T hücre klonalitesi gösteren primer saf eritrosit aplazili iki olgu: İmmunsupressif tedavi yanıtı

Clonality of acquired primary pure red cell aplasia in two cases: Effectiveness of immunosuppressive treatment

Meliha NALÇACI, Melih AKTAN, Hüseyin KESKİN, Günçağ DİNÇOL, Koray DİNÇOL, A. Selim YAVUZ, Mehmet TURGUT

Özet

Primer saf eritrosit aplazisi (SEA) 65 ve 69 yaşlarında iki erkek hastada teşhis edildi. Her iki olguda çevre kanı mononiikleer hücrelerinin yüzey markır incelemeleriyle T hücre reseptörü¬nün (TCR) ap+ fenotipinde olduğu saptandı. PCR ile T hücre klonalitesi gösterildi. Her iki ol¬guda aneminin 1 mg/kg/gün prednisolon ile düzeldiği, ilaç dozu azaltılınca tekrarladığı, at kaynaklı antitimosit globulinle (ATG) (20 mg/kg/gün, 8 gün süreyle) tekrar düzeldiği ve ikinci olguda makalenin yazıldığı tarihte hemoglobinin 15 g/dl'yi bulduğu tesbit edildi.

Abstract

Clonality of acquired primary pure red cell aplasia in two cases: Effectiveness of immuno-suppressive treatment. Primary pure red cell aplasia (PRCA) was diagnosed in two male pati¬ents, 65 and 69 years old respectively. In both, surface markers of peripheral blood nuclear cells revealed the presence of TCR ap+ phenotype. Clonality of T cells was confirmed by the polymerase chain reaction in both patients, in whom, prednisone at a dose of 1 mg/kg/day cor¬rected the anemia and lower doses caused its renewal, whereas horse antithymocyte globulin (ATG) (20 mg/kg/day 1 to 8 +) seems to have treated it as if permanently (second case) since hemoglobin values are about 15 g/dl at the time of writing. We, therefore, suggest that, patients with acquired primary PRCA should be screened to detect the presence of a T-cell clone and recommend that, treatment should start earlier with ATG, if PRCA is due to a T-cell clonal disorder.