Clinical, hematological, and biochemical features of Hb SC disease

Abstract

Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.

Protein C and S and inflammation in sickle cell disease

“…sVCAM-1 thus most likely reflects vascular endothelial activation in sickle cell disease. A fundamental difference between the HbSS and HbSC genotypes is the lower concentration of sickle hemoglobin in red cells of HbSC patients with subsequently less in vivo sickling-desickling and red cell membrane changes, less exposure of the endothelium to reactive oxygen species, less circulating rigid irreversibly sickled erythrocytes, and a lower rate of hemolysis [12,[31][32][33]. Such factors can all activate and damage endothelial cells, and the fact that these occur to a greater extent in HbSS patients as compared to HbSC patients may explain the more profound serum sVCAM-1 enhancements in HbSS subjects as compared to HbSC patients (without increased vWF levels in this last group) [34][35][36][37].…”

Hemoglobin sickle cell disease complications: a clinical study of 179 cases

“…Among the 69% of foreign born patients, the mean duration of residence in France was 13.8 years and the mean duration of follow up at the center was 5.1 years. Fifty-eight percent of female patients had been pregnant with a mean of 1.9 children per woman and 32 spontaneous abortions were reported in 12 females (5 multiple abortions, range [2][3][4][5][6][7][8][9][10][11]. High body mass index (≥25kg/m 2 ) and blood hypertension (> 130/80 mmHg) were reported in 33% and 14% of HbSC patients, respectively.…”

“…The main reason is that HbC enhances the formation of intracellular polymer of HbS by dehydrating red cells. 2,3 The major acute features of SCA are recurrent painful vasoocclusive crisis (VOC) and acute chest syndrome (ACS), priapism and anemia. Chronic organ dysfunctions are of increasing concern in adult patients leading to lifethreatening end-stage organ failures.…”

“…5 Curiously, whereas SCA clinical features have been extensively studied, very few studies have been dedicated specifically to HbSC disease. 3,[6][7][8][9] Indeed, HbSC disease is generally considered to be a variant of SCA, sharing similar clinical complications though with a milder severity and a lower frequency. 7 Therefore, the aim of our study was to examine the specific clinical and biological features of HbSC disease in a single center patient cohort.…”

Murine glutathione S‐transferase A1‐1 in sickle transgenic mice

“…Ten percentage of SCA patients admitted to hospitals have hepatic complications [13,14] and 50% of SCA patients in crisis have abnormal liver function tests [15].…”