GM-CSF in sickle cell anemia patients with elevated Hb F

We estimated plasma GM-CSF levels in a group of 28 steady-state sickle cell anemia (SS) patients in Kuwait, using an ELISA technique. There were 24 ag e-matched Hb AA controls, 14 of whom were healthy while 10 were acutely ill at the time of the study. Five SS patients were also studied during 6 epis odes of painful crisis. Among the SS patients, 82.1% were homozygous for th e Saudi Arabia/India (SAI) haplotype with Hb F ranging from 15 to 35% and t otal Hb from 8.5 to 11 g/dl. Three patients (siblings) were SAI/Benin compo und heterozygotes with Hb F of 9-23% and total Hb >10 g/dl. One patient eac h was homozygous for the Benin or the Bantu haplotype; they had Hb F <2% an d total Hb of 6.6 and 7.2 g/dl, respectively. Four (14.3%) steady-state SS patients had detectable plasma GM-CSF ranging from 75 to 1,817.6 pg/ml. The se included the 2 patients with Hb F <2.0% and 2 with the SAI/Benin compoun d heterozygotes with Hb F of 11 and 9%, respectively. Four (66.7%) SS patie nts in crisis, 6 (42.9%) healthy controls and 6 (60%) acutely ill controls had detectable plasma GM-CSF. A clearcut association of GM-CSF with Hb F le vel or degree of anemia in steady-state SS patients could not be establishe d. The appearance of GM-CSF in the plasma of patients in crisis and also am ong control subjects raises the possibility that other factors are involved in the production of this cytokine in the subjects studied. Copyright (C) 2000 S. Karger AG, Basel.