1974 Volume 15 Issue 9 Pages 1011-1020
The hereditary spherocytosis (HS) is probably inherited as a mendelian dominant, but the clinical abnormality is less than the theoretical frequency. This may be due to the reason why the mildly affected cases could not be easily distinquished from the normal persons.
Recently, many papers reported that serum haptoglobin (Hp) disappeared from the blood circulation in hemolytic diseases.
This study investigated the correlation between HS and Hp, especially on the family and relative examinations.
The results were as follows.
1. The sera of 4 indibiduals with HS had no free Hp.
2. After splenectomy, serum Hp levels were rapidly raised and almost kept within the normal range.
3. The family and relative examinations were performed in case 11 who had no history of familiar transmission of HS, and 5 cases of hypohaptoglobinemia were found out of 20 cases, including the patient.
4. 5 cases were comparatively located in some families, that is, 2 cases in 56 years old uncle's, 2 cases in patient's and 1 case in 46 years old uncle's families.
5. Hypohaptoglobinemia was related to the decreased osmotic fragility rather than the anemia, high reticulocytosis or splenomegaly.
6. We considered that they might be the affected family members with slightly chronic hemolysis.
