Seventeen cases of hereditary spherocytosis who were admitted to the Department of
Pediatrics, SNUH, from 1966 to Aug. 1982, were reviewed.
The results were as follows:
1. The incidence of HS among the pediatric hematologic disorder was 2.16% in SNUH.
2. The male and female were 6 and 11 respectively, with the M:F ratio of 1:1.8.
3. The age of onset of symptoms was below 10 years in 16 of 17 (94%).
4. The family history of HS was obtained in 7 of 17 (41%) and the family history of
anemia, in 3 of 17 (18%).
5. The most frequent chief complaint was the symptoms associated with anemia
(76%), and the next were abdominal mass or distension (29%) and jaundice (18%).
6. The acute crises were suspected in past history in 11 of 17 (65%).
7. The prominant physical findings were pallor(100%), splenomegaly(88%),
hepatomegaly (88%), and jaundice (65%).
8. The major laboratory findings were as follows:
ⅰ) Anemia was present in all cases with the mean hemoglobin of 7.72 (range: 4.5∼
11) gm%.
ii) Reticulocytosis was present in 16 of 17 (94%) with the mean of 7.2 (range: 0.1∼
22.7)%
iii) Serum bilirubin was increased in 12 of 17 (71%) with the mean of 2.84 (range:0.8
3∼6.6)㎎%.
iv) Osmotic fragility, incubated, was increased in all cases with the mean
concentration
of begin, 0.72% and that of complete hemolysis, 0.40%.
9. Radiologic abnormalities of skull were found in 2 of 17 (12%) with diploic space
wi-
dening and hair-on-end appearance.
10. Splenectomy was performed in 11 of 17 and the mean weight of spleen was 452
(range 150∼ 1,100)gm.
Accessory spleens were found in 5 of 11 (46%).
Pathologic findings of spleen were marked congestion of cords with relatively empty
sinuses.
Gallstones were found in 1 case during the operation.
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