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YÜZ ALTMIŞ İKİ KRONİK İDİYOPATİK TROMBOSİTOPENİK PURPURA OLGUSUNUN KLİNİK SEYİR VE TEDAVİ CEVABI YÖNÜNDEN DEĞERLENDİRİLMESİ

CLINICAL FOLLOW-UP AND TREATMENT RESPONSE OF THE 162 PATIENTS WITH CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA

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Abstract (2. Language): 
Aim: In this report, we investigated the presenting clinical features, therapy indications, clinical follow-up and treatment results of the 162 patients with chronic idiopathic thrombocytopenic purpura (ITP). Material and methods: The cases that were followed-up in our outpatient clinic between years 1972 to 2003 were retrospectively analyzed. Results: A hundred and twenty-two of the patients were female, 40 of them were male and their age distribution was between 12 and 84 years (median: 35 years). Prednisolone (1mg/kg) was the first line treatment option in 138 of the patients; 24 of them was followed-up without any treatment. Complete response was achieved in 64 (46,3 %) of the patients. Platelet counts decreased to the beginning level in 26 of the complete responders when the PRD doses were decreased. Eleven of the complete responders showed relapse in between 6 months to 4 years. Platelet counts were found in the range of 5.000/mm3 to 125.000/mm3 in the patients who did not get complete response. There was not any serious bleeding problem other than bruising in none of the patients including those who had severe trombocytopenia. None of our patients died because of bleeding complication during the follow-up period. Conclusion: ITPis an autoimmune disorder characterized by muco-cutaneous bleeding. The pathogenesis of ITP is presumed to be related to platelet destruction via specific auto-antibodies. Bleeding signs and symptoms of the patients with ITP are not always obvious and not as severe as in bone marrow failure syndromes. Cytotoxic agents shouldn’t be used if they are not really necessary and the patients’ symptoms should be tried to be treated, not the thrombocytopenia itself.
Abstract (Original Language): 
Amaç: Bu çal›flmada 162 eriflkin kronik idiyopatik trombositopenik purpura (‹TP)’l› hastan›n tan› s›ras›ndaki özelliklerini, tedavi endikasyonlar›n›, klinik seyir ve tedavi sonuçlar›n› araflt›rd›k. Gereç ve yöntem: 1972-2003 y›llar› aras›nda poliklini¤imize baflvuran ve gözlem alt›na al›nan olgular retrospektif olarak de¤erlendirildi. Bulgular: Yafllar› 12 ile 84 yafl aras›nda (ortanca: 35 yafl) de¤iflen hastalar›n 122’ si kad›n 40’ › erkek idi (K/E: 3,0). Prednizolon (1mg/kg) 138 olguda ilk tedavi seçene¤i olarak kullan›ld›; 24 olgu tedavisiz olarak takip edildi. Olgular›n 64’ünde (% 46,3) tam yan›t saptand›. Bu olgular›n 26’s›nda PRD dozunun azalt›lmas›yla trombositler iki ay içinde tekrar eski haline geriledi. Tam yan›t sa¤lanan hastalar›n 11’inde 6 ay ile 4 y›l içinde nüks geliflti¤i görülmüfltür. Tam yan› t›n sa¤lanamad›¤› olgularda son trombosit de¤erleri 5.000 ile 125.000/m3 aras›nda (ortanca: 51.000/m3) bulundu. A¤›r trombositopenisi (<10.000/mm3) olan olgular dahil (10 olgu) hastalar›m›z›n hiç birinde cilt kanamalar› d›fl›nda ciddi kanama sorunu gözlenmedi. Bu süre içinde ‹TP’ye ba¤l› kanamalar nedeniyle kaybedilen olgumuz olmad›. Sonuç: ‹TP genellikle deri ve mukoza kanamalar›yla kendini gösteren otoimmun bir hastal›kt›r. Hastal›¤›n patogenezinde trombositlere karfl› oluflan otoantikorlar›n etkisiyle trombositlerde y›k›m kusuru sorumlu tutulur. Kanamalar kemik ili¤i yetersizli¤ine ba¤l› hastal›klarda oldu¤u gibi a¤›r de¤ildir. Bu nedenle özellikle sitotoksik ajanlar› çok gerekmedikçe kullanmamal›, trombositopeninin kendisi de¤il hastalar›n semptomlar› tedavi edilmeye çal›fl›lmal›d›r.
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REFERENCES

References: 

1. Chend Y, Wong RSM, Soo YOY, Chui CH, Lau FY, Chan NPH,
Wong WS, Cheng G. Initial treatment of immune thrombocytopenic
purpura with high-dose dexamethazone. N Engl J Med
2003; 349:831-836.
2. Cines DB, Blanchette VS. Immune thrombocytopenic purpura.
N Engl J Med 2002; 346:995-1008.
3. Diz-Küçükkaya R, Gushiken FG, Lopez JA. Thrombocytopenia.
In: Lichtman MA (Ed) Williams Hematology New York
USA 7th Ed., 2006; pp 1758-1763.
4. George JN, Davidoff F. Idiopathic thrombocytopenic purpura:
Lessons from a guideline. Ann Intern Med 1997; 126:317-318.
5. George JN, El-Harake MA, Raskob GE. Chronic idiopathic
thrombocytopenic purpura. N Engl J Med 1994; 331:1207-1211.
6. Wasser JS, Aledort LM, Ballem PJ, Blanchette VS, Bussel JB,
Cines DB, Kelton JG, Lichtin AE, McMillan R, Okerbloom JA,
Regan DH, Warrie I. Idiopathic thrombocytopenic purpura: A
practice guideline developed by explicit methods for the American
Society of Hematology. Blood 1996; 88:3-40.
7. Guidelines for the investigation and management of idiopathic
thrombocytopenic purpura in adults, children and in pregnancy.
Br J Haematol 2003; 120:574-596.
8. Imbach P, Kuhne T, Signer E. Historical aspects and present
knowledge of idiopathic thrombocytopenic purpura. Br J Haematol
2002; 119:894-900.
9. Kojouri K, George JN. Recent advances in the treatment of chronic
refractory immune thrombocytopenic purpura. Int J Hematol
2005; 81:119-125.
10. Kojouri K, Vesely SK, Terrell DR, George JN. Splenectomy for
adult patients with idiopathic thrombocytopenic purpura: a
systematic review to assess long-term platelet count responses,
prediction of response, and surgical complications. Blood 2004;
104:2623-2634.
11. Kumar S, Diehn FE, Gertz MA, Tefferi A. Splenectomy for immune
thrombocytopenic purpura: long-term results and treatment
of postsplenectomy relapses. Ann Hematol 2002; 81:312-319.
12. Menke DM, Colon-Otero G, Cockerill KJ, Jenkins RB, Noel P,
Pierre RV. Refractory thrombocytopenia: A myelodysplastic
syndrome that may mimic immune thrombocytopenic purpura.
Am J Clin Pathol 1992; 98:502-510.
13. Najean Y, Lecompte T. Chronic pure thrombocytopenia in elderly
patients: An aspect of the myelodysplastic syndrome. Cancer
1989; 64:2506-2510.
14. Penalver FJ, Jimenez-Yuste V, Almagro M, Alvarez-Larran A,
Rodríguez L, Casado M, Gallur L, Giraldo P, Hernandez R, Menor
D, Rodríguez MJ, Caballero D, Gonzalez R, Mayans J, Millan
I and Cabrera JR. On behalf of the Multi-institutional Retrospective
Spanish Study on the use of rituximab in refractory ITP.
Rituximab in the management of chronic immune thrombocytopenic
purpura: an effective and safe therapeutic alternative in refractory
patients. Ann Hematol 2006; 85:400-406.
15. Portielje JE, Westendorp RG, Kluin-Nelemans HC, Brand A.
Morbidity and mortality in adults with idiopathic thrombocytopenic
purpura. Blood 2001; 97:2549-2554.
16. Stasi R, Stipa E, Masi M, Cecconi C, Scimò M, Oliva F, Sciarra
A, Perrotti A, Adomo G, Amadori S. Long-term observation of
208 adults with chronic idiopathic thrombocytopenic purpura.
Am J Med 1995; 98:436-442.
17. Vesely SK, Perdue JJ, Rizvi MA, Terrell DR, George JN. Management
of adult patients with persistent idiopathic thrombocytopenic
purpura following splenectomy: a systematic review. Ann
Intern Med 2004; 140:112-120.
18. Wandt H, Frank M Ehninger G, Schneider C, Brack N, Daoud
A, Fackler-Schwalbe I, Fischer J, Gäckle R, Geer T, Harms P,
Löffler B, Öhl S, Otremba B, Raab M, Schönrock-Nabulsi P,
Strobel G, Winter R, Link H. Safety and cost effectiveness of a
10 x 10(9)/L trigger for prophylactic platelet transfusions compared
with the traditional 20 x 10(9)/L trigger: A prospective
comparative trial in 105 patients with acute myeloid leukemia.
Blood 1998; 91:3601-3606.

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