Gastrointestinal vaso-occlusive crisis in a woman with hemoglobin SC disease

2022 ◽  
Author(s):  
Jean M. Clement ◽  
Andrew Y. Li ◽  
Brian W. Berg ◽  
Jennie Y. Law ◽  
Maria R. Baer
Keyword(s):  
Hemoglobin Sc Disease

scholarly journals Hydroxyurea-induced splenic regrowth in an adult patient with severe hemoglobin SC disease

2003 ◽  
Vol 74 (2) ◽  
pp. 125-126 ◽  
Author(s):  
Yiwu Huang ◽  
Thyagarajan Ananthakrishnan ◽  
Joseph E. Eid
Keyword(s):  
Adult Patient ◽  
Hemoglobin Sc Disease

Bacterial Infection and Splenic Reticuloendothelial Function in Children with Hemoglobin SC Disease

PEDIATRICS ◽  
1983 ◽  
Vol 72 (1) ◽  
pp. 93-98
Author(s):  
George R. Buchanan ◽  
Susan J. Smith ◽  
Christine A. Holtkamp ◽  
John P. Fuseler
Keyword(s):  
Bacterial Infection ◽  
Bacterial Infections ◽  
Infection Risk ◽  
Bacterial Disease ◽  
Normal Children ◽  
Splenic Function ◽  
Focus Of Infection ◽  
Serious Bacterial Infections ◽  
Primary Focus ◽  
Hemoglobin Sc Disease

Although the epidemiology and pathophysiology of serious bacterial infection in homozygous sickle cell anemia (SS disease) have become increasingly well understood, information about infection risk and splenic reticuloendothelial function in hemoglobin SC disease is quite limited. Therefore, the type and frequency of invasive bacterial disease were examined in 51 children with SC disease followed for 370 person-years and splenic function was assessed in 31 patients by quantitation of pitted erythrocytes. Seven serious bacterial infections occurred in four of the patients, five due to Streptococcus pneumoniae and two to Haemophilus influenzae. A primary focus of infection was present in all episodes, none of which proved fatal. Although 30 episodes of pneumonia or chest syndrome occurred in 20 of the patients, a bacterial etiology was proven in only three instances. Splenic function was usually impaired, with a mean pit count of 7.1% ± 8.2% (range 0% to 22.9%). This is significantly greater than normal, but less than pit counts in patients with SS disease or asplenic subjects. Children with SC disease may have a greater risk of bacterial infection than normal children, but their infection rate is not nearly as high as that in patients with SS disease.

E. COLl OSTEOMYELITIS COMPLICATING HEMOGLOBIN SC DISEASE

1963 ◽  
Vol 245 (2) ◽  
pp. 116-123 ◽  
Author(s):  
James H. Greenwald ◽  
Arnold L. Widen
Keyword(s):  
Hemoglobin Sc Disease

Case Report: Splenic Infarction and Acute Splenic Sequestration in Adults with Hemoglobin SC Disease

1991 ◽  
Vol 302 (6) ◽  
pp. 374-379 ◽  
Author(s):  
Eugene P. Orringer ◽  
Vance G. Fowler ◽  
Catrell M. Owens ◽  
Adrena E. Johnson ◽  
Matthew A. Mauro ◽  
...  
Keyword(s):  
Case Report ◽  
Splenic Infarction ◽  
Splenic Sequestration ◽  
Hemoglobin Sc Disease

Hemolytic Anemia Associated With a Radiopaque Contrast Agent in a Patient With Hemoglobin SC Disease

1981 ◽  
Vol 71 (12) ◽  
pp. 1552 ◽  
Author(s):  
MARILYN DARR ◽  
STEPHEN HAMBURGER ◽  
BRENT KOPRIVICA ◽  
EDWARD ELLERECK
Keyword(s):  
Contrast Agent ◽  
Hemolytic Anemia ◽  
Hemoglobin Sc Disease

scholarly journals Functional asplenia in hemoglobin SC disease

Blood ◽  
1995 ◽  
Vol 85 (8) ◽  
pp. 2238-2244 ◽  
Author(s):  
PA Lane ◽  
JL O'Connell ◽  
JL Lear ◽  
ZR Rogers ◽  
GM Woods ◽  
...  
Keyword(s):  
Blood Cells ◽  
Prospective Multicenter Study ◽  
Reliable Measure ◽  
Sickle Hemoglobin ◽  
Splenic Function ◽  
Hemoglobin C ◽  
Routine Administration ◽  
Life Threatening ◽  
Infants And Young Children ◽  
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.

scholarly journals Recurrent acute splenic sequestration crisis due to interacting genetic defects: hemoglobin SC disease and hereditary spherocytosis

Blood ◽  
1990 ◽  
Vol 75 (1) ◽  
pp. 266-270 ◽  
Author(s):  
TE Warkentin ◽  
RD Barr ◽  
MA Ali ◽  
N Mohandas
Keyword(s):  
Hereditary Spherocytosis ◽  
Genetic Defects ◽  
Mechanical Instability ◽  
Splenic Sequestration ◽  
Alpha Thalassemia ◽  
Unusual Occurrence ◽  
Distinguishing Features ◽  
Hemoglobin Sc Disease ◽  
Reduced Surface ◽  
In Cells

Abstract A 14-year-old boy with hemoglobin SC disease and alpha-thalassemia-2 experienced five episodes of acute splenic sequestration crisis (ASSC), while two of his siblings with identical globin genotypes (SC and - alpha/alpha alpha) had no such experience. To determine if an additional red blood cell (RBC) defect was responsible for the unusual occurrence of frequent ASSCs, we performed detailed rheologic characterization and membrane protein analysis on RBCs from the proband and other members of his family. Reduced surface area, increased mechanical instability, and decreased spectrin content of the membrane, distinguishing features of RBCs in hereditary spherocytosis, were observed in cells from the proband and his mother, but not in cells from other family members. These findings are consistent with the dominant inheritance of spherocytosis by the proband. We suggest that the combined effects of SC disease and spherocytosis in the proband resulted in decreased RBC deformability and led to increased splenic trapping, intrasplenic sickling, and consequently, recurrent sequestration crisis. Marked clinical and hematologic improvement occurred from splenectomy. Thus, inheritance of interacting genetic defects, sickling hemoglobinopathy, and hereditary spherocytosis appear to be responsible for the unusual clinical manifestation of recurrent ASSC in this patient.

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