemia, lymphoma, hairy cell leukemia, hyperreactive malarial splenomegaly, kala-azar (visceral leishmaniasis), Gaucher disease, splenic cystic lymphangiomatosis, systemic lupus erythematosus, and sarcoidosis. In MMM, splenomegaly is secondary to extramedullary hematopoiesis.2 Progressive splenomegaly in MMM is associated with mechanical discomfort, early satiety, episodes of severe left upper quadrant pain from splenic infarcts, profound cachexia, increased red blood cell transfusion requirement, thrombocytopenia, and complications of portal hypertension. Spleen-directed management in MMM includes chemotherapy (initial choice), sur-
Mayo Clin Proc. 2004;79:503
gical removal of the spleen (in drug-resistant cases), and splenic irradiation (in poor surgical candidates). In a retrospective study of 223 consecutive splenectomies in MMM, the median spleen mass was 2700 g (range, 380-7735 g).3 Splenectomy in MMM does not alter the natural history of the disease but benefits a proportion of patients with the aforementioned spleenrelated symptoms. 1. 2.
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Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255-1265. Koch CA, Li C-Y, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003;78:12231233. Tefferi A, Mesa RA, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients. Blood. 2000; 95:2226-2233.
© 2004 Mayo Foundation for Medical Education and Research
For personal use. Mass reproduce only with permission from Mayo Clinic Proceedings.
Running Head
503
Medical Images
The Spleen in Myelofibrosis With Myeloid Metaplasia AYALEW TEFFERI, MD,* AND DAVID M. NAGORNEY, MD† *Divsion of Hematology and Internal Medicine and †Division of Gastroenterologic and General Surgery, Mayo Clinic College of Medicine, Rochester, Minn
T
he differential diagnosis of massive splenomegaly, operationally defined as a palpable spleen that extends below the transumbilical line, includes myelofibrosis with myeloid metaplasia (MMM),1 chronic myeloid leukemia, lymphoma, hairy cell leukemia, hyperreactive malarial splenomegaly, kala-azar (visceral leishmaniasis), Gaucher disease, splenic cystic lymphangiomatosis, systemic lupus erythematosus, and sarcoidosis. In MMM, splenomegaly is secondary to extramedullary hematopoiesis.2 Progressive splenomegaly in MMM is associated with mechanical discomfort, early satiety, episodes of severe left upper quadrant pain from splenic infarcts, profound cachexia, increased red blood cell transfusion requirement, thrombocytopenia, and complications of portal hypertension. Spleen-directed management in MMM includes chemotherapy (initial choice), sur-
Mayo Clin Proc. 2004;79:503
gical removal of the spleen (in drug-resistant cases), and splenic irradiation (in poor surgical candidates). In a retrospective study of 223 consecutive splenectomies in MMM, the median spleen mass was 2700 g (range, 380-7735 g).3 Splenectomy in MMM does not alter the natural history of the disease but benefits a proportion of patients with the aforementioned spleenrelated symptoms. 1. 2.
3.
503
Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255-1265. Koch CA, Li C-Y, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc. 2003;78:12231233. Tefferi A, Mesa RA, Nagorney DM, Schroeder G, Silverstein MN. Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients. Blood. 2000; 95:2226-2233.
© 2004 Mayo Foundation for Medical Education and Research
For personal use. Mass reproduce only with permission from Mayo Clinic Proceedings.