The relationship of erythromonocytic leukemia to other myeloproliferative disorders

The evolution of erythromonocytic leukemia has been studied in two patients. One (Case 1) presented with DiGuglielmo's syndrome manifested by erythroid hyperplasia, dyserythropoiesis, periodic acid-Schiff-positive erythroblasts, normal heme biosynthesis and ineffective erythropoiesis. In vitro...

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Veröffentlicht in:The American journal of medicine 1973-10, Vol.55 (4), p.542-548
Hauptverfasser: Shaw, Michael T., Bottomley, Sylvia S., Bottomley, Richard H., Hussein, Khader K.
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container_end_page 548
container_issue 4
container_start_page 542
container_title The American journal of medicine
container_volume 55
creator Shaw, Michael T.
Bottomley, Sylvia S.
Bottomley, Richard H.
Hussein, Khader K.
description The evolution of erythromonocytic leukemia has been studied in two patients. One (Case 1) presented with DiGuglielmo's syndrome manifested by erythroid hyperplasia, dyserythropoiesis, periodic acid-Schiff-positive erythroblasts, normal heme biosynthesis and ineffective erythropoiesis. In vitro stimulation of the patient's bone marrow with exogenous erythropoietin demonstrated decreased responsiveness. Progressive infiltration of the bone marrow and peripheral blood with primitive monocytic cells was accompanied by an increased number of hypodiploid cells. The second patient (Case 2) presented with a picture of polycythemia vera, progressing to myelofibrosis and terminating as erythromonocytic leukemia in which a paroxysmal nocturnal hemoglobinuria-like defect was demonstrated in the erythroid cells. The leukemic cells were hyperdiploid. It is concluded that erythromonocytic leukemia may result from clonal evolution occurring in any of the myeloproliferative syndromes.
doi_str_mv 10.1016/0002-9343(73)90212-X
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One (Case 1) presented with DiGuglielmo's syndrome manifested by erythroid hyperplasia, dyserythropoiesis, periodic acid-Schiff-positive erythroblasts, normal heme biosynthesis and ineffective erythropoiesis. In vitro stimulation of the patient's bone marrow with exogenous erythropoietin demonstrated decreased responsiveness. Progressive infiltration of the bone marrow and peripheral blood with primitive monocytic cells was accompanied by an increased number of hypodiploid cells. The second patient (Case 2) presented with a picture of polycythemia vera, progressing to myelofibrosis and terminating as erythromonocytic leukemia in which a paroxysmal nocturnal hemoglobinuria-like defect was demonstrated in the erythroid cells. The leukemic cells were hyperdiploid. 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subjects Aged
Bone Marrow Cells
Bone Marrow Examination
Chromosomes - analysis
Clone Cells
Erythropoiesis
Female
Heme - biosynthesis
Hemoglobinuria, Paroxysmal - etiology
Humans
Leukemia, Erythroblastic, Acute - complications
Leukemia, Erythroblastic, Acute - diagnosis
Leukemia, Erythroblastic, Acute - etiology
Leukemia, Erythroblastic, Acute - pathology
Leukemia, Myeloid - etiology
Male
Middle Aged
Myeloproliferative Disorders - pathology
Periodic Acid
Polycythemia Vera - complications
Primary Myelofibrosis - complications
Primary Myelofibrosis - pathology
Syndrome
title The relationship of erythromonocytic leukemia to other myeloproliferative disorders
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