Yonsei Medical Journal  1987;28(3):234-242

doi:10.3349/ymj.1987.28.3.234

A Case of Congenital Microspherocytosis Requiring Early Splenectomy.

Fan Chen MONG 1 ; Kwang Shin CHO ; Kir Young KIM

Affiliations

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Keywords

Microspherocytosis; osmotic fragility test; autohemolysis test; acidified glycerol lysis test; spectrin; actin

Country

Republic of Korea

Language

English

Abstract

Microspherocytosis is known as a hallmark of hereditary spherocytosis (HS) which is one of the most common hemolytic anemias with a prevalence of one per 5000, and is inherited as Mendelian dominant. In this disorder, the patient's red cells become spheroidal, osmotically less resistant in the peripheral circulation, and are selectively trapped in the spleen, but survive normally after splenectomy. The exact mechanism for the formation of microspherocytosis has not been elucidated, although extensive investigations demonstrate that HS red cells are intrinsiclly defective and the patient's spleen does "condition" the metabolically abnormal red cells. The authors report here, one case of severe microspherocytosis in which changes in facial bone structure and transfusion dependency are noted, and an early splenectomy is indicated.