TROMBOTİK TROMBOSİTOPENİK PURPURA: DİRENÇLİ İKİ OLGUNUN BİLDİRİMİ VE LİTERATÜRÜN GÖZDEN GEÇİRİLMESİ

Trombotik trombositopenik purpura TTP nadir görülen bir hematolojik hastalıktır. Ancak, acilen tanı koyulup tedavi edilmezse, ölümcül sonuçları olan bir hematolojik acildir. TTP’in en etkin tedavisi hastaların %80’inden fazlasında kür elde edilebilen plazmaferezdir. Dirençli TTP’si olan hastalarda immunsupresif tedavi kullanılabilir. Rituksimab otoimmun ve malign hastalıklarda kullanılan önemli bir immunmodulator tedavidir. Biz burada, konvensiyonel tedaviye dirençli olan ve rituksimab ile başarıyla tedavi edilen iki hastayı sunduk

THROMBOTIC THROMBOCYTOPENIC PURPURA: A REPORT OF TWO RESISTANCE CASES AND REVIEW OF THE LITERATURE

Thrombotic thrombocytopenic purpura TTP is a rare hematological disease, but it is a hematological emergency with fatal consequences if not promptly diagnosed and appropriately treated. Plasmapheresis is the most effective therapy of TTP through which more than 80% of patients have been cured. Immunosuppressive therapy is used for patients who have resistance TTP. Rituximab is an important immune modulator therapy in certain autoimmune and malignant disorders. Herein, we report two patients with resistance TTP to conventional therapy who were successfully treated by rituximab. Using rituximab as part of the standard therapy may be more effective to prevent potential mortality and morbidity of TTP than standard approach

___

  • ) Crawley JTB, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program 2013;2013:292–
  • ) Vesely SK, George JN, Lämmle B, Studt J-D, Alberio L, El- Harake MA, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003;102(1):60–8.
  • ) Tsai H-M. Pathophysiology of thrombotic thrombocyto penic purpura. Int J Hematol 2010;91(1):1–19.
  • ) Tsai H-M. Is severe deficiency of ADAMTS-13 specific for thrombotic thrombocytopenic purpura? Yes. J Thromb Haemost 2003;1(4):625–31.
  • ) Terrell DR, Williams LA, Vesely SK, Lämmle B, Hovinga JAK, George JN. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost 2005;3:1432–6.
  • ) Kiss JE. Thrombotic thrombocytopenic purpura: recognition and management. Int J Hematol 2010;91(1):36–
  • )Booth KK, Terrell DR, Vesely SK, George JN. Systemic infections mimicking thrombotic thrombocytopenic purpura. Am J Hematol 2011;86(9):743–51.
  • ) George JN. Evaluation and management of patients with thrombotic thrombocytopenic purpura. J Intensive Care Med ;22(2):82–91. ) Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991;325(6):393–7.
  • ) Tsai H-M. Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency. Hematol Oncol Clin North Am 2007;21(4):609–32, v.
  • ) Petryk M, Grossbard ML. Rituximab therapy of B-cell neoplasms. Clin Lymphoma 2000;1(1):186–94; discussion –6.
  • ) Cerny T, Borisch B, Introna M, Johnson P, Rose AL. Mechanism of action of rituximab. Anticancer Drugs 2002;13 Suppl 2:S3–10.
  • ) Boye J, Elter T, Engert A. An overview of the current clinical use of the anti-CD20 monoclonal antibody rituximab. Ann Oncol 2003;14(4):520–35.
  • ) Czuczman MS, Grillo-López AJ, White CA, Saleh M, Gordon L, LoBuglio AF, et al. Treatment of patients with low- grade B-cell lymphoma with the combination of chimeric anti-CD20 monoclonal antibody and CHOP chemotherapy. J Clin Oncol 1999;17(1):268–76.
  • ) Micallef IN, Kirk A, Norton A, Foran JM, Rohatiner AZ, Lister TA. Peripheral T-cell lymphoma following rituximab therapy for B-cell lymphoma. Blood 1999;93(7):2427–8.
  • ) Cobo-Ibáñez T, Loza-Santamaría E, Pego-Reigosa JM, Marqués AO, Rşa-Figueroa I, Fernández-Nebro A, et al. Efficacy and safety of rituximab in the treatment of non-renal systemic lupus erythematosus: A systematic review. Semin Arthritis Rheum 2014;44(2):175-185.
  • ) Wendler J, Burmester GR, Sörensen H, Krause A, Richter C, Tony H-P, et al. Rituximab in patients with rheumatoid arthritis in routine practice (GERINIS): 6-year results from a prospective, multicentre, non-interventional study in 2,484 patients. Arthritis Res Ther 2014;16(2):R80.
  • ) Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991;325(6):393–7.
  • ) Ziman A, Mitri M, Klapper E, Pepkowitz SH, Goldfinger D. Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution’s experience and review of the literature. Transfusion 2005;45(1):41–9.
  • ) George JN. Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura. Am J Hematol 2012;87 Suppl 1:S88–91.
  • ) Chai W, Chaudhry A, Rabinowitz AP. Successful management of thrombotic thrombocytopenic purpura in a Jehovah’s Witness without plasma exchange. J Clin Apher ;doi:10.1002/jca.21325.
  • ) Theodoropoulos G, Arampatzi S, Diamantidis M, Perifanis V, Diza E, Kaiafa G. Successful treatment with Rituximab in a patient with life-threatening resistant Thrombotic Thrombopenic Purpura. Hippokratia 2012;168(4):388.
  • ) Reddy PS, Deauna-Limayo D, Cook JD, Ganguly SS, Blecke C, Bodensteiner DC, et al. Rituximab in the treatment of relapsed thrombotic thrombocytopenic purpura. Ann Hematol 2005;84(4):232–5.
  • ) Ahmad A, Aggarwal A, Sharma D, Dave HP, Kinsella V, Rick ME, et al. Rituximab for treatment of refractory/relapsing thrombotic thrombocytopenic purpura (TTP). Am J Hematol 2004;77(2):171–6.
  • ) Heidel F, Lipka DB, von Auer C, Huber C, Scharrer I, Hess G. Addition of rituximab to standard therapy improves response rate and progression-free survival in relapsed or refractory thrombotic thrombocytopenic purpura and autoimmune haemolytic anaemia. Thromb Haemost ;97(2):228–33. ) Cataland SR, Jin M, Lin S, Kraut EH, George JN, Wu HM. Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura. Am J Hematol 2008;83(12):911–
  • ) Nosari A, Bernuzzi P, Corneo R, Pungolino E, Muti G, Rossi V, et al. Late response to cyclosporine in refractory thrombotic thrombocytopenic purpura. Int J Hematol ;76(3):284–6. ) Honda K, Hidaka S, Kobayashi S. Successful treatment with cyclosporine of thrombotic thrombocytopenic purpura refractory to corticosteroids and plasma exchange. Ther Apher Dial 2011;15(2):215–7.
İzmir Eğitim ve Araştırma Hastanesi Tıp Dergisi-Cover
  • ISSN: 1305-5151
  • Başlangıç: 1995
  • Yayıncı: İzmir Bozyaka Eğitim ve Araştırma Hastanesi
Sayıdaki Diğer Makaleler

LARİNGEAL NÖROFİBROM: OLGU SUNUMU

Enver VARDAR, Tolgahan ÇATLI, Tulu KEBAT, Asuman ARGON, Çağrı ÇELİK, Didem ERSÖZ

İNFERTİL ERKEKLERDE KROMOZOMAL ANOMALİ VE POLİMORFİZM SONUÇLARI: RETROSPEKTİF BİR ÇALIŞMA

Gülüzar Arzu TURAN, Esra Bahar GÜR, Fatma ESKİCİOĞLU, Betül UĞUZ, İşın SÖZER, Şaban ADAKAN, Musa SARAÇOĞLU, İşın KAYA

SOL VENTRİKÜL FONKSİYONLARI İLERİ DERECEDE BOZULMUŞ AORT YETMEZLIĞI HASTALARINDA CERRAHİ: AORT KAPAK REPLASMANI İÇİN ÖNEMLİ BİR SORUN

Yuksel BESİR, Levent YİLİK, Orhan GOKALP, Sahin BOZOK, Banu LAFCİ, İsmail YUREKLİ, Ufuk YETKİN, Ali GURBUZ

BEL AĞRISI İLE PREZENTE OLAN D VİTAMİNİ EKSİKLİĞİNE BAĞLI OSTEOMALAZİ

As Low Back PAIN, Nesibe DOĞAN, Şeniz Akçay YALBUZDAĞ, Altınay Göksel KARATEPE, Taciser KAYA, İlker ŞENGÜL

HEPATİK ARTER ANEVRİZMASI: NADİR BİR OLGU SUNUMU

Harun ARSLAN, Ayşe ARSLAN, Sebahattin ÇELİK, Edip GÖNÜLLÜ

ACİL SERVİSTE TÜKENMİŞLİK SENDROMU

Özgür DURAN, Fulya Yilmaz DURAN, Gülbin AYDOĞDU

UNUTULMUŞ DJ STENTLERDE ENKRUSTASYON OLUŞUMU: ON YIL UNUTULMUŞ DJ STENT, OLGU SUNUMU

Süleyman MİNARECİ

ÜRETERAL GİRİŞ KILIFI KULLANILMADAN GERÇEKLEŞTİRİLEN RETROGRAD İNTRARENAL CERRAHİ SONUÇLARIMIZ

Tarık YONGUÇ, Serkan YARIMOĞLU, İsmail BASMACI, Volkan ŞEN, Tansu DEĞİRMENCİ, Bülent GÜNLÜSOY, İbrahim Halil BOZKURT

OTOİMMUN TİROİDİT OLGULARININ İDRAR İYOT DÜZEYLERİ İLE DİYETLERİNDEKİ TUZ İYOT DÜZEYLERİ ARASINDAKİ İLİŞKİ

Halis Çetin DEMİRCAN, Ümmühan Öztürk KADIOĞLU, Proje NO

TROMBOTİK TROMBOSİTOPENİK PURPURA: DİRENÇLİ İKİ OLGUNUN BİLDİRİMİ VE LİTERATÜRÜN GÖZDEN GEÇİRİLMESİ

Mehmet CALAN, Ferda BİLGİR, Gulsum CAGLİYAN, Can OZLU, Arif Yüksel, Oktay BİLGİR