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Transitional Myeloproliferative Disorder
Author(s) -
Pettit J. E.,
Lewis S. M.,
Nicholas A. W.
Publication year - 1979
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1979.tb03739.x
Subject(s) - polycythaemia , myelofibrosis , medicine , splenectomy , polycythemia vera , erythropoiesis , myeloproliferative disorders , ineffective erythropoiesis , extramedullary hematopoiesis , ruxolitinib , medullary cavity , bone marrow , surgery , spleen , anemia , haematopoiesis , stem cell , biology , genetics
S ummary . Eleven patients have been observed with clinical features of both polycythaemia vera and myelofibrosis. Detailed follow‐up and repeated haematological and isotopic investigations, including the assessment of erythropoietic distribution by 52 Fe scanning, over a 10 year period, have indicated that patients who initially present with this syndrome may remain in a steady state for several years and that this transitional syndrome does not necessarily imply an active or irreversible transformation into classical myelofibrosis. Therapy with iron, folic acid, alkylating agents, splenectomy or splenic irradiation may reduce the extramedullary component of myeloproliferation and allow occasional patients to revert to more classical polycythaemia vera. Radioactive phosphorus ( 32 P) therapy may be inappropriate in polycythaemic patients with dominant extramedullary erythropoiesis, as this form of therapy has a preferential medullary action and may selectively encourage extramedullary myeloproliferation.