All issues > Volume 33(1); 1990

Original Article

J Korean Pediatr Soc. 1990;33(1):81-87. Published online January 31, 1990.

A Clinical Study of Hereditary Spherocytosis.

Ki Ho Kim¹, Kun Soo Lee¹, Haeng Mi Kim¹, Doo Hong Ahn¹

¹Department of Pediatrics, School of Medicine, Kyungpook National University, Taegu, Korea

Received: May 11, 1989;  Accepted: July 24, 1989.

Abstract

The following results were obtained from eight cases of hereditary spherocytosis (HS) who were admitted at the Department of Pediatrics, Kyung-pook National University Hospital from July 1984 to January 1989. The percentage of HS among the pediatric hematology patients during the same period was 2.6%. Male amd female were one and seven respectively. The mean age at the diagnosis and at onset of symptom was 7.1 years (8 months-13.4 years) and 4.9 years (2 months-10.4 years). The duration from initial symptom to diagnosis was 2.2 years (5 months-5 years). The chief complaint was jaundice in 2, hepatosplenomegaly in 2, splenomegaly in 1, easy fatigability in 1, and pallor in 1. The liver and spleen were enlarged in 6 cases with 2.2 cm and 5.3 cm below the costal margin respectively. The mean hemoglobin was 8.2 g/dl, the reticulocyte count 12.3%, the mean reticulocyte production index 4.4, the mean MCV 78u3, the mean MCH 28 pg, the mean MCHC 34 g/dl. The mean spherocyte was 33% and the mean bilirubin 2.4 mg% with indirect predominant. The bone marrow aspiration was done in 7 cases and showed erythroid hyperplasia in all. In osmotic fragility test, the hemolysis was started in the mean concentration of 0.7%NaCl solution and was completed in 0.48%. The autohemolysis test at 48 hours showed 9.8%, 4.2%, 2.6%, 6.6%, 40.8%, and 60.8% of hemolysis in 6 cases and showed partial correction by adding glucose in 2 cases. Aplastic crisis was deveploped in one case and was recovered with packed cell transfusion after several days. Splenectomy was performed in 5 cases (10,11,13.4,5.9, and 10.6 years old) and cholecystectomy was done in one of five concomitantly.

Keywords :Hereditary Spherocytosis, Reticulocyte production index, Aplastic crisis, Splenectomy, Cholecystectomy