Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen

Asada, Yujiro; Sumiyoshi, Akinobu; Hayashi, Tohru; Suzumiya, Junji; Kaketani, Kazutoshi

doi:10.1016/0049-3848(85)90180-x

Cited by 281 publications

(166 citation statements)

References 22 publications

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“…21 In accordance with the current pathophysiologic hypothesis, 19 we found a severe reduction of ADAMTS-13 proteolytic activity, but we could not detect inhibiting antibodies. Thus, we have shown that IgM and IgG antibodies are present in plasma from a TTP patient and that these antibodies react with ADAMTS-13, but do not inhibit its activity.…”

Section: Resultssupporting

confidence: 74%

Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura

Scheiflinger

Knöbl

Trattner

et al. 2003

Blood

212

192

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Acquired thrombotic thrombocytopenic purpura (TTP) has been linked to severe deficiency of ADAMTS-13 activity caused by autoantibodies inhibitory to ADAMTS-13. We report data on a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely used fluid phase activity assay. With a newly developed enzyme-linked immunosorbent assay, using immobilized recombinant ADAMTS-13, we found high titers of IgM and IgG antibodies that bound to ADAMTS-13, but did not neutralize protease activity. These autoantibodies probably influenced the half-life of ADAMTS-13 or its binding to the endothelial cell surface, thereby compromising ADAMTS-13 activity in vivo. Given that ADAMTS-13 may interact physiologically with various receptors or ligands, the occurrence, distribution, and the epitope mapping of nonneutralizing antibodies will be an important area for future research. (Blood. 2003;102:3241-3243)

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Section: Resultssupporting

confidence: 74%

Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura

Scheiflinger

Knöbl

Trattner

et al. 2003

Blood

212

192

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“…In contrast to disseminated intravascular coagulation (DIC), the venules are conspicuously uninvolved and there is minimal fibrin deposition and less prominent vascular injury in TTP. 28 In both platelets and endothelial cells, vWF is stored as large multimers which, following release into the circulation, are cleaved by a metalloprotease into smaller forms. 3,4 The highly multimeric forms of vWF are extremely efficient mediators of platelet aggregation.…”

Section: Discussionmentioning

confidence: 99%

Porcine hematopoietic cell xenotransplantation in nonhuman primates is complicated by thrombotic microangiopathy

Bühler

Goepfert

Kitamura

et al. 2001

Bone Marrow Transplant

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Summary:Thrombotic microangiopathy (TM) is a serious complication of bone marrow transplantation (BMT) that resembles thrombotic thrombocytopenic purpura (TTP). In attempting to achieve hematopoietic cell chimerism in the pig-to-baboon model, we have observed TM following infusion of high doses (Ͼ10 10 cells/kg) of porcine peripheral blood mobilized progenitor cells (PBPC) into baboons. We performed investigations to analyze the pathobiology of this TM and to test therapeutic interventions to ameliorate it. PBPC were obtained by leukapheresis of cytokine-stimulated swine. The initial observations were made in two baboons that underwent a non-myeloablative regimen (NMR) prior to PBPC transplantation (TX) (group 1). We then studied three experimental groups. Group 2 (n = 2) received NMR without PBPC TX. Group 3 (n = 2) received PBPC TX alone. Group 4 (n = 6) received NMR + PBPC TX combined with prostacyclin, low-dose heparin, methylprednisolone, and cyclosporine was replaced by anti-CD40L mAb in five cases. Baboons in groups 1 and 3 developed severe thrombocytopenia (Ͻ10 000/mm 3 ), intravascular hemolysis with schistocytosis (Ͼ10/high powered field (hpf)), increase in plasma lactate dehydrogenase (LDH) (2500-9000 U/l), transient neurologic changes, renal insufficiency, and purpura. Autopsy on two baboons confirmed extensive platelet thrombi in the microcirculation, and, similar to clinical BMT-associated TM/TTP, no unusually large vWF multimers or changes in vWF protease activity were observed in the plasma of baboons with TM. In group 2, self-limited thrombocytopenia occurred for 10-15 days following NMR. Group 4 baboons developed thrombocytopenia (Ͻ20 000/mm 3 ) rarely requiring plat-

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“…In BMT recipients a relationship has been suggested with cytomegalovirus (CMV) or fungal infection, graft-versus-host disease (GVHD) and cyclosporin A (CsA) toxicity. [1][2][3][4][5] However, some cases have been reported without any evidence of other complications or after autologous grafting (ABMT). 6,7 Moreover, the pretransplant conditioning regimen may play a role in the pathogenesis of TMA.…”

Section: Discussionmentioning

confidence: 99%

Severe thrombotic microangiopathy: an infrequent complication of bone marrow transplantation

Iacopino

Pucci

Arcese

et al. 1999

Bone Marrow Transplant

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Summary:Thrombotic microangiopathy (TMA) usually occurs during the first weeks following transplantation in the setting of systemic infections or graft-versus-host reaction. However, some cases without any evidence of other complications or after autologous transplantation have been reported. Transplant-associated TMA (BMT-TMA) incidence ranges from 0% to 74%, possibly due to different diagnostic criteria. The GITMO Group provided the opportunity to retrospectively study 4334 consecutive Italian patients who received bone marrow transplants (1759 allogeneic and 2575 autologous BMT), during the 1985-1995 period. The present report focuses on patients with severe TMA requiring specific treatment. We identified nine cases of TMA as a complication of allogeneic BMT (0.51%), whereas three patients developed the syndrome after ABMT (0.13%); four of the 12 patients were not receiving CsA at the time of TMA onset. Finally, it is noteworthy that TMA occurred in seven patients as a late complication (up to 90 days after BMT). Despite intensive treatment, five of the seven patients with thrombotic thrombocytopenic purpura died. One death was observed among the five cases with hemolytic uremic syndrome.

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Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen

Cited by 281 publications

References 22 publications

Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura

Nonneutralizing IgM and IgG antibodies to von Willebrand factor–cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura

Porcine hematopoietic cell xenotransplantation in nonhuman primates is complicated by thrombotic microangiopathy

Severe thrombotic microangiopathy: an infrequent complication of bone marrow transplantation

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