1.Genealogical Study of a Married Couple from Two Families with Hereditary Spherocytosis.
Tadashi ARAI ; Yo YASUDA ; Yuji ITOU ; Kazuyoshi HAYAKAWA ; Tadatake TAKAYA ; Satoshi TOSHIMA ; Chiken SHIBUYA ; Shigeko SATOU ; Tokie NAKAGAWA ; Yoshitomo KASHIKI
Journal of the Japanese Association of Rural Medicine 1996;44(5):676-679
Hereditary spherocytosis (HS) is a condition characterized by jaundice, hepato-splenomegaly tumors, and the presence of spherocytes. It accounts for about 70% of the reported cases of congenital hemolytic anemia in Japan. Many researchers state that this pathologic condition primarily arises from an abnormality of the red blood cell membrane. We have encountered a familiy, all the members (23-year-old daughter, 20-year-old son, 53-year-old-father, and 46-year-old-mother) of which had their cases diagnosed as HS by peripheral blood examination and electron microscopy. Excluding the farher, the peripheral blood examination also revealed anemia in the other family members. Moreover, the common HLA was found in all the members of these two families including the one on the mother's side, but human parvovirus B19 was not detected at all. We are providing the socalledprophylactic visiting care for the two families, while giving them information about HS which is arelatively easily-misunderstood hereditary disease. We did not find any report about married coupleswith hereditary spherocythosis. In this paper, we report our findings so far obtained through visiting care with some bibliographic discussion on HS.