Thrombotic Thrombocytopenic Purpura Fails to Respond to Fresh Frozen Plasma Infusion

Ansell, Jack; Beaser, Richard S.; Pechet, Liberto

doi:10.7326/0003-4819-89-5-647

Cited by 50 publications

(21 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In these reports the increased PAIgG resolved as the patient recovered. In other studies the level of PAIgG has, however, been reported as normal (Ansell et al, 1978;Cines & Schreiber, 1979). Increased PA-C3 has also been reported (Garvey & Freedman, 1983).…”

Section: Discussionmentioning

confidence: 82%

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

et al. 1999

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 82%

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

et al. 1999

View full text Add to dashboard Cite

show abstract

“…Later on during the same year, Bukowski et al [30] introduced plasma exchange as a first-line treatment, which has significantly improved the outcome of this rare yet potentially fatal hematological disease. Several studies have shown the superiority of plasma exchange over FFP infusion in treating TTP [3,31,32]. The American Association of Blood Banks (AABB) and the American Society for Apheresis (ASFA) classifies therapeutic plasma exchange as indication category I in the treatment of TTP.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura: 24 years of experience at the American University of Beirut Medical Center

Shamseddine

Saliba

Aoun

et al. 2004

J of Clinical Apheresis

View full text Add to dashboard Cite

Thrombotic thrombocytopenic purpura (TTP) is a hematological syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent etiology. Patients may also suffer from fever in addition to neurological and renal impairment. Treatment should be initiated as soon as possible, otherwise this rare disease can be fatal. The main treatment options include therapeutic plasma exchange, fresh frozen plasma infusion, and adjuvant agents such as steroids and antiplatelet drugs. A search of patient records was carried out at the American University of Beirut Medical Center looking for patients who developed TTP over a 24-year period extending from 1980 to 2003. Relevant information was collected and analyzed. A total of 47 records were found. All presented with anemia and thrombocytopenia, 83% had neurological symptoms, 61.7% had fever and 34% had renal impairment. All patients were treated with a multimodality regimen including therapeutic plasma exchange, FFP infusion, steroids, antiplatelet agents, vincristine and others. 38 (81%) cases achieved complete remission. Out of these, 12 (31.6%) relapsed and responded to treatment. Patients who did not receive plasma exchange were more likely to relapse (P = 0.032). A second relapse was observed in 6 cases. The overall mortality rate from TTP over 24 years was 21.3%. TTP remains a fatal disease. A high index of suspicion should, therefore, always be present. Treatment options should be further developed and patients should directly be referred to tertiary care centers.

show abstract

“…A necropsy study of 50 patients with neurological manifestations of SLE showed pathological central nervous system alterations in 50% of patients. In the final stages of their disease, 14 patients presented a clinical syndrome of thrombotic thrombocytopenic purpura; in half of these pathological thrombotic thrombocytopenic purpura changes in the central nervous system (occlusion of arterioles and capillaries with platelet-fibrin thrombi, segmental subendothelial hyalin deposits, and arteriolar microaneurysms) were observed. In ten of the remaining patients, cerebral infarctions secondary to embolism of cardiac origin (Libman-Sacks endocarditis in five, chronic valvulitis in two, heart mural thrombus in two), were reported.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.

Simeón-Aznar¹,

Cuenca-Luque²,

Fonollosa-Plá³

et al. 1992

Annals of the Rheumatic Diseases

View full text Add to dashboard Cite

The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that ofthrombotic thrombocytopenic purpura.The unusual sequence and the chronological separation of the two diseases is emphasised.

show abstract

Thrombotic Thrombocytopenic Purpura Fails to Respond to Fresh Frozen Plasma Infusion

Cited by 50 publications

References 8 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

Thrombotic thrombocytopenic purpura: 24 years of experience at the American University of Beirut Medical Center

Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.

Contact Info

Product

Resources

About