抄録
We present a case of multiple myeloma associated with hereditary spherocytosis. The combination of these two diseases is very rare. In addition, this patient revealed ambiguous physical and laboratory findings of anemia that bewildered us to make diagnosis.
A 71-year-old male had yellowish discoloration since juvenile but made no clinical visit for it. On Oct. 1, 1986, he had onset of sudden frontal headache. On Oct. 14, he was admitted to our hospital because of conspicuous pallor, jaundice and splenomegaly. Skull X-ray revealed multiple punched-out lesions. In the bone marrow, myeloma cells were increased up to 26% of nucleated cells. L chain (κ) was detected in urinary immunoelectrophoresis. From these findings, Bence Jones type multiple myeloma was diagnosed. Anemia was unusually severe for an early phase of multiple myeloma, and eminent splenomegaly and icterus were difficult to be explained by myeloma alone. Family history, existence of spherocytosis on peripheral blood smear, positive osmofragility test of red blood cell and negative Coombs test strongly suggested hereditary spherocytosis. Splenectomy was not performed on this patient, because of the high risk of infection.
