scholarly journals Thrombotic Thrombocytopenic Purpura— Coagulation Parameters in Twelve Patients

Blood ◽  
1973 ◽  
Vol 42 (4) ◽  
pp. 499-507 ◽  
Author(s):  
Eric A. Jaffe ◽  
Ralph L. Nachman ◽  
Clarence Merskey
Keyword(s):  
Blood Coagulation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Disseminated Intravascular Coagulation ◽  
Temporal Relationship ◽  
Primary Factor ◽  
Thrombocytopenic Purpura ◽  
Intravascular Coagulation ◽  
Coagulation Abnormalities ◽  
Heparin Administration

Abstract Twelve patients with thrombotic thrombocytopenic purpura have been studied with emphasis on the changes in blood coagulation and the occurrence of disseminated intravascular coagulation (DIC). No coagulation abnormalities were found in six patients. Minimal abnormalities were present in three patients and major abnormalities compatible with DIC were present in three patients. There was little temporal relationship between the coagulation abnormalities and the clinical pathological course except for the extent of hemolysis. In addition, heparin administration in four patients had no effect on the course of the disease. Intravascular coagulation does not appear to be a primary factor in the pathogenesis of thrombotic thrombocytopenic purpura and the changes suggestive of DIC, when present, may well result from, rather than cause, the hemolytic anemia.

scholarly journals Thrombotic Thrombocytopenic Purpura: Report of a Case With Disseminated Intravascular Platelet Aggregation

Blood ◽  
1973 ◽  
Vol 42 (5) ◽  
pp. 805-814 ◽  
Author(s):  
P. B. Neame ◽  
J. Lechago ◽  
E. T. Ling ◽  
A. Koval
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Disseminated Intravascular Coagulation ◽  
Endothelial Lining ◽  
Electron Microscopic ◽  
Thrombocytopenic Purpura ◽  
Intravascular Coagulation ◽  
Coagulation Study ◽  
Platelet Aggregates ◽  
Vascular Occlusions

Abstract The nature and etiology of the vascular occlusions encountered in thrombotic thrombocytopenic purpura (TTP) have been subject to controversy for a number of years. Disseminated platelet thrombosis has been suggested in the earlier literature, although later views have favored fibrin thrombi resulting from vascular damage or disseminated intravascular coagulation (DIC). The serial coagulation study and the lightand electron-microscopic findings in a case of TTP are described here. The multiple vascular occlusions were due to the presence of densely packed platelet aggregates in which a variable quantity of fibrin was present. Less commonly, loose platelet aggregates were noted. Fibrin under the endothelial lining was occasionally observed in relationship to the vascular occlusion and was thought to be secondary to the release of various substances from aggregating platelets. The serial coagulation study and the histologic examination of tissues showed no evidence of disseminated intravascular coagulation. This case shows that the occlusions observed in TTP can be due to disseminated intravascular platelet aggregation in the absence of DIC. Although TIP might be of variable etiology, it is felt that cases showing disseminated intravascular platelet aggregation should be distinguished from DIC in order to plan therapy on a rational basis.

Microangiopathic and Vascular Disorders

2020 ◽  
Author(s):  
Nathan T. Connell
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Diagnostic Criteria ◽  
Antiphospholipid Syndrome ◽  
Disseminated Intravascular Coagulation ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Vascular Disorders ◽  
Intravascular Coagulation ◽  
Presenting Symptoms

The thrombotic microangiopathies are characterized by microangiopathic hemolytic anemia and thrombocytopenia and can be classified as autoimmune, drug induced, complement mediated, and infectious/other. Reaching a definitive diagnosis for these disorders can be challenging due to the similarity of presenting symptoms and laboratory findings. Specific disorders described in this review include thrombotic thrombocytopenic purpura, the hemolytic-uremic syndrome, thrombotic microangiopathies of pregnancy (including preeclampsia and HELLP syndrome), disseminated intravascular coagulation, and antiphospholipid syndrome. Vascular disorders that lead to hematologic abnormalities are also discussed. Figures show the major classifications of the thrombotic microangiopathies; ADAMTS13 activity in normal and thrombotic thrombocytopenic purpura plasma; a fragmented red blood cell (arrow), also known as a schistocyte or helmet cell; major considerations in the initial treatment of thrombotic thrombocytopenic purpura and options for refractory patients as well as treatment considerations after discontinuation of plasma exchange; and a diagram of the complement pathway showing regulatory proteins as well as the site of action for the monoclonal antibody eculizumab. Tables list medications associated with thrombotic thrombocytopenia purpura, diagnostic criteria for HELLP, major classifications and examples of the causes of disseminated intravascular coagulation, diagnostic criteria for the antiphospholipid syndrome, vascular purpuras, and criteria for diagnosing hereditary hemorrhagic telangiectasia. This review contains 5 highly rendered figures, 10 tables, and 74 references Key words: anemia, hereditary hemorrhagic telangiectasia,thrombotic microangiopathies, thrombotic Thrombocytopenic Purpura, von Willebrand factor

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