Rituximab als effektive Therapie bei schwerster thrombotisch thrombozytopenischer Purpura (TTP)

Illner, N.; Wolf, Günter

doi:10.1055/s-0029-1244819

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…This concept is translated into therapy as patients are treated with rituximab, a therapeutic immunomodulatory and B cellblocking antibody [49]. Treatment with rituximab was successful for several patients and resulted in a reduction of plasma cells in the circulation and a reduction of autoantibody titers [51,52].…”

Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 99%

Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura?

Zipfel

Wolf²,

John

et al. 2011

Pediatr Nephrol

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Thrombotic microangiopathies (TMA) represent a spectrum of related disorders associated with newly formed thrombi that block perfusion and thus affect the function of either renal or neurological organs and tissue. Recent years have seen a dramatic development in the field of TMA and for the two major forms hemolytic uremic syndrome (HUS) and thrombocytopenic purpura (TTP), new genetic causes and also autoimmune forms have been identified. This development indicates a similar pathophysiology and suggests that the two acute disorders are based on common principles. HUS is primarily a kidney disease and TTP also develops in the kidney and at neurological sites. In HUS thrombi formation is likely due to a deregulated complement activation and inappropriate platelet activity. In TTP thrombi formation occurs because of inappropriate processing of released multimers of von Willebrand Factor (vWF). Defining both the similarities and the unique features of each disorder will open up new ways and concepts that are relevant for diagnosis, for therapy, and for the prognostic outcome of kidney transplantations. Here we summarize the most relevant topics and timely issues that were presented and discussed at the 4th International Workshop on Thrombotic Microangiopathies held in Weimar in October 2009 (www.hus-ttp.de).

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Section: Thrombotic Thrombocytopenic Purpuramentioning

confidence: 99%

Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura?

Zipfel

Wolf²,

John

et al. 2011

Pediatr Nephrol

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“…Daher erfolgte neben dem initialen Einsatz von Kortikosteroiden [22], deren primärer Nutzen in Bezug auf die Hemmung der Antikörperbildung gegen ADAMTS13 umstritten ist, die viermalige Applikation von Rituximab zur Inhibition einer weiteren ADAMTS13-Antikörper-Produktion. Der gegen das CD20-Oberflächenan-tigen von B-Lymphozyten gerichtete monoklonale Antikörper Rituximab wurde aufgrund der hohen Mortalität und der hohen Rückfallquote von 40-50% [21] der Erkrankung in kleineren Serien und Fallberichten in den letzten Jahren trotz "off-label use" [8,23] in das Therapieregime der TTP implementiert [15,[24][25][26][27][28][29][30][31][32][33]. Die Arbeitsgruppe von Fakhouri zeigte, dass bei Patienten, die kein Ansprechen auf die Plasmapherese und die Gabe von Kortikosteroiden sowie persistierend hohe Antikörperlevel aufwiesen, die viermalige Rituximabgabe im Abstand von 1 Woche zu einer kompletten Remission der TTP führte [26].…”

Section: Diskussionunclassified

Schwerste thrombotisch-thrombozytopenische Purpura (TTP) nach H1N1-Vakzinierung

et al. 2010

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“…Due to severe hemodynamic compromise by the time a provisional diagnosis of TTP was made, plasma exchange was not possible. Early treatment with Rituximab 375 mg/m 2 once a week for 1 month has been found to be beneficial [7,8]. Unfortunately, it did not change the outcome in this case.…”

Section: An `Unusual Presentation Of Ttpmentioning

confidence: 66%

Thrombotic thrombocytopenic purpura: An unusual presentation with intracranial bleed

Tahaseen¹,

Kirti²,

Kumar³

2021

Indian J Case Reports

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Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet-rich thrombi. We present the case of a 44-year-old lady who presented with severe thrombocytopenia and anemia that did not respond to repeated transfusions and steroids. Non-contrast computed tomography scan of the brain revealed an intracranial bleed. Schistocytes were seen on the peripheral blood smear. A provisional diagnosis of TTP was made. Plasmapheresis could not be done due to her deteriorating hemodynamic status. She succumbed to her illness in spite of the best possible efforts. This case highlights the need for keeping a high index of suspicion for TTP as early diagnosis and prompt initiation of plasmapheresis are crucial for preventing death.

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Rituximab als effektive Therapie bei schwerster thrombotisch thrombozytopenischer Purpura (TTP)

Abstract: Severe autoantibody TTP can be successfully treated by administering rituximanb, an anti-CD20 antibody, in addition to the standard treatment with plasmapheresis.

Cited by 5 publications

References 10 publications

Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura?

Novel developments in thrombotic microangiopathies: is there a common link between hemolytic uremic syndrome and thrombotic thrombocytic purpura?

Schwerste thrombotisch-thrombozytopenische Purpura (TTP) nach H1N1-Vakzinierung

Thrombotic thrombocytopenic purpura: An unusual presentation with intracranial bleed

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