Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations

Zimmerman, Sherri A.; Ware, Russell E.

doi:10.1046/j.1365-2257.2000.00304.x

Cited by 19 publications

(11 citation statements)

References 20 publications

(45 reference statements)

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“…Our 65 Hb SC subjects with grade III or above retinopathy had a mean age of diagnosis of 31 ± 14.1 years. Recent literature focused on case reports of Hb SC subjects describing complications and unusual clinical presentations [11,19,20,[41][42][43]. Detailed case studies reported on therapeutic options in the management of retinopathy and osteonecrosis of the femoral head [44,45].…”

Section: Discussionmentioning

confidence: 99%

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Outcome in hemoglobin SC disease: A four‐decade observational study of clinical, hematologic, and genetic factors

et al. 2002

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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 ␤-globin haplotypes in 82 subjects and the ␣-gene status in 79. Twenty-nine percent had ␣-thalassemia-2. The ␤ CI haplotype was present in 85.4%. We found a decreased incidence of retinopathy in the ␤ CI subjects compared to the non-␤ 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 ␣-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 ␣-thalassemia-2 might benefit from erythrocyte rehydration therapy. Am. J. Hematol. 70:206-215, 2002.

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Section: Discussionmentioning

confidence: 99%

“…Childhood growth and development are near normal [9]. The onset of splenic dysfunction and renal concentrating defect occurs during late adolescence or young adulthood [10][11][12][13]. The low mortality rates in children living in the United States have remained unchanged for many years [14].…”

Section: Introductionmentioning

confidence: 99%

Outcome in hemoglobin SC disease: A four‐decade observational study of clinical, hematologic, and genetic factors

et al. 2002

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“…The prevalence of splenomegaly by palpation or imaging has been reported in up to 50–60% of adult patients with HbSC disease. In a cohort of paediatric patients with HbSC, palpable splenomegaly was found in 34 of 100 children over 2 years of age (mean age 11·0 ± 5·4 years; range2·2–23·8 years) (Zimmerman & Ware, ).…”

Section: Spleen Dysfunction In Hbsc Diseasementioning

confidence: 99%

The spleen and sickle cell disease: the sick(led) spleen

2014

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Summary The spleen has a combined function of immune defence and quality control of senescent or altered red cells. It is the first organ injured in sickle cell anaemia (SCA) with evidence of hyposplenism present before 12 months in the majority of children. Repeated splenic vaso‐occlusion leads to fibrosis and progressive atrophy of the organ (autosplenectomy), which is generally complete by 5 years in SCA. The precise sequence of pathogenic events leading to hyposplenism is unknown. Splenic injury is generally silent and progressive. It can be clinically overt with acute splenic sequestration of red cells, an unpredictable and life‐threatening complication in infants. Splenomegaly, with or without hypersplenism, can also occur and can coexist with loss of function. Hyposplenism increases the susceptibility of SCA children to infection with encapsulated bacteria, which is notably reduced by penicillin prophylaxis and immunization. Whether hyposplenism indirectly increases the risk of vaso‐occlusion or other circulatory complications remains to be determined.

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“…In another study of 100 children with Hb SC disease, palpable splenomegaly was 34% (12). The median age was 11 years (range; 2–23.8 years).…”

Section: Discussionmentioning

confidence: 96%

Acute splenic complications and implications of splenectomy in hemoglobin SC disease

Subbannan

Üstün

Natarajan

et al. 2009

European J of Haematology

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Splenectomy indications and outcome were evaluated in 124 adults with hemoglobin SC disease (Hb SC). Twelve patients (9.6%) required splenectomy. There was a significant difference between the splenectomy group and the non-splenectomy group, respectively, regarding Hb levels (median 7.2 g/dL vs. 12.5 g/dL, P < 0.0001), platelet counts (median 146 x 10(6)/L vs. 275 x 10(6)/L, P = 0.031), palpable spleen rate (66% vs. 16%, P = 0.0003%), acute chest syndrome frequency (75% vs. 12%P = 0.0004) and cholecystectomy rate (66% vs. 13%, P = 0.0004). No significant morbidity or mortality occurred postsplenectomy. There is a subgroup of Hb SC patients requiring splenectomy, in which splenectomy is effective. Although it appears to be safe regarding short-term complications of surgery, long-term adverse effects such as infections have to be evaluated cautiously.

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Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations

Cited by 19 publications

References 20 publications

Outcome in hemoglobin SC disease: A four‐decade observational study of clinical, hematologic, and genetic factors

Outcome in hemoglobin SC disease: A four‐decade observational study of clinical, hematologic, and genetic factors

The spleen and sickle cell disease: the sick(led) spleen

Acute splenic complications and implications of splenectomy in hemoglobin SC disease

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