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Pseudosickling of hemoglobin Setif

1987; Elsevier BV; Volume: 70; Issue: 1 Linguagem: Inglês

10.1182/blood.v70.1.237.bloodjournal701237

1528-0020

Samuel Charache, Eva Raik, David Holtzclaw, PJ Hathaway, Elizabeth G. Powell, P. J. Fleming,

Hemoglobin structure and function

Hemoglobin Setif produces pseudosickling of red cells in vitro; the nature of the process and the conditions that “trigger” it are unknown. Studies of red cells, hemolysates, purified hemoglobin solutions, and artificial mixtures of Hb A and Setif suggest that pseudosickling is produced by intracellular crystallization of insoluble hemoglobin. Increased tonicity of the suspending medium accentuates the process, probably by causing a rise in intracellular hemoglobin concentration. If precipitates from A/Setif mixtures are analyzed, they always contain Hb A, suggesting an unusual mechanism for the process. Despite the fact that osmolality in the renal medulla is similar to that which produces pseudosickling in vitro, carriers do not have renal dysfunction of the type found in patients with sickle cell disease.