How I treat patients with thrombotic thrombocytopenic purpura: 2010

George, James N.

doi:10.1182/blood-2010-07-271445

Cited by 427 publications

(443 citation statements)

References 64 publications

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“…to more than 80% [13][14]. However, up to one third of patients relapse after successful treatment of an acute episode, and especially patients with initial severe ADAMTS13…”

Section: Word Countsmentioning

confidence: 99%

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

et al. 2010

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Rituximab may be used to treat patients with thrombotic thrombocytopenic purpura (TTP) refractory to plasma exchange or recurrent disease. While initial response rates are reported to be high, long-term follow-up data of patients treated with rituximab are not available to date however important to estimate the safety and benefit of this treatment. 12 patients with non-familial idiopathic TTP refractory to plasma exchange or with recurrent disease treated with rituximab between 2000 and 2008 were re-examined. The median follow-up was 49.6 months, ranging from 11 to 97 months. All patients achieved initial complete remission after application of rituximab. During follow-up, nine patients remained disease-free and three patients suffered from recurrent disease. All patients with recurrent disease responded to subsequent rituximab therapy. No long-term side effects were noted during the follow-up period. In conclusion, rituximab represents an effective second-line treatment option in relapsing or refractory TTP. Still, patients need to be closely monitored for relapses with extended follow-up.Response to Reviewers: Reviewer #11. Abstract, line 2: I think "acceptable" is not appropriate until larger studies are done. I would suggest changing to: "Rituximab may be used to treat relapsed or refractory TTP" Changes were made accordingly. 2.Abstract, line 5: While initial response rates are "reported to be" high..Changes were made accordingly.3. Abstract, line 32: "Still, patients need to be closely monitored as the relapse rate increases with longer follow-up." Change to ".monitored for relapses with extended follow-up".Changes were made accordingly. 4.Introduction, line 41: Remove "not otherwise explained"Changes were made accordingly. 5.Introduction, line 51: Revise: "Acquired idiopathic TTP is differentiated from hereditary TTP (Upshaw-Schulman syndrome)." ie how are they differentiated?The differentiation between acquired and congenital TTP follows in detail in the next paragraph: "Large progress was made in recent years in understanding the pathophysiology of TTP. Moake et al. described unusually large von Willebrand factor (VWF) multimers in the plasma of patients with relapsing acquired or congenital TTP causing intravascular platelet aggregation occluding the microvasculature [2]. Tsai et al. [3] and Furlan et al. [4] independently identified a VWF-cleaving protease (ADAMTS13) missing from the plasma of patients with congenital TTP [5] and severely deficient in patients with acquired idiopathic TTP. Mutations in the ADAMTS13 gene were shown to cause congenital TTP [6], whereas IgG autoantibodies inhibit the enzyme in most cases of the acquired form of the disease [7][8]." To avoid repetitions we left this part of the introduction as it was. If the editor has a distinct opinion to this point, we are more than willing to change it. 6.Introduction, line 12 (2nd page of introduction) and throughout the paper: "plasma exchange against fresh frozen plasma" change to ". WITH fresh frozen plasma."Changes w...

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“…to more than 80% [13][14]. However, up to one third of patients relapse after successful treatment of an acute episode, and especially patients with initial severe ADAMTS13…”

Section: Word Countsmentioning

confidence: 99%

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

et al. 2010

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“…Ez a kezelés a betegek többségében remisszió kialakulásához vezet [43]. A plazmaferézist a TTP megalapozott gyanúja esetén azonnal el kell kezdeni, és a klinikai remisszióig kell folytatni.…”

Section: Thromboticus Thrombocytopeniás Purpura (Ttp)unclassified

“…A kezelést rituximabbal (monoclonalis anti-CD20-antitest) kiegészítve csökken a remisszió kialakulásához szükséges idő, ritkábbá válnak a relapsusok [44]. Mivel a PEX-szel és hagyományos immunszuppresszió-val kezelt betegek többsége sohasem relabál, van, aki a rituximab adását csak a hagyományos kezeléssel szemben refrakter betegek esetében, illetve relapsus kezelésében ajánlja [43].…”

Section: Thromboticus Thrombocytopeniás Purpura (Ttp)unclassified

“…Még a diagnózis felállítása előtt került sor thrombocyta adására súlyos ADAMTS13-hiányos betegeik 61%-ában, és köz-leményük szerint egy vagy több thrombocytatranszfúzió adása nem járt nagyobb halálozással, és a neurológiai szö -vődmények sem voltak gyakoribbak. Ezért súlyos thrombocytopenia vagy vérzés esetén szükség lehet thrombocytatranszfúzió adására [43,45].…”

Section: Thromboticus Thrombocytopeniás Purpura (Ttp)unclassified

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Thrombocytopenia

Gadó

Domján

2014

Orvosi Hetilap

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Thrombocytopenia alatt a vérlemezkék számbeli csökkenését értjük. Ez a vérzékenység leggyakoribb oka. Klinikailag petechiák, purpurák megjelenése, nyálkahártyavérzés jellemzi. Előfordulhat azonban akár életet fenyegető, emésztő-rendszert vagy központi idegrendszert érintő vérzés is. A haemostaseologiai laboratóriumi vizsgálatok közül a megnyúlt vérzési idő utal thrombocytaeredetű vérzékenységre. Kialakulásában vagy a csontvelői csökkent megakaryopoesis, vagy a fokozott thrombocytapusztulás, illetve a kettő kombinációja játszik szerepet. Thrombocytopenia észlelésekor a teendő az ok tisztázása. Ebben a csontvelő és a perifériás vérkenet vizsgálata mellett a háttérben álló feltételezett alapbetegség célzott diagnosztikája lehet segítségünkre. A kezelés részint az alapbetegséget célozza, ré-szint a hiányzó komponens -a thrombocyta -pótlását jelenti. Utóbbi a thrombocyták aktiválódása és felhasználó-dása által előidézett kórképek esetén azonban szigorúan tilos, mivel a kórfolyamat progresszióját idézheti elő. Orv. Hetil., 2014, 155(8), 291-303. Kulcsszavak: thrombocytopenia, vérzékenység, csontvelő, immunthrombocytopenia, terhességi thrombocytopenia ThrombocytopeniaThrombocytopenia means low platelet count. This is the most frequent cause of bleeding abnormalities. Petechias, purpuras, mucosal bleeding are typical clinical fi ndings. Severe, even life threatening gastrointestinal or intracranial bleeding may also occur. Diagnostic laboratory fi nding is the prolonged bleeding time. There are several causes of thrombocytopenia. The major mechanisms for a reduced platelet count are decreased production and increased destruction of platelets, or both. The major task is to reveal the underlying cause. Examination of the bone marrow and the peripheral blood smear can be helpful as well as special diagnostics of the assumed disease. Therapy targets the underlying disease, and also involves platelet transfusion. However, in case of diseases with increased platelet activation and consumption, platelet transfusion is forbidden because it may lead to aggravation of the pathologic process.Keywords: platelet, bleeding disorders, bone marrow, immune-thrombocytopenia, gestation thrombocytopenia Gadó, K., Domján, Gy. [Thrombocytopenia]. Orv. Hetil., 2014, 155(8), 291-303. (Beérkezett: 2013 elfogadva: 2013. december 27.) Rövidítések aPTI = aktivált parciális tromboplasztin; DIC = disszeminált intravascularis coagulopathia; DITP = gyógyszer indukálta immunthrombocytopenia; FDA = Food and Drug Administration (USA); HIT = heparin indukálta thrombocytopenia; HPA = human platelet antigen; ITP = immunthrombocytopeniás purpura; IVIG = intravénás immunglobulin; LMVH = alacsony molekulatömegű heparin; MDS = myelodysplasiás szindróma; TTP = thromboticus thrombocytopeniás purpura Thrombocytopeniáról beszélünk, ha a thrombocytaszám alacsony. Általában a normálérték alsó határát (<150×109/L) tekintjük küszöbnek, azonban a 100× 109/L alatti érték elfogadása mellett is szólnak érvek [1]. Ezek közé tartozik az, hogy így elkerülhetjük a teendőt...

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“…conditions [18][19][20]. Patients with persistent anti-ADAMTS13 antibodies or undetectable levels of ADAMTS13 are at a higher risk for recurrence [16].…”

Section: Transplantationmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura

Saifan¹

2013

J Blood Disord Transfus 2012

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How I treat patients with thrombotic thrombocytopenic purpura: 2010

Cited by 427 publications

References 64 publications

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

Long-term follow-up of idiopathic thrombotic thrombocytopenic purpura treated with rituximab

Thrombocytopenia

Thrombotic Thrombocytopenic Purpura

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