scholarly journals Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura

Nature ◽  
2001 ◽  
Vol 413 (6855) ◽  
pp. 488-494 ◽  
Author(s):  
Gallia G. Levy ◽  
William C. Nichols ◽  
Eric C. Lian ◽  
Tatiana Foroud ◽  
Jeanette N. McClintick ◽  
...  
Keyword(s):  
Gene Family ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
Adamts Gene

Normal Prostacyclin-Like Activity in Thrombotic Thrombocytopenic Purpura

1981 ◽  
Vol 46 (02) ◽  
pp. 571-571 ◽  
Author(s):  
M Pini ◽  
C Manotti ◽  
R Quintavalla ◽  
A G Dettori
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura

The Pathogenesis of Thrombotic Thrombocytopenic Purpura

1979 ◽  
Author(s):  
H. C. Kwaan
Keyword(s):  
Plasminogen Activator ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Degradation Products ◽  
Vascular Lesions ◽  
Local Defect ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Fibrin Degradation Products ◽  
Severity Of The Disease ◽  
Circulating Levels

The vascular lesions with microthrombi were studied in 12 patients with thrombotic thrombocytopenic purpura (TTP), diagnosed by the characteristic clinical and laboratory findings and confirmed histologically in each case. While defibrination was not observed, and with only minimal changes in the circulating levels of fibrinogen, fibrin degradation products and plasminogen activator, the microthrombotic lesion was invariably present. Immunofluorescent and histochemical studies indicated that both platelet and fibrin were present in the microthrombi with the platelet components dominant in many cases. Using the fibrin slide method, plasminogen activator was demonstrated in the uninvolved blood vessels but totally absent in the vessels occluded by microthrombi. in contrast, fibrinolysis is always present in the vessels afflicted with other types of thrombosis, such as the microthrombi in disseminated intravascular coagulation. Since circulating fibrinolytic activity was normal in TTP, the absence of vascular fibrinolysis is a local defect due to either inhibition by the platelet deposits or by local vascular damage. The inability of thrombolysis may explain the absence of systemic defibrination and the severity of the disease.

Thrombotic Thrombocytopenic Purpura: Mechanism for Effectiveness of Plasmapheresis

1979 ◽  
Author(s):  
J. G. Kelton ◽  
P. B. Neame ◽  
I. Walker ◽  
A. G. Turpie ◽  
J. McBride ◽  
...  
Keyword(s):  
Platelet Count ◽  
Thrombotic Thrombocytopenic Purpura ◽  
The Other ◽  
Unknown Etiology ◽  
Normal Range ◽  
Antiplatelet Antibody ◽  
Thrombocytopenic Purpura ◽  
Serious Illness ◽  
Normal Platelet ◽  
Very High

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious illness of unknown etiology. Treatment by plasmapheresis has been reported to be effective but the mechanism for benefit is unknown. We have investigated the effect of plasmapheresis in 2 patients with TTP by quantitating platelet associated IgG (PAIgG) levels prior to and following plasmapheresis. Both patients had very high levels of PAIgG at presentation (90 and A8 fg IgG/platelet respectively, normal 0-5). in both, the PAIgG levels progressively fell to within the normal range and the platelet count rose following plasmapheresis. One patient remained in remission with normal platelet counts and PAIgG levels. The other relapsed after plasmapheresis and the PAIgG level rose prior to the fall in platelet count. Plasmapheresis was repeated and resulted in normalization of both the platelet count and PAIgG level. It is suggested that plasmapheresis removes antiplatelet antibody or immune complexes which may be of etiological importance in this illness.

Influence of Thrombotic Thrombocytopenic Purpura on Depressivity, Cognitive Conduction, Quality of Life, and Resilience of Patients

2020 ◽  
Author(s):  
Falter Tanja ◽  
Böschen Sibylle ◽  
Manfred Beutel ◽  
Bernhard Lämmle ◽  
Scharrer Inge ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura

The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: Key findings at Enrolment until 2017

2020 ◽  
Author(s):  
Hendrika A. van Dorland ◽  
Magnus Mansouri Taleghani ◽  
Kazuya Sakai ◽  
Kenneth D. Friedman ◽  
James N. George ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura
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