Successful Remission of Thrombotic Thrombocytopenic Purpura With Rituximab in a Patient With Undifferentiated Connective Tissue Disorder

Gupta, Deepak; Roppelt, Heidi; Bowers, Brian; Kunz, Daniel A.; Natarajan, Malarvizhi; Gruber, Barry L.

doi:10.1097/rhu.0b013e31816b4ab1

Cited by 8 publications

(4 citation statements)

References 10 publications

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“…In addition to our four cases, literature search revealed two prospective trials (14,20), five retrospective case series (21-25) and 12 case reports of one or two patients (26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37). Required individual patient data was available for all reports to allow combined analysis of outcomes of interest, except for the two prospective trials and one of the larger retrospective series (14,20,24).…”

Section: Resultsmentioning

confidence: 99%

Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune‐mediated severe ADAMTS13‐deficiency: a report of four cases and a systematic review of the literature

Elliott

Heit

Pruthi

et al. 2009

European J of Haematology

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Thrombotic thrombocytopenic purpura (TTP) is a potentially life-threatening disorder that in significant proportion of cases is related to the development of autoantibodies to, and resulting severe deficiency of, the ADAMTS13 protease. However, ADAMTS13 deficiency does not account for all cases. Response to plasma exchange (PE) is seen in TTP with and without ADAMTS13 deficiency and is therefore indicated for all with a clinical diagnosis of TTP, although the pathogenesis of the latter group remains to be defined. Although the majority of cases respond to PE, a significant percent are refractory or experience relapse. Rituximab is being increasingly used off-label in this setting, but many reports do not define the pathogenesis of TTP so treated. We here report our experience with, and systematically review the published experience to date, of rituximab in management of refractory and or relapsing TTP specifically related to immune-mediated severe ADAMTS13-deficiency. In total, 73 patients met defined study inclusion criteria. The majority (approximately 95%) achieved complete remission within weeks of the first application of rituximab. The reported relapse rate was low in this patient subgroup, which carry an anticipated relapse rate of up to 60%. However, caution in interpretation of this data is needed given the relatively short median duration of follow-up of approximately 10 months. Rituximab was generally well tolerated, with few serious adverse events reported. However, three severe infectious complications were identified, including viral reactivation in keeping with black box warnings for this agent. Furthermore, reflecting the rarity of this disorder, only a relatively small number of patients have been treated and data with regards to long-term follow-up are largely based on individual case reports. Prospective studies are urgently needed to define the true efficacy and long-term safety of rituximab.

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Section: Resultsmentioning

confidence: 99%

Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune‐mediated severe ADAMTS13‐deficiency: a report of four cases and a systematic review of the literature

Elliott

Heit

Pruthi

et al. 2009

European J of Haematology

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“…It has also shown benefit as a prophylactic treatment for relapse prevention. However, follow-up on these reports is short and late relapses with TTP are possible [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. This study is a retrospective review of 12 cases of TTP who were refractory to plasma exchange transfusion and subsequently treated with rituximab at our institution.…”

Section: Introductionmentioning

confidence: 99%

Rituximab as an adjunct to plasma exchange in TTP: A report of 12 cases and review of literature

Jasti

Coyle

Gentile

et al. 2008

J of Clinical Apheresis

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Idiopathic thrombotic thrombocytopenic purpura (TTP) is caused by the production of autoantibodies against the Von Willebrand factor cleaving enzyme. This provides a rationale for the use of rituximab in this disease. We report a retrospective review of 12 patients treated with rituximab for TTP refractory to plasma exchange. Eleven patients were treated during initial presentation, and one patient was treated for recurrent relapse. Ten patients responded to treatment. Median time to response after first dose of rituximab was 10 days (5-32). Of the 11 patients treated during initial presentation, nine remain free of relapse after a median follow-up of 57+ months (1+-79+). Two patients died during initial treatment. One patient was lost to follow-up 1 month after achieving complete response. The patient treated for recurrent disease during second relapse remained disease free for 2years, relapsed and was treated again with rituximab, and was in remission for 22 months. She relapsed again, was retreated, and has now been in remission for 21+ months. We conclude that rituximab is an useful addition to plasma exchange treatment in TTP, but its exact role and dosing need to be verified in prospective studies.

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“…In patients with autoimmune TTP, rituximab was first introduced at the acute phase, in the more severe cases [30,48], and in patients with an insufficient (or suboptimal) response to conventional, TPE-based, treatment. Many single cases [49][50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67] and small series [48,[68][69][70][71][72][73][74][75][76][77][78][79][80][81][82][83][84], most often from patients with a refractory disease or experiencing multiple relapses, reported encouraging results (Table 1). TPE were continued daily and rituximab was administered immediately after a TPE (except in one study [71], in which TPE were suspended).…”

Section: Rituximab At the Acute Phasementioning

confidence: 99%

Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story

Froissart

Veyradier

Hié

et al. 2015

European Journal of Internal Medicine

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Despite a significant improvement of thrombotic thrombocytopenic purpura (TTP) prognosis since the use of plasma exchange, morbidity and mortality remained significant because of poor response to standard treatment or exacerbations and relapses. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte CD20 antigen, has shown a particular interest in this indication. Recent studies also reported strong evidence for its efficiency in the prevention of relapses. This review addresses these recent progresses and still opened questions in this topic: should rituximab be proposed in all patients at the acute phase? Should all patients benefit from a preemptive treatment? Is the infectious risk acceptable in this context?

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Successful Remission of Thrombotic Thrombocytopenic Purpura With Rituximab in a Patient With Undifferentiated Connective Tissue Disorder

Cited by 8 publications

References 10 publications

Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune‐mediated severe ADAMTS13‐deficiency: a report of four cases and a systematic review of the literature

Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune‐mediated severe ADAMTS13‐deficiency: a report of four cases and a systematic review of the literature

Rituximab as an adjunct to plasma exchange in TTP: A report of 12 cases and review of literature

Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story

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