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Primary Pulmonary Hypertension: Its Association With Microangiopathic Hemolytic Anemia and Thrombocytopenia

Primary Pulmonary Hypertension: Its Association With Microangiopathic Hemolytic Anemia and... Abstract Four patients with primary pulmonary hypertension, microangiopathic hemolysis, and thrombocytopenia were seen at the Charleston (WVa) Area Medical Center between 1983 and 1988. Characteristic laboratory and clinical features of these patients were the following: mild anemia, reticulocytosis, low serum haptoglobin value, microangiopathic red blood cell changes on peripheral blood smear, mild to moderate thrombocytopenia, normal clotting studies (ie, prothrombin time, partial thromboplastin time, fibrinogen), negative direct Coombs' test, negative glucose water test, normal serum urea nitrogen and creatinine levels, lack of improvement of hemolysis and thrombocytopenia in response to vasodilators or calcium channel blockers, and severe plexiform lesions in the pulmonary vasculature with fibrin deposition at autopsy. The hemolysis and thrombocytopenia probably developed as a result of flow through the fibrin deposition in the plexiform lesions in the pulmonary circulation and subsequent shearing of red blood cells and platelets. (Arch Intern Med. 1991;151:1221-1223) References 1. Hughes JD, Rubin LJ. Primary pulmonary hypertension: an analysis of 28 cases and a review of the literature . Medicine. 1986;65:56-72.Crossref 2. Rich S. Primary pulmonary hypertension . Prog Cardiovasc Dis. 1988;31:205-238.Crossref 3. Stuard DI, Heusinkveld RS, Moss AJ. Microangiopathic hemolytic anemia and thrombocytopenia in primary pulmonary hypertension . N Engl J Med. 1972;287:869-870.Crossref 4. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM. Primary pulmonary hypertension . Ann Intern Med. 1987;107:216-223.Crossref 5. Wang Y, From AHL, Krivit W, Segal MA, Edwards J. Disseminated pulmonary arterial thrombosis associated with thrombocytopenia: occurrence in identical twins . Circulation. 1965;32( (suppl) ):215. 6. Pare PD, Chan-Yan C, Wass H, Hooper R, Hogg JC. Portal and pulmonary hypertension with microangiopathic hemolytic anemia . Am J Med. 1983;74:1093-1096.Crossref 7. Loyd JE, Atkinson JB, Pietra GG, et al. Heterogeneity of pathologic lesions in familial primary pulmonary hypertension . Am Rev Respir Dis. 1988;138:952-957.Crossref 8. Bull BS, Kuhn IN. The production of schistocytes by fibrin strands (a scanning electron microscope study) . Blood. 1970;35:104-111. 9. George JN, Aster RH. Thrombocytopenia due to enhanced platelet destruction by nonimmunologic mechanisms . In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA, eds. Hematology . New York, NY: McGraw-Hill International Book Co; 1990:1351-1370. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Primary Pulmonary Hypertension: Its Association With Microangiopathic Hemolytic Anemia and Thrombocytopenia

Archives of Internal Medicine , Volume 151 (6) – Jun 1, 1991

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Publisher
American Medical Association
Copyright
Copyright © 1991 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1991.00400060133023
Publisher site
See Article on Publisher Site

Abstract

Abstract Four patients with primary pulmonary hypertension, microangiopathic hemolysis, and thrombocytopenia were seen at the Charleston (WVa) Area Medical Center between 1983 and 1988. Characteristic laboratory and clinical features of these patients were the following: mild anemia, reticulocytosis, low serum haptoglobin value, microangiopathic red blood cell changes on peripheral blood smear, mild to moderate thrombocytopenia, normal clotting studies (ie, prothrombin time, partial thromboplastin time, fibrinogen), negative direct Coombs' test, negative glucose water test, normal serum urea nitrogen and creatinine levels, lack of improvement of hemolysis and thrombocytopenia in response to vasodilators or calcium channel blockers, and severe plexiform lesions in the pulmonary vasculature with fibrin deposition at autopsy. The hemolysis and thrombocytopenia probably developed as a result of flow through the fibrin deposition in the plexiform lesions in the pulmonary circulation and subsequent shearing of red blood cells and platelets. (Arch Intern Med. 1991;151:1221-1223) References 1. Hughes JD, Rubin LJ. Primary pulmonary hypertension: an analysis of 28 cases and a review of the literature . Medicine. 1986;65:56-72.Crossref 2. Rich S. Primary pulmonary hypertension . Prog Cardiovasc Dis. 1988;31:205-238.Crossref 3. Stuard DI, Heusinkveld RS, Moss AJ. Microangiopathic hemolytic anemia and thrombocytopenia in primary pulmonary hypertension . N Engl J Med. 1972;287:869-870.Crossref 4. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM. Primary pulmonary hypertension . Ann Intern Med. 1987;107:216-223.Crossref 5. Wang Y, From AHL, Krivit W, Segal MA, Edwards J. Disseminated pulmonary arterial thrombosis associated with thrombocytopenia: occurrence in identical twins . Circulation. 1965;32( (suppl) ):215. 6. Pare PD, Chan-Yan C, Wass H, Hooper R, Hogg JC. Portal and pulmonary hypertension with microangiopathic hemolytic anemia . Am J Med. 1983;74:1093-1096.Crossref 7. Loyd JE, Atkinson JB, Pietra GG, et al. Heterogeneity of pathologic lesions in familial primary pulmonary hypertension . Am Rev Respir Dis. 1988;138:952-957.Crossref 8. Bull BS, Kuhn IN. The production of schistocytes by fibrin strands (a scanning electron microscope study) . Blood. 1970;35:104-111. 9. George JN, Aster RH. Thrombocytopenia due to enhanced platelet destruction by nonimmunologic mechanisms . In: Williams WJ, Beutler E, Erslev AJ, Lichtman MA, eds. Hematology . New York, NY: McGraw-Hill International Book Co; 1990:1351-1370.

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Jun 1, 1991

References