Yıldız YILDIRMAK, Ayten ARCASOY, Zümrüt UYSAL

Çocukta kronik immun trombositopenik purpura ( 63 hastada tedavi sonuçları )

1977-93 yılları arasında -Kliniğimizde izlenen 63 kronik immun trombositopenik purpuralı (ITP) hasta retrospektif olarak değerlendirildi. Hastaların 45'inde (%71,42) sadece deri ve mukoza kanamaları varken geri kalanlarda ayrıca epistaksis, dişeti kanaması ve genitoüriner sistem kanamaları eşlik ediyordu. Steroid tedavisi 43 hastada oral steroid, 77 hastada yüksek doz metil prednizolon (YDMP) şeklinde yapıldı. Oral steroid uygulanan hastaların 6'sında (%14), YDMP uygulanan 77 hastanın sadece birinde (%5,3) kalıcı yanıt elde edildi. 16 hastada intravenöz immunglobulin (IVIC) uygulandı ve hiçbirinde kalıcı yanıt elde edilmedi. 8 hastaya splenektomi uygulandı, 4'ünde kalıcı yanıt elde edildi. Hiçbir hastada spontan remisyon gözlenmedi. Kanamalarla veya santral sinir sistemi kanaması ile kaybedilen hasta olmadı. Oral steroid, YDMP ve IVIG'in kalıcı ve geçici remisyon yönünden birbirlerine üstünlükleri olmadığı, splenektominin kalıcı remisyon açısından en etkin tedavi şekli olduğu sonucuna varıldı.

Chronic immune thrombocytopenic purpura in children

Chronic Immune Thrombocytopenic Purpura in Children Sixty-three chronic ITP. cases followedin our department between 1977-93 were evaluated retrospectively. Skin and mucous membrane bleedings were in 45(71.42%) of patients and the rest had presented with epistaxes, gum and genitourinary bleeding. Steroid treatment was given orally 1-2 mg/kg/day in 43 patients and as H DM P in 17. Complete sustained remission was obtanied in 6(14%) of the oral steroid and 1(5.3%) of HDMP patients. IVIC was given to 16 patients but hone achieved sustained remission. Eight patients were splenectomized and 4 had complete remission eventually. HDMP and IVIC were superior to oral steroids with respect to transient response. There were no spontaneous remissions and hone of the patients were lost due to hemorrhages or CNS bleeding. IVIG and HDMP were more effective to raise platelet counts in a short time, whereas oral steroids were more effective in obtaining sustained remission. Early initiation of steroids were observed to be a better predictor to achive sustained remission. Splenectomy should be performed in patients who are unresponsive to other treatment schemes have higher risk of bleeding. Finally, we conclude that, in respect to transient and sustained remission; oral steroid, YDMP and IVIC has no superiority to each other and splenectomy is the best treatment whe sustained remission is considered.

PDF

___

1. Aronis S, Platokouki H, Mitsika A. Seventeen years of expe-rience with chronic idiopathic thrombocytopenic pur-pura in childhood. Pediatr Hemat Oncol 1994,T 1: 487-498.
2. Breardsley DS. Platelet abnormalities in infancy and child-hood. İn: Nathan DC, Oski FA. Hematology of İnfancy and Childhood 4th ed. WB Saunders Company, 1993:1561-1604.
3. Bithell TC. Thrombocytopenia caused by immunologic pla-telet destruction. İn: Lee GR, Bithell TC Foerster). Wint-robe's Clinical Hematology 9th ed. Lea&Febiger 1993:1329-1355.
4. Lusher JM, Emami A, Ravindranath Y, VVarrier Al. İdiopat-hic thrombocytopenic purpura in children. The Am J Pediatr Hemat Oncol 1984;6.:149-157.
5. Tamary H, Kaplinsky C, Levy I, Cohen IJ, Yaniv I et al. Chro-nic childhood thrombocytopenic purpura: long t'erm fol-lovv up Açta Pediatr. 1994; 83:931-934.
6. Russel EC, Maurer HM. Alternatives to splenectomy in the management of chronic idiopathic purpura in childho-od. Tha Am J Pediatr Hemat Oncol 1984; 6:175-179.
7. Branehög I, Kutti J, VVeinfeld A. Platelet survival and plate-let production in idiopathic thrombocytopenic purpura. Brit J Hemat 1974;27:127-143.
8. Berlucci S, Han ZC, Caen JP. Studies of in vitro megacaryo-poesis in adult immune thrombocytopenic purpura (ITP). EurJ Haematol 1991;47:86-90.
9. Mcmilian R, Luiken GA, Levy R, Yelenosky R, Longmire RL. Antibody against megacaryocytes in idiopathic throm-bocytopenic purpura. JAMA 1978; 239:2460-2462.
10. Pizzi F, Carrara PM, Aldeghi A, Eridani S. Immunoflorescen-ce of megacaryocytes in the thrombocytopenic purpu-ras. Blood 1966; 27:521-526.
11. Berchtold P, McMillan R. Therapy of chronic idiopathic thrombocytopenic purpura in adults. Blood 1989; 74:2309-2317.
12. Kiefel V, Spaeth P, Mueller C. Immune thrombocytopenic purpura autoimmune or immune complex disease?. Brit J Haematol 1986; 64:57-68.
13. Blanchette VS, Luke B, Andrew M, Sommerville-Nielsen S, Barnard D et al. The J Pediatr 1993; 123:989-995.
14. Bussel JB, Fitzgerald-Pedersen J, Feldman C. Alternation of tvvo doses of ıntravenous gammaglobulin inthe mainte-nance treatment of patients with immune thrombocyto-penic purpura. Am J Hematol 1990; 33:184-188.
15. Hoff J, Ritchey AK. Pulse methylprednisolone therapy t'or acute childhood idiopathic thrombocytopenic purpura. The ) Pediatr 1988; 113:563-566.
16. Özsoylu Ş. Bolus Methylprednisolone therapy in chronic idiopathic thrombocytopenic purpura in children.
17. Hollenberg JP, Subak LL, Ferry JJ, Bussel JB. Cost-effective-ness of splenectomy versus intravenouns gammaglobu-lin in treatment of chronic immune thrombocytopenic purpura in chilhood. The J Pediatr 1988; 112:530-539.
18. Naouri A, Feghali B, Chabal J, Boulez J, Dechavanne M et al. Results of splenectomy for idiopathic thrombocyto-penic purpura. Açta Haematol 1993; 89:200-203.
19. Yehuda DB, Cillis S, Eldor A. Clinical and therapeutic expe-rience in 712 Israeli patients vvith idiopathic throm-bocytopenic purpura. Açta Haematol. 1994; 91:1-6.
20. VValker RW, VValker W. İdiopathic thrombocytopenia, initi-al illness and long term follovv up Arch Dis Child. 1984; 59:316-322.
21. Buelli M, Cortelazzo S, Viero P, Minetti B, et al. Danazol for the treatment of idiopathic thrombocytopenic purpura. Açta Haemat 1985; 74:97-98.
22. Mazzuconi MC, Francessoni M, Falcione E, Ferrari A, et al. Danazol therapy in refractory chronic immune throm-bocytopenic purpura. Açta Haemat 1987; 77:45-47.
23. Khalifa as, Tolba KA, El-Alfy MS, et al. idiopathic throm-bocytopenic purpura in Egyptian children. Açta Haema-. tol 1993; 90:125-129.
24. Steven M, Simmons BS, Charles AM, Hassan MY, et al. İdi-apothic thrombocytopenic purpura in children The J Pe-diatr 1975; 87:16-22.
25. Ballin A, Kenet G, Tamary H, Rechavi G, et al. Infantile idi-apothic thrombocytopenic purpura Pediatr Hemat On-col 1990; 7:323-328.
26. Imbach P, d'Apuzzo V, Hirt A, et al. High dose intravenous gammaglobulin for idiopathic purpura in childhood. Lancet 1981; 1228-31.
27. Najean Y, Dufour V, Rain JD, Toubert ME. The site of pla-telet destruction in thrombocytopenic purpura as a pre-dictive index of the efficacy of splenectomy Brit J Ha-ematol 1991; 79:271-276