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At least 19 recordsLinked to original sources
PubMed1996

Hematologic profile and lymphocyte subpopulations in hemoglobin SC disease: comparison with hemoglobin SS and black controls. The Transfusion Safety Study Group.

Compared with subjects with homozygous SS disease (Hb SS), persons with hemoglobin SC (Hb SC) are known to have a more gradual loss of splenic function, a lower incidence of bacterial infections, and fewer end-organ failures. We studied hematological indices and lymphocyte subpopulations of 27 Hb SC subjects and compared them with 173 Hb SS patients and 131 black controls. Hb SC patients had higher hemoglobin levels than Hb SS subjects, lower total leukocyte, granulocyte, monocyte, and lymphocyte counts. Platelets decreased with age but not significantly, instead of increasing as among Hb SS patients. Mononuclear cells were generally similar to controls with the exception of CD8+HLA-DR+ counts resembling Hb SS. Hematologic changes in Hb SC are limited to moderate granulocytosis in children and adults, mild monocytosis in adults, and increased activation of just one lymphocyte subset among those measured.

Adolescent↗
PubMed1984

Neurological complications of hemoglobin SC disease.

The records of 68 patients with hemoglobin SC disease and 68 age- and sex-matched control patients were reviewed for neurological problems. A significant increase in retinopathy, stupor/coma, and seizures was noted in the hemoglobin SC group. Hemiplegia, noted in two young patients, was probably also secondary to hemoglobin SC disease. Hemoglobin SC disease may often go unrecognized as a cause of stupor and coma in older patients without other obvious manifestations of a sickling hemoglobinopathy. Factors known to precipitate sickling crisis and the associated neurological complications should be avoided, especially in patients undergoing surgery or parturition.

Adolescent↗
PubMed1986

Densitometry and microchromatography compared for determination of the hemoglobin C and A2 proportions in hemoglobin C and hemoglobin SC disease and in hemoglobin C trait.

Using both densitometry and anion-exchange microchromatography, we measured hemoglobin C (Hb C) and Hb A2 proportions in 11 patients, eight of whom had Hb AC, two Hb SC, and one Hb CC. For one patient with Hb SC, we made the determinations before and after a transfusion. The mean (and SD) for the sum of Hb C + Hb A2 by densitometry and anion-exchange microchromatography for the nine patients with Hb AC and Hb CC were 45 (18) and 40 (18)%, respectively (p greater than 0.1, r = 0.98); for the three determinations involving the two Hb SC patients, the respective proportions were 40 (9.9) and 38 (6.6)% (r = 0.88). Electrophoretic analysis of microchromatographic eluates from the Hb AC and Hb CC patients showed that 6% of the absorbance of the late high-ionic-strength eluate was due to Hb C, which was responsible for the statistically insignificant difference between densitometric and chromatographic values for Hb C + A2 values. Electrophoresis on cellulose acetate of concentrated eluates of the Hb C + A2 fraction from the two Hb SC patients revealed no contamination by Hb S. Evidently, microchromatography can be used to determine Hb C + A2 in patients with Hb C or Hb SC disease or Hb C trait.

Anemia, Sickle Cell↗
PubMed1980

Bacillus cereus bacteremia and hemolytic anemia in a patient with hemoglobin SC disease.

A patient with hemoglobin SC disease and cholelithiasis was found to have Bacillus cereus bacteremia. Hemolytic anemia developed, for which common causes of hemolysis were excluded, suggesting a relationship with the bacteremia. Following in vitro incubation, type O erythrocytes were hemolyzed by the culture, but not by a bacteria-free filtrate. This case confirms the association between sickle cell disorders and cholelithiasis with B cereus infections. In addition, it provides evidence for in vivo hemolysis with B cereus bacteremia, an organism not previously associated with hemolytic anemia.

Adult↗
PubMed1986

Clinical and hematological features of hemoglobin SC disease in Rio de Janeiro, Brazil.

1. Thirty-two patients with hemoglobin SC (Hb SC) disease from Rio de Janeiro, Brazil, are described. Mean patient age at the time of study was 20 years, and average age at onset nine years. 2. The main complaint at presentation was bone or joint pains. Symptoms involved bone or joint pains in 72% of the patients, while 59% presented hepatomegaly and 50% splenomegaly. Mean hemoglobin level was 10.6 g/dl, erythrocyte count, 3.9 X 10(6)/mm3, and serum bilirubin, 1.4 g/dl. Reticulocytes and Hb F were 3.0% and 2.2%, respectively. 3. The characteristics of Hb SC disease observed in the present study did not differ significantly from those of other series studied elsewhere. This relative homogeneity of data is probably related to the physiopathological mechanism that promotes sickling in this condition.

Adolescent↗
PubMed1995

Functional asplenia in hemoglobin SC disease.

The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life-threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% +/- 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% +/- 8.8%) or with sickle cell anemia (20.5% +/- 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (> 20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.

Adolescent↗
PubMed1978

Myocardial necrosis following general anesthesia in hemoglobin SC disease.

A 4-year-old girl with hemoglobin SC disease died following general anesthesia. Autopsy showed widespread intravascular sickling; staining with hematoxylin-basic fuchsin-picric acid demonstrated newly developed massive myocardial necrosis, a rarely documented finding. Anesthesia may produce conditions that provoke "crises" in patients with sickle hemoglobinopathies. Preoperative identification of sickle states and careful attention to hydration and oxygenation may minimize anesthetic risks in these patients.

Anemia, Sickle Cell↗
PubMed1993

[Fatal course of a sequestration crisis in hemoglobin SC disease].

A 14 year-old patient developed severe anemia and splenomegaly 2 days after the onset of a febrile upper airway infection. The hemoglobin concentration had dropped to 1.1 g/dl. Death occurred as consequence of the acute anemia and peripheral circulatory failure. The crisis was caused by an acute splenic sequestration. Hemoglobin SC disease could be identified as the underlying disorder. Hemoglobin SC disease usually has a milder course than sickle cell disease. However the patients may develop the same crisis-like symptoms. Splenic sequestration is caused by the occlusion of the splenic sinuses due to sickled and aggregated erythrocytes with subsequent trapping of large blood volumes and circulatory failure. Regular transfusions and/or splenectomy are recommended to prevent splenic sequestration crisis.

Adolescent↗
PubMed1994

Fatal pneumococcal septicemia in hemoglobin SC disease.

We retrospectively examined the medical and autopsy records of seven previously unpublished cases of fatal pneumococcal septicemia in children with hemoglobin SC disease. The earliest death occurred in a 1-year-old child who had congenital heart disease with cyanosis; the other children were aged 3 1/2 to 15 years. Only one child had received pneumococcal vaccine or prophylactic penicillin therapy. All seven children had an acute febrile illness and rapid clinical deterioration despite parenterally administered antibiotic therapy and intensive medical support. Erythrocyte pit counts in two patients were 40.3% and 41.7%, respectively (normal, < or = 3.6%). Autopsy data from five cases showed marked splenic congestion without infarction in five, splenomegaly in four, and bilateral adrenal hemorrhage in three. These cases illustrate that functional asplenia predisposes some children with hemoglobin SC disease to the development of fatal septicemia after the age of 3 years. We conclude that pneumococcal vaccine should be administered to all children with hemoglobin SC disease and that acute febrile illnesses should be investigated promptly for the possibility of septicemia. The routine use of prophylactic penicillin therapy in infants and children with hemoglobin SC disease remains controversial.

Adolescent↗
PubMed1978

Partial exchange transfusion as treatment for hemoglobin SC disease in pregnancy.

Serious infarctions and embolic events can complicate the course of pregnant patients with hemoglobin SC disease. In two cases, partial exchange transfusion preceded recovery in severely ill pregnant women with hemoglobin SC disease. There seem to be pathophysiological correlations for the observed clinical findings, and there are potential beneficial effects of partial exchange transfusion. Based on our experience, partial exchange transfusion should be considered as a means of reversing the often fatal complications attending hemoglobin SC disease and pregnancy. The exchange should be of sufficient volume to ensure a postexchange level of hemoglobin A of at least 30%.

Adult↗
PubMed2002

Outcome in hemoglobin SC disease: a four-decade observational study of clinical, hematologic, and genetic factors.

Over the past 40 years, we observed 284 subjects with hemoglobin SC disease (Hb SC) for 2,837 person-years. We examined the association of the course of clinical events with hematologic and genetic factors. The mean entry age was 21 years, although 15% entered before one year of age. The mean Hb concentration was 11.3 g/dL, the mean fetal hemoglobin was 2.5%, and the mean MCV was 84.4 fL. Twenty-five subjects died at a median age of 37 years. Chronic organ-specific complications occurred in 112 subjects (39.4%), with advanced retinopathy in 65 subjects (22.9%) and osteonecrosis (avascular necrosis) in 42 subjects (14.8%). We identified the beta-globin haplotypes in 82 subjects and the alpha-gene status in 79. Twenty-nine percent had alpha-thalassemia-2. The beta(CI) haplotype was present in 85.4%. We found a decreased incidence of retinopathy in the beta(CI) subjects compared to the non-beta(CI) subjects (33% vs. 67%; P = 0.049) with a later mean onset age (29 years vs. 21 years; log-rank test, P= 0.026). We also found a consistent pattern of decreased morbidity in subjects who had alpha-thalassemia-2 in comparison to those who did not. We found a reduced risk of chronic organ-specific complications (log-rank test, P= 0.003), lower incidence of sickle crisis (48% vs. 80%, P= 0.001), later onset of gallbladder disease (age of onset: 55 years vs. 34 years; P= 0.055), and lower risk of osteonecrosis (log-rank test, P= 0.024). Our findings suggest that Hb SC subjects who have not inherited alpha-thalassemia-2 might benefit from erythrocyte rehydration therapy.

Adolescent↗
PubMed1980

Preferential binding of beta C relative to beta S globin to stroma in hemoglobin SC disease.

Globin synthesis was measured in blood samples of four patients with hemoglobin SC Disease. In the stroma-free supernatant, globin synthesis was balanced with alpha/(beta S + beta C) = 1. In the exhaustively washed stroma, there was a preferential binding of beta chains to stroma, similar to that reported for Sickle Cell Anemia. However, while the beta S/beta C ratio was balanced in the supernatant (0.8-0.9), the beta S/beta C ratio of the stroma varied from 0.2 to 0.4. Therefore, beta C is bound to stroma in preference to beta S in hemoglobin SC disease. These findings are consistent with the concept that binding of globin to stroma is related to the electrophoretic charge of the hemoglobin.

Anemia, Sickle Cell↗
PubMed1983

Splenic function in children with hemoglobin SC disease and sickle beta-thalassemia.

Splenic function in children with hemoglobin SC disease and sickle β-thalassemia was evaluated using direct interference contrast microscopy. Children with both disorders demonstrated significant degrees of hyposplenism as evidenced by increased numbers of erythrocytes containing surface "pits." Patients with these variants of sickle cell disease frequently exhibit splenic dysfunction and may be at increased risk to develop the life-threatening septicemias associated with hyposplenism.

Adult↗
PubMed1986

Hemoglobin SC disease and alpha-thalassemia. Prolonged survival and mild clinical course.

An 86-year-old black woman admitted for an elective cataract extraction was found to have moderate hypochromic microcytic anemia. Hematologic evaluation disclosed the presence of hemoglobin SC disease and heterozygous alpha-thalassemia-2 (alpha alpha/alpha-). A red cell density profile of the patient's peripheral blood revealed an absence of the typical uniform shift toward higher-density values seen in hemoglobin SC disease, indicating a "normalization" in the distribution of intracellular hemoglobin types. It is suggested that the co-inheritance of hemoglobin SC disease and heterozygous alpha-thalassemia-2, probably by decreasing the tendency toward intracellular hemoglobin S polymerization, contributed to her prolonged survival and relatively mild clinical course.

Aged↗
PubMed2007

Limb amputation in hemoglobin SC disease after application of ice and elevation.

A 31-year-old man with hemoglobin SC disease was admitted with acute chest syndrome, treated with antibiotics and ventilator support. His restrained right wrist and hand became swollen and cool, thought due to infiltration of an intravenous line. The hand was elevated, ice packs applied around the clock, and prophylactic low molecular weight heparin given. The arm quickly turned gangrenous. In spite of attempted thrombectomy and red blood cell exchange transfusion, amputation was required. Cold, elevation, and measures that reduce blood flow are known precipitators of sickling and crises. Vasoocclusion of the extremities to the point of gangrene is rarely reported in sickle cell disorders, and this case highlights the dangers of measures limiting blood flow in such patients.

Adult↗