Thrombotic Thrombocytopenic Purpura and the Hemolytic Uremic Syndrome

Brain, M. C.; Neame, Peter B.

doi:10.1055/s-2007-1005051

Cited by 29 publications

(7 citation statements)

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“…The specificity of such antibodies remains unknown and may be due to platelet adsorption of immune complexes, binding of specific antibody to platelet antigen(s), or due to non‐specific adsorption to damaged platelets. Brain & Neame (1982) postulated that soluble immune complexes resulting from the response to bacterial or viral infection may interact with platelets and cause them to aggregate and release. This is supported by the finding of circulating immune complexes in some patients with TTP ( Meister et al , 1979 ; Cofrancesco et al , 1982 ).…”

Section: Discussionmentioning

confidence: 99%

Characterization of platelet glycoproteins and platelet/endothelial cell antibodies in patients with thrombotic thrombocytopenic purpura

et al. 1999

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Platelets and sera from 12 patients with thrombotic thrombocytopenic purpura (TTP) and 12 healthy normal control subjects were examined. As determined by quantitative flow cytometry, prior to plasma exchange therapy platelet surface glycoprotein (GP) Ib levels were similar in TTP patients and normal controls (mean 20 188 and 20 226 molecules/platelet, respectively). Platelets from patients with TTP did, however, have significantly reduced levels of GPIIb/IIIa prior to plasmapheresis (mean 36 348 v 52 505 molecules/platelet in controls; P = 0.0004) and of GPIV (mean 13 321 v 26 212 molecules/platelet in controls; P = 0.0002). An increase in activated platelets, as determined by CD62 expression, was observed in 82% of patients. Increased platelet-associated immunoglobulins and/or complement was also seen in approximately 60% of the patients. In general, with return of platelet counts to normal levels following seven plasmaphereses, the above abnormalities were reversed, although often not to normal levels. Western blot analysis indicated the presence of antibodies reactive to platelet GPIV (88 kD) in 70% of pretreatment sera from patients with TTP; a similar band was observed in 80% of patient sera against microvascular endothelial cells. Immunofluorescence microscopic examination indicated the presence of antibody in pretreatment sera from patients with TTP to microvascular (73%) and large vessel (36%) endothelial cells. As measured by an indirect flow cytometric assay, pretreatment sera from 55% of patients with TTP were reactive with large vessel endothelial cells and 100% reacted with microvascular endothelial cells; reactivity was significantly greater against the microvascular endothelial cells (P = 0.0048) and was reduced following plasma exchange therapy. These results indicate abnormalities in platelet glycoprotein expression in TTP and suggest that anti-platelet and anti-endothelial cell antibodies play a role in the thrombocytopenia and vasculitis characteristic of this disorder.

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Section: Discussionmentioning

confidence: 99%

Characterization of platelet glycoproteins and platelet/endothelial cell antibodies in patients with thrombotic thrombocytopenic purpura

et al. 1999

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“…The results of the analysis that included only the 18 patients for whom ANA results were available did not differ from the results of the analysis based on the entire cohort (n ϭ 35) (Tables 3 and 4). The results of tests for specific ANA were only available for 4 of the patients reported in the literature (11,19,63,64). All 4 patients were grouped into the SLE/TTP group (patient 1, positive for anti-dsDNA and anti-Sm antibodies; patient 2, positive for anti-dsDNA and anti-Ro and anti-La antibodies; patient 3, positive for anti-Sm and anti-RNP antibodies; patient 4, positive for anti-dsDNA antibodies).…”

Section: Patient 3 Patient 3 Is a 14-year-old Girl Of Easternmentioning

confidence: 99%

Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood

Brunner¹,

Freedman²,

Silverman³

1999

Arthritis & Rheumatism

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Section: Commentsmentioning

confidence: 99%

“…TTP and HUS have several features in common [51]. They are thought to represent opposite ends of the spectrum of the same disease process and are classified together as thrombotic microangiopathy by some [51]. There is considerable overlap of the clinical and laboratory findings between these two syndromes, the difference being mainly in the age of the patients, predominance of neurologic findings, and the severity of renal involvement.…”

Section: Commentsmentioning

confidence: 99%

“…The presence or absence of uremia is of less diag nostic import, since classic cases of TTP can be just as 'uremic' as cases of hemolytic 'uremic' syndrome, as evi denced by the above case. The proposal of demonstrable laboratory differences in the coagulation abnormalities between cases of HUS and TTP may help resolve this diagnostic dilemma [53,54], Clearly, the differentiation between these two entities is of clinical import given the differences in prognosis and therapy between them [32,46,51].…”

Section: Commentsmentioning

confidence: 99%

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Renal Involvement in Patients with Thrombotic Thrombocytopenic Purpura

Eknoyan

Riggs

1986

Am J Nephrol

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A retrospective review of the records of 15 patients with thrombotic thrombocytopenic purpura (TTP) was performed to determine the spectrum of renal involvement that occurs in this disease. All cases exhibited some evidence of renal involvement, the most common manifestation of which was an abnormal urinalysis. Twelve cases (80%) had some degree of elevation of the serum urea nitrogen (SUN) or creatinine level at some time during the course of their disease. Renal involvement could be categorized into three types depending on the severity of TTP. In those cases presenting as an acute devastating illness, renal insufficiency, when present, was severe and a dominant component of the disease. In those that pursued a more protracted course, with subsequent acute exacerbations of TTP, renal insufficiency was variable and less severe than in the first group. In those presenting with a mild form of TTP, renal involvement consisted primarily of an abnormal urine sediment and azotemia that corrected rapidly following fluid replacement. A review of the literature beginning with 1966, when renal disease was established as part of the features that characterize TTP, provided 216 cases in whom sufficient data on renal involvement were recorded. Of the 168 cases where urinalysis was reported, hematuria was noted in 78% of the cases, proteinuria in 75, pyuria in 31 and cylindriuria in 24%. Of the 181 cases where the admission SUN was reported, it was higher than 20 mg/dl in 69% and greater than 60 mg/dl in 17% of the cases. The level of SUN was a significant determinant of the final prognosis of these patients.

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Thrombotic Thrombocytopenic Purpura and the Hemolytic Uremic Syndrome

Cited by 29 publications

References 85 publications

Characterization of platelet glycoproteins and platelet/endothelial cell antibodies in patients with thrombotic thrombocytopenic purpura

Characterization of platelet glycoproteins and platelet/endothelial cell antibodies in patients with thrombotic thrombocytopenic purpura

Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood

Renal Involvement in Patients with Thrombotic Thrombocytopenic Purpura

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