The Nature of the Vascular Lesion in Thrombotic Thrombocytopenic Purpura.

Kwaan, Hau C.; Gallo, Guillermo; Potter, Elizabeth; Cutting, Hunter O.; Stanzler, Robert M.

doi:10.7326/0003-4819-68-5-1169_3

Cited by 29 publications

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“…In adult TTP, the pathogenic lesion is the formation of von Willebrand rich platelet microthrombi with a small amount of fibrin [23]. The persistence of platelet plugs with associated fibrin led Kwaan and colleagues [24,25] to postulate that hypofibrinolysis may be contributory to TTP. Kwaan et al [24,25] have reported that tissue plasminogen activator (tPA) activity is absent from the occluded vessels of patients with TTP but present in uninvolved vessels.…”

Section: Discussionmentioning

confidence: 99%

“…The persistence of platelet plugs with associated fibrin led Kwaan and colleagues [24,25] to postulate that hypofibrinolysis may be contributory to TTP. Kwaan et al [24,25] have reported that tissue plasminogen activator (tPA) activity is absent from the occluded vessels of patients with TTP but present in uninvolved vessels. By contrast, the vessels of patients with intravascular thrombosis due to conditions other than TTP had a normal tPA staining pattern.…”

Section: Discussionmentioning

confidence: 99%

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Plasminogen activator inhibitor (PAI-1) antigen levels in primary TTP and secondary TTP post-bone marrow transplantation

et al. 1998

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Our objectives were to measure and compare plasminogen activator inhibitor levels (PAI-1) in primary adult thrombotic thrombocytopenic purpura (TTP) and in secondary TTP associated with bone marrow transplantation (BMT)-TTP. PAI-1 antigen levels were measured by an enzyme linked immunosorbent assay on platelet poor plasma samples obtained from patients at the time of diagnosis of the TTP disorder and from a group of normal volunteers. The samples were frozen at -70 degrees C. Patients with TTP secondary to bone marrow transplantation had their grade determined by percentage fragmented cells and lactate dehydrogenase levels. The primary TTP samples were contributed by investigators in the multi-institutional North American TTP Group, and the bone marrow transplant samples were obtained from an adult bone marrow transplant program. Nineteen patients with adult TTP, and 47 patients with bone marrow transplant-TTP were evaluated. Of the latter, 14 had Grade 2, 13 had Grade 3, and 20 had Grade 4 BMT-TTP. PAI-1 levels were elevated compared to control volunteers in both primary adult TTP and BMT-TTP, P < 0.001. Levels did not differ from normal in Grade 2 BMT-TTP (median = 16 ng/ml; quartiles = 9-20). PAI-1 levels were similar in primary TTP (median = 32 ng/ml; quartiles = 25-51) and Grade 3 BMT-TTP (median = 35 ng/ml; quartiles = 19-48 ng/ml), P = 0.7. However, PAI-1 levels were significantly higher in Grade 4 BMT-TTP (median = 83 ng/ml; quartiles = 60-143) than Grade 3 BMT-TTP, and primary TTP, P < 0.001. PAI-1 levels are high in primary TTP and secondary bone marrow transplant-TTP (Grades 3-4). In contrast, normal levels are seen in Grade 2 BMT-TTP, which is a self-limited disorder. Therefore, high PAI-1 levels may contribute to hypofibrinolysis in the pathogenesis of primary TTP and of moderate to severe TTP (Grades 3-4) following bone marrow transplantation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Plasminogen activator inhibitor (PAI-1) antigen levels in primary TTP and secondary TTP post-bone marrow transplantation

et al. 1998

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“…A recent study was carried out in my laboratory on 12 patients, seen over the past 12 years. It is an extension of similar work done earlier [46]. The diagnosis was based on the patient's clinical data of fever, fluctuating neurologic symptoms, hemolytic anemia, thrombocytopenia, renal failure with characteristic bone marrow hyperplasia of the erythroid percursors and megakaryocytes ( Table 1).…”

Section: Ttpmentioning

confidence: 99%

“…The nature of the subendothelial hyaline material has also been investigated. Immunofluorescent studies revealed that the material reacted to antibodies against fibrin, gamma globulin and beta-1-C-globulin [46,51]. Though electron microscopic studies showed the presence of fibrin-like material lacking the specific cross-striation, most workers accept the contention that fibrin was present.…”

Section: The Nature Of the Hyaline Thrombimentioning

confidence: 99%

“…This is not unexpected if the thrombi consist primarily of platelets. The finding of impaired fibrinolysis has led to the use of urokinase therapy in one patient with success, but to disastrous visceral hemorrhage in others [46]. In the presence of widespread petechial hemorrhagic lesions and of marked thrombocytopenia, thrombolytic agents are dangerous and contraindicated.…”

Section: The Significance Of Impaired Fibrinolysis In Ttpmentioning

confidence: 99%

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The Pathogenesis of Thrombotic Thrombocytopenic Purpura

Hc¹

2008

Semin Thromb Hemost

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Chronic relapsing thrombotic thrombocytopenic purpura and antiphospholipid antibodies: A report of two cases

1997

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We report on 2 cases of chronic relapsing thrombotic thrombocytopenic purpura, in which anti-phospholipid antibodies were also found. The first patient was felt to have the antiphospholipid antibody syndrome, while the second patient had anti-phospholipid antibodies without clinical manifestations of the anti-phospholipid antibody syndrome. We discuss chronic relapsing thrombotic thrombocytopenic purpura and the anti-phospholipid antibody syndrome. Furthermore, we introduce the possibility of an association between chronic relapsing thrombotic thrombocytopenic purpura and the presence of anti-phospholipid antibodies. Am. J. Hematol. 54:155-159, 1997 ᮊ 1997 Wiley-Liss, Inc.Key words: thrombotic thrombocytopenic purpura; anti-phospholipid antibodies; antiphospholipid antibody syndrome INTRODUCTION acute onset of transient dysarthria and bilateral upper extremity clumsiness and numbness. Her physical examiThe anti-phospholipid antibody syndrome (APS) has nation was significant only for a systolic murmur. Laborabeen defined as the presence of anti-phospholipid anti-tory studies revealed a hematocrit of 25% with numerous bodies or lupus anticoagulant in association with certain schistocytes on the peripheral blood smear, platelet count clinical events, including recurrent arterial or venous of 49,000/l, lactate dehydrogenase (LDH) of 1,090 U/ thromboses and recurrent fetal loss [1,2], and it has been l (normal range, 80-235 U/l), normal prothrombin time treated successfully with prolonged anticoagulation [3,4]. (PT) and partial thromboplastin time (PTT), and serum Thrombotic thrombocytopenic purpura (TTP) is a severe, creatinine of 1.5 mg/dl. Direct Coombs' test and an antimultisystemic disorder characterized by microangio-nuclear antibody test were negative. A CT scan of the head pathic hemolytic anemia, thrombocytopenia, neurologic was unremarkable. A transesophageal echocardiogram manifestations, and often fever and renal dysfunction [5], revealed a solitary 4 ϫ 6 mm pedunculated echogenic which has been effectively treated with plasma transfu-structure on the left atrial surface of the posterior mitral sion and/or plasmapheresis [6]. The majority of patients valve leaflet. Cultures of blood were repeatedly negative. experience only a single episode of TTP, but relapses do An atrial myxoma was suspected, and the patient underoccur. Occasionally, chronic relapsing TTP is observed. went surgical excision of two masses from the posterior This entity can be defined as frequent episodes of TTP leaflet of the mitral valve and one from the anterior leaflet. varying in severity at regular intervals [7]. The interval On pathologic review these were found to be thrombi between TTP relapses is usually a few weeks. An associa-with ossification and calcification. The patient did well tion between TTP and APS or anti-phospholipid antibod-postoperatively and was discharged with a hematocrit of ies has not been clearly established. We describe a patient 25% and a platelet count of 98,000/l. with both chronic relapsing TTP and A...

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The Nature of the Vascular Lesion in Thrombotic Thrombocytopenic Purpura.

Cited by 29 publications

References 0 publications

Plasminogen activator inhibitor (PAI-1) antigen levels in primary TTP and secondary TTP post-bone marrow transplantation

Plasminogen activator inhibitor (PAI-1) antigen levels in primary TTP and secondary TTP post-bone marrow transplantation

The Pathogenesis of Thrombotic Thrombocytopenic Purpura

Chronic relapsing thrombotic thrombocytopenic purpura and antiphospholipid antibodies: A report of two cases

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