Comparison of low fixed dose versus standard-dose rituximab to treat thrombotic thrombocytopenic purpura in the acute phase and preemptively during remission

Reddy, Manasa; Hofmann, Sandra L.; Shen, Yu; Nagalla, Srikanth; Rambally, Siayareh; Usmani, Amena; Sarode, Ravindra

doi:10.1016/j.transci.2020.102885

Cited by 9 publications

(5 citation statements)

References 15 publications

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“…According to the clinical guidelines, the standard dose of rituximab was 375 mg/m 2 for a week for four doses. In addition, a small prospective trial [19] and retrospective studies [20] reported the curative effects of the low dose of rituximab (100-200 mg/time), but this treatment method was not widely accepted by clinical guidelines.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

Yao

Zhong

et al. 2021

Preprint

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Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy with clinical quintuple symptoms, including fever, thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, and renal insufficiency. TTP onset in children is rare, and the percentage of acute TTP with these five symptoms at the same time is <10%. In this study, we reported a typical case of TTP onset in a child with clinical quintuple symptoms.

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Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

Yao

Zhong

et al. 2021

Preprint

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“…The standard dosing for rituximab is 375 mg/m 2 given weekly for a total of four doses, which is recommended for both initial iTTP episodes and the acute phase of relapsing episodes. Emerging evidence for the efficacy of low dose rituximab (100 mg-200 mg/per dose) comes from a small prospective trial [161] and retrospective studies [162] but it has not yet been widely incorporated into standard practice.…”

Section: Immune Suppressionmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

Sukumar

Lämmle

Cataland

2021

JCM

133

132

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Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTTP) is often detected in childhood or during pregnancy. iTTP occurs more often in women and is potentially lethal without prompt recognition and treatment. Front-line therapy includes daily plasma exchange with fresh frozen plasma replacement and immunosuppression with corticosteroids. Immunosuppression targeting ADAMTS13 autoantibodies with the humanized anti-CD20 monoclonal antibody rituximab is frequently added to the initial therapy. If available, anti-VWF therapy with caplacizumab is also added to the front-line setting. While it is hypothesized that refractory TTP will be less common in the era of caplacizumab, in relapsed or refractory cases cyclosporine A, N-acetylcysteine, bortezomib, cyclophosphamide, vincristine, or splenectomy can be considered. Novel agents, such as recombinant ADAMTS13, are also currently under investigation and show promise for the treatment of TTP. Long-term follow-up after the acute episode is critical to monitor for relapse and to diagnose and manage chronic sequelae of this disease.

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“…Ma már alkalmaznak fix dózis terápiát is igen széles dózistartományban (100 mg-1000 mg). A kis dózisú rituximab (100 mg hetente 1-szer, 4 héten át) hatékonyságát több friss vizsgálat is alátámasztja [59,60], saját magunk a Dél-pesti Centrumkórházban évek óta eredményesen alkalmazzuk. Ha a beteg állapota megengedi, a beadást követő napon plazmacserét nem végzünk, helyette FFP infúziót adunk kb.…”

Section: Ajánlás 25 a Corticosteroid Steroid Terápia Leépítésénél A T...unclassified

Egészségügyi szakmai irányelv – A thrombotikus thrombocytopeniás purpura (TTP) és a haemolytikus uraemiás syndroma (HUS) kezeléséről

2022

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Az egészségügyi szakmai irányelv készítése során a szerzői függetlenség nem sérült. Az egészségügyi szakmai irányelvben foglaltakkal a fent felsorolt egészségügyi szakmai kollégiumi tagozatok dokumentáltan egyetértenek.Az irányelvfejlesztés egyéb szereplői Betegszervezet(ek) tanácskozási joggal: Nem vettek részt.Egyéb szervezet(ek) tanácskozási joggal: Nem vettek részt. Szakmai társaság(ok) tanácskozási joggal:Nem vettek részt. Független szakértő(k):Nem vettek részt. ORVOSI HETILAP2023 ■ 164. évfolyam, Szupplementum 1 Érintett ellátottak köre:Minden thrombotikus thrombocytopeniás purpurában és haemolyticus uraemiás syndromában szenvedő betegÉrintett ellátók köre Szakterület:

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Comparison of low fixed dose versus standard-dose rituximab to treat thrombotic thrombocytopenic purpura in the acute phase and preemptively during remission

Cited by 9 publications

References 15 publications

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management

Egészségügyi szakmai irányelv – A thrombotikus thrombocytopeniás purpura (TTP) és a haemolytikus uraemiás syndroma (HUS) kezeléséről

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