An Idiotypic Natural Autoantibody Network in Hemolytic Uremic Syndrome (Hus).† 1020

Gomez, Henry F.; Diaz-Gonzalez, Vicente A.; Rowe, Peter C.; Lior, H.; McLaine, Peter N.; Orrbine, Elaine; Caceres, Enrique; Cleary, Thomas G.

doi:10.1203/00006450-199604001-01042

Cited by 1 publication

(3 citation statements)

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“…These findings may have significance, however, in considering a possible kidney transplant. Overall, the data suggest that HUS, like TTP [7][8][9]22] and heparin-induced thrombocytopenia, has an immunological basis.…”

Section: Discussionmentioning

confidence: 88%

“…The slightly elevated serum isoenzyme Platelet aggregating antibodies in HUS Rock et al 543 Protein blot of platelet lysate probed with patient's plasma. Lysate prepared from normal human platelets (8 mg protein/lane) was separated on 7.5% sodium dodecyl sulfate-polyacrylamide gel electrophoresis reducing gel, transferred to a polyvinylidene fluoride membrane membrane and blotted with normal (NP) or patient's plasma samples (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12) collected during different days of hospitalization (see Table 1). Kaleidoscopes prestained protein standards (BioRad) were run in the last lane (Std).…”

Section: Discussionmentioning

confidence: 99%

“…The endothelial damage triggers a cascade of events that results in microvascular lesions with platelet-rich and fibrin-rich microthrombi that occlude arterioles and capillaries. Several studies have demonstrated that HUS is mediated by immune complexes [7][8][9][10], and antibodies against endothelial cells have been reported [8][9][10]. HUS and thrombotic thrombocytopenic purpura (TTP) represent different ends of what is probably the same disease continuum.…”

Section: Introductionmentioning

confidence: 99%

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Identification of plasma antifibrin/fibrinogen antibodies in a patient with hemolytic uremic syndrome

Rock¹,

Kolajova²,

Faught³

et al. 2006

Blood Coagulation &Amp; Fibrinolysis

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We investigated a patient with atypical hemolytic uremic syndrome without diarrhea to determine the presence of antibodies and the specificity of the related antigens. The patient experienced repeated episodes of hemolytic uremic syndrome. She is dialysis dependent. von Willebrand factor (vWF), vWF multimers, platelet aggregation, ADAMTS-13 activity and platelet immunoblots were determined. During acute episodes vWF increased threefold, with unusually large vWF multimers on two occasions. Platelet aggregation was normal but the plasma caused spontaneous aggregation of normal platelets. Reactivity was removed after absorption with protein A. Protein blotting against platelet and microvascular endothelial cells showed strong and persistent reactivity against antigens of 200 and 55 kDa. Two-dimensional immunoblots of the whole platelet proteome and incubation with plasma identified strong immunoreactivity with two target spots in the 55-kDa area. Mass spectroscopy confirmed the target as beta-fibrin, molecular weight 50.73 kDa, isoelectric point 7.95, with MASCOT scores of 859 and 750, Two years after presentation another band was detected at 66 kDa and identified as the alpha subunit of fibrin. This patient's plasma contained a platelet-aggregating factor that was removed by immunoglobulin absorption. She developed antibodies against the alpha and beta subunits of fibrin/fibrinogen.

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Section: Discussionmentioning

confidence: 88%