Heredıtary spherocytosıs ın an adult

Abstract

A 58 year old male complaining from fatigue and enlargement of the spleen during last 3 years was admitted.His uncle had been undergone a splenectomy in his family history.There were the enlargement of spleen (15 cm from arcus costarum)and the liver (3cm from arcus costarum)on his examination.His laboratory findings were:Hb:5.2gr,reticulocyte 41 % , 30 percenspherocytosis in periferal blood smear.Direct and indirect Coombs tests were negative.Glucose 6 phosphate dehydrogenase level was normal.There was increased activity of erythropoiesis in his bone marrow exam.Osmotic fragility of his erythrocytes has increased.He experienced no complication after splenectomy.

Özet

58 yaşındaki erkek hastada 3 yıldır halsizlik ve dalak büyüklüğü öyküsü vardı.Aile öyküsünde dayısının dalağı ameliyatla alınmıştı.Fizik bakıda Kosta kavsini dalak 15cm,karaciğer 3cm aşıyordu.Hemoglobin 5.2gr,retilolosit%41 ve periferik yay- mada %30 sferositoz saptandı.Total bilirübin %2.6mg ve CRP 30.5 mg/dl idi.Direk ve indirek Coombs testleri olumsuz- du.Glukoz 6 fosfat dehidrogenaz normal bulundu.Kemik iliği aspirasyonunda artmış hemopoez saptandı.Eritrositlerin osmotik frajilitesinin arttığı saptandı.Vitamin B12 düzeyi düşüktü.Splenektomi sonrası hemolitik kriz görülmedi.