Premium
A Congenital Haemolytic Anaemia with Thermal Sensitivity of the Erythrocyte Membrane
Author(s) -
Zarkowsky H. S.,
Mohandas Narla,
Speaker Connie B.,
Shohet S. B.
Publication year - 1975
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1975.tb02740.x
Subject(s) - fragmentation (computing) , phospholipid , splenectomy , red blood cell , spleen , morphology (biology) , incubation , erythrocyte membrane , biology , chemistry , membrane , medicine , endocrinology , immunology , biochemistry , ecology , genetics
S ummary Microspherocytes, measuring 2‐3 μm in diameter, and cells with blunted projections or triangular in shape characterized the erythrocyte morphology in three children with congenital haemolytic anaemia. Since the erythrocyte morphology resembled that associated with thermal injury, heat‐induced changes in erythrocyte morphology and membrane composition were studied. Erythrocytes developed filaments and spheroid bodies which fragmented, resulting in micro‐spherocyte transformation. Normal cells required exposure to 49° C, whereas, the patients’ cells fragmented at 45°incubation of patients’ cells at 37°C for 1711. The heat‐induced transformation of the patients’ cells was associated with an increase in the membrane cholesterol :phos‐ pholipid and cholesterol :protein ratios. The phospholipid :protein ratio was unchanged. This suggests that fragmentation produces a selective loss of membrane components. Splenectomy ameliorated the haemolytic process. We propose that the patients’ red‐cell morphology is the result of in vivo fragmentation, and that the spleen is the major site of microspherocyte and poikilocyte destruction.