Assessment of patients with thrombotic thrombocytopenic purpura

Yıl 2010, Cilt: 15 Sayı: 1, 15 - 20, 15.01.2013

Cengiz Demir Abdullah Altıntaş Semir Paşa M. Ali Kaplan Yüksel Küçükzeybek Orhan Ayyıldız

Öz

 

Abstract. Thrombotic thrombocytopenic purpura (TTP) is a severe microvascular occlusive thrombotic microangiopathy characterized by systemic platelet aggregation, organ ischemia, profound thrombocytopenia, and fragmentation of erythrocytes. Unexplained occurrence of thrombocytopenia and anemia should prompt immediate consideration of the diagnosis and evaluation of peripheral blood smear for evidence microangiopathic hemolytic anemia. Excellent remission and survival rates were achieved by therapeutic plasma exchange. We reviewed characteristics and response rates to plasmapheresis of our TTP patients. A total of 25 cases were diagnosed. The parameters of hemoglobin and platelet were analyzed at presentation, as well as the number of plasmapheresis sessions and adjunctive treatment given. We found a response rate of 80 percent to plasma exchange. Response was better in 23 patients who presented with idiopathic TTP. Response was poor in patients with TTP secondary to underlying metastatic carcinoma. Two patients relapsed and one of the relapsed patients died. Plasmapheresis is mandatory and effective for primary TTP. Plasmapheresis may not be effective in all instances, especially if TTP is secondary to underlying disseminated cancer.

 

Key words: Thrombotic thrombocytopenic purpura, therapeutic plasmapheresis

 

 

 

Anahtar Kelimeler

-

Kaynakça

• 1. Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589-600.
• 2. Terrel DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpurahemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost 2005; 3: 1432- 1436.
• 3. Levine SP. Thrombotic thrombocytopenic purpura and other form of nonimmunologic platelet destruction. In: Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B (eds). Wintrobe’s clinical hematology. 2th ed. Philadelphia, Lippincott Williams & Wilkins 2004; 1555-1564.
• 4. Rock GA. Management of thrombotic thrombocytopenic purpura. Br J Haematol 2000; 96: 1223-1229.
• 5. Ridolfi LR, Bell WR. Thrombotic thrombocytopenic purpura: report of 25 cases and review of the literature. Medicine 1981; 60: 413- 428.
• 6. Vesely SK, George JN, Lämmle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:relation to presenting features and clinical outcomes in prospective cohort of 142 patients. Blood 2003; 102: 60-68.
• 7. George JN, Vesely SK. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: Diagnosis and treatment. Cleve Clin Med 2001; 68: 857-878.
• 8. Mc Crae KR, Sadler JE, Cines D. Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. In: Hoffman R, Benz EJ, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, McGlave P (eds). Hematology Basic Principles and Practice. 4th ed. Philadelphia, Elsevier Churchill Livingstone 2005; 2287-2304.
• 9. Zheng XL, Kaufman RM, Goodnough LT, Sadler E. Effect of plasma exchange on plasma ADAMTS 13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 2004; 103: 4043-4049.
• 10. George JN. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354: 1927-1935.
• 11. George JN, Rizvi MA. Thrombocytopenia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U (eds). Williams Hematology. 6th ed. McGraw-Hill Companies 2001; 1495-1539.
• 12. George JN, Sadler JE, Lämmle B. Platelets: thrombotic thrombocytopenic purpura. Am Soc Hematol 2002; 315-334.
• 13. Pereira A, Mazzara R, Monteagudo J, et al. Thrombotic thrombocytopenic purpura/ hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange. Ann Hematol 1995; 70: 319-323.
• 14. Hwang WYK, Chai LYA, Ng HJ, Tan PHC. Therapeutic plasmapheresis for the treatment of the thrombotic thrombocytopenic purpura-haemolytic ureamic syndromes. Singapore Med J 2004; 45: 219-223. 15. Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost 2005; 3: 2420-2427.
• 16. Guvenc B, Unsal C, Gurkan E, et al. Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. Transfus Apheresis Sci 2004; 31: 17-20.
• 17. Cookson S, Krueger ML, Bennett RM. Fulminant thrombotic thrombocytopenic purpura in a patient with the limited form of scleroderma: succesful outcome using plasma exchange. J Rheumatol 1991; 18: 900-901.
• 18. Ter Borg EJ, Houtman PM, Kallenberg CGM, et al. Thrombocytopenia and hemolytic anemia in apatient with mixed connective tissue due to thrombotic thrombocytopenic purpura. J Rheumatol 1988; 15: 1174-1177.
• 19. Ellingson TL, Wilske K, Aboulafia DM. Case report: thrombotic thrombocytopenic purpura in a patient with polymyositis: therapeutic importance of early recognition and discussion of pathogenic mechanisms. Am J Med Sci 1992; 303: 407-410.
• 20. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine 1998; 77: 195-207.
• 21. Chang JC, Naqvi T. Thrombotic thrombocytopenic purpura associated with bone marrow metastasis and secondary myelofibrosis in cancer. The Oncologist 2003; 8: 375-380.
• 22. Pirrotta MT, Bucalossi A, Forconi F, et al. Thrombotic thrombocytopenic purpura secondery to an occult adenocarcinoma. The Oncologist 2005; 10: 299-300.
• 23. Robson MG, Abbs IC. Thrombotic thrombocytopenic purpura following hemicolectomy for colonic carcinoma. Nephrol Dial Transplant 1997; 12: 198-199.
• 24. Fontana S, Gerritsen HE, Hovinga K, et al. Microangiopathic haemolytic anaemia in metastasizing tumours is not associated with a severe deficiency of the von Willebrand factorcleaving protease. Br J Haematol 2001; 113: 100- 102.
• 25. Lee JL, Lee JH, Kim MK, et al. A case of bone marrow necrosis with thrombotic thrombocytopenic as an manifestation of occult colon cancer. Jpn J Clin Oncol 2004; 34: 476-480.
• 26. Shumak KH, Rock GA, Nair RC& the Canadian apheresis group. Late relapses in patients succesfully treated for thrombotic thrombocytopenic purpura. Ann Intern Med 1995; 122: 569-572.