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Agnogenic Myeloid Metaplasia: Natural History and Treatment

Agnogenic Myeloid Metaplasia: Natural History and Treatment Abstract AGNOGENIC myeloid metaplasia is a disorder of unknown etiology. Most authors consider the disease as a myeloproliferative process.1,2 It is characterized by moderate-to-massive splenomegaly with active hematopoiesis in the spleen. Peripheral blood exhibits erythrocytic changes such as normoblasts and teardrop poikilocytes. Evidence of myeloid immaturity, bizarre or giant platelets, and megakaryocytic fragments in the peripheral blood also may be seen. The bone marrow is fibrotic in most patients, but that of some patients is normocellular or hypercellular. Symptomatic patients present with manifestations of anemia, bleeding, or abdominal pain secondary to splenic infarction or a huge spleen. The basic management of this disorder consists of control of these major symptoms through the use of transfusions, androgens, busulfan (Myleran), adrenal steroids, splenic irradiation, and splenectomy. The purpose of this communication is to review the natural history of this disorder and the influence of various therapies in a large series References 1. Vaughan, J.M., and Harrison, C.V.: Leucoerythroblastic Anaemia and Myelosclerosis , J Path Bact 48:339-352 ( (March) ) 1939.Crossref 2. Dameshek, W.: Some Speculations on Myeloproliferative Syndromes, editorial , Blood 6:372-375 ( (April) ) 1951. 3. Elveback, L.: Estimation of Survivorship in Chronic Disease: The "Actuarial" Method , J Amer Statistical Assoc 53:420-440 ( (June) ) 1958. 4. Greene, T.W., et al: Splenectomy for Myeloid Metaplasia of the Spleen , New Eng J Med 248: 211-219 ( (Feb 5) ) 1953.Crossref 5. Linman, J.W., and Bethell, F.H.: Agnogenic Myeloid Metaplasia: Its Natural History and Present Day Management , Amer J Med 22:107-122 ( (Jan) ) 1957.Crossref 6. Fishman, N., and Ballinger, W.F., II: Splenectomy: For Agnogenic Myeloid Metaplasia and Myelofibrosis , Arch Surg 90:240-246 ( (Feb) ) 1965.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Agnogenic Myeloid Metaplasia: Natural History and Treatment

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1967.00300040030005
Publisher site
See Article on Publisher Site

Abstract

Abstract AGNOGENIC myeloid metaplasia is a disorder of unknown etiology. Most authors consider the disease as a myeloproliferative process.1,2 It is characterized by moderate-to-massive splenomegaly with active hematopoiesis in the spleen. Peripheral blood exhibits erythrocytic changes such as normoblasts and teardrop poikilocytes. Evidence of myeloid immaturity, bizarre or giant platelets, and megakaryocytic fragments in the peripheral blood also may be seen. The bone marrow is fibrotic in most patients, but that of some patients is normocellular or hypercellular. Symptomatic patients present with manifestations of anemia, bleeding, or abdominal pain secondary to splenic infarction or a huge spleen. The basic management of this disorder consists of control of these major symptoms through the use of transfusions, androgens, busulfan (Myleran), adrenal steroids, splenic irradiation, and splenectomy. The purpose of this communication is to review the natural history of this disorder and the influence of various therapies in a large series References 1. Vaughan, J.M., and Harrison, C.V.: Leucoerythroblastic Anaemia and Myelosclerosis , J Path Bact 48:339-352 ( (March) ) 1939.Crossref 2. Dameshek, W.: Some Speculations on Myeloproliferative Syndromes, editorial , Blood 6:372-375 ( (April) ) 1951. 3. Elveback, L.: Estimation of Survivorship in Chronic Disease: The "Actuarial" Method , J Amer Statistical Assoc 53:420-440 ( (June) ) 1958. 4. Greene, T.W., et al: Splenectomy for Myeloid Metaplasia of the Spleen , New Eng J Med 248: 211-219 ( (Feb 5) ) 1953.Crossref 5. Linman, J.W., and Bethell, F.H.: Agnogenic Myeloid Metaplasia: Its Natural History and Present Day Management , Amer J Med 22:107-122 ( (Jan) ) 1957.Crossref 6. Fishman, N., and Ballinger, W.F., II: Splenectomy: For Agnogenic Myeloid Metaplasia and Myelofibrosis , Arch Surg 90:240-246 ( (Feb) ) 1965.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Nov 1, 1967

References