Intravenous Gamma Globulin in Refractory Thrombotic Thrombocytopenic Purpura

Nosari, Annamaria; Muti, Giuliana; Busnach, G; Cantoni, Silvia; Strinchini, Alberto; Morra, Enrica

doi:10.1159/000203797

Cited by 22 publications

(9 citation statements)

References 11 publications

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“…Failure of plasma exchange to result in benefit should prompt a re‐evaluation for an alternative explanation for clinical manifestations. If none is found, subsequent treatments are largely based on anecdotal reports and include cytotoxic agents such as vincristine (34–36) or cyclophosphamide (37,38), immunomodulation with high‐dose γ‐globulin (39–41) or cyclosporin (42), or splenectomy (43–45).…”

Section: Treatmentmentioning

confidence: 99%

Post-operative thrombotic thrombocytopenic purpura: A review

Naqvi

Baumann

Chang

2004

International Journal of Clinical Practice

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Post-operative thrombotic thrombocytopenic purpura (TTP) is a recently recognised life-threatening clinical syndrome with considerable similarity to classic TTP in presentation and response to early treatment with plasma exchange. To date, 29 cases of TTP associated with surgery have been reported. The majority of cases have complicated vascular surgeries, with a few cases seen following gastrointestinal or orthopaedic procedures. Characteristically, patients develop microangiopathic haemolytic anaemia and consumptive thrombocytopenia 5 to 9 days following surgery with variable presence of fever, impaired renal function and altered mental status. The pathogenesis of post-operative TTP is speculative but may involve the release of large amounts of high-molecular-weight von Willebrand factor (vWF) multimers due to endothelial damage resulting from surgery in the setting of marginal levels of vWF-cleaving enzyme. The myriad of common post-surgical complications that may present with clinical manifestations similar to TTP may result in confusion with the potential for delay in the initiation of life-saving plasma-exchange therapy. It is important that physicians be alert to the phenomenon of post-operative TTP so that prompt recognition and treatment will prevent serious morbidity or mortality.

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Section: Treatmentmentioning

confidence: 99%

Post-operative thrombotic thrombocytopenic purpura: A review

Naqvi

Baumann

Chang

2004

International Journal of Clinical Practice

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“…However, if patients do not respond within the first 5-7 days, an alternative therapeutic strategy should be tried. Various immunosuppressive approaches, including high-dose immunoglobulin, splenectomy, combination chemotherapy, and vincristine (VCR), have been reported as effective salvage treatment for patients with refractory TTP [1,[4][5][6]10]. In this study we report the disease characteristics and treatment results from a series of eight patients suffering from refractory TTP who were given VCR as salvage treatment.…”

Section: Introductionmentioning

confidence: 99%

Vincristine as salvage treatment for refractory thrombotic thrombocytopenic purpura

Ferrara¹,

Copia²,

Annunziata³

et al. 1999

Annals of Hematology

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Vincristine (1.4 mg/m(2) on day 1, followed by 1 mg on days 4 and 7) was given to eight patients with thrombotic thrombocytopenic purpura (TTP) who were refractory to plasma exchange (n=4) or plasma infusion (n=4). Seven of eight patients (87%) achieved a complete response; one was refractory to treatment and died within a few weeks. After a median follow-up of 50 months, all responding patients are alive and well. Two patients relapsed and were successfully retreated with vincristine. Toxicity was mild, consisting of two episodes of leukopenia and one of autonomic neuropathy leading to paralytic ileus in a patient aged 70 years. We conclude that vincristine is highly effective in the treatment of patients suffering from refractory TTP, with negligible toxicity.

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“…Although platelet transfusion may be beneficial in clinically serious bleeding, it is not routinely recommended (18–21). Cases that are resistant to usual therapy are treated with immunomodulation (22) with vincristine (23, 24) cyclophosphamide (25), cyclosporine or splenectomy (26). …”

Section: Discussionmentioning

confidence: 99%