Red-Blood-Cell Survival After Splenectomy in Congenital Spherocytosis

Baird, Robin; Macpherson, Allan; Richmond, John C.

doi:10.1016/s0140-6736(71)90380-1

Cited by 25 publications

(11 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In the first year of life, erythropoietin treatment may be beneficial and may lower transfusion requirements [27,28]. Splenectomy is very effective in reducing hemolysis, leading to significant prolongation (although not necessarily to normal) of the red cell lifespan [29,30]. Splenectomy should be performed in children with severe HS, considered in those who have moderate disease, and probably not performed in those with mild disease [14,26].…”

Section: Discussionmentioning

confidence: 99%

Hereditary Spherocytosis: Evaluation of 68 Children

Konca

Söker

Taş

et al. 2014

Indian J Hematol Blood Transfus

View full text Add to dashboard Cite

To determine the clinical and hematologic features of 68 children with hereditary spherocytosis (HS). In this retrospective study, we analyzed recorded information of 68 HS patients diagnosed between March 1997 and March 2007, including clinical manifestations at admission, gender, median age at diagnosis, family history, hematologic and biochemical data, patient management, complications, median age of splenectomy, and median follow-up time. Sixty-eight patients with HS (36 male and female) were investigated. The median age at diagnosis was 5.6 years (range 3 months to 18 years). Twenty-seven (39.7 %) had parents with consanguineous marriages, and 20 (29.4 %) had parents with first-degree consanguinity. Predominant clinical manifestations at admission were anemia in 59 patients (86.76 %), splenomegaly in 49 (72.05 %), and jaundice in 33 (48.52 %). Patients were classified as mild, moderate, or severe in 29.4, 61.7, and 8.8 % of patients, respectively. Five patients (7.3 %) underwent splenectomy. Major complications of HS were hemolytic, aplastic, and megaloblastic crises and cholelithiasis in 7 (10.2 %), 1 (1.4 %), 7 (10.2 %), and 6 (8.8 %) of patients, respectively. There were no deaths during follow-up. HS should be considered in evaluating possible diagnoses in patients with hemolytic anemia. In this study, the clinical course of patients with HS was relatively benign, with low proportions of patients having splenectomized and aplastic crises.

show abstract

Section: Discussionmentioning

confidence: 99%

Hereditary Spherocytosis: Evaluation of 68 Children

Konca

Söker

Taş

et al. 2014

Indian J Hematol Blood Transfus

View full text Add to dashboard Cite

show abstract

“…However, the hematologic improvement after subtotal splenectomy is less spectacular than that observed after total splenectomy. 23 A mild hemolytic state is still persistent. The occurrence of severe anemia in 4 patients with virus-related acute red cell aplasia illustrates the fact that the decrease in hemolysis after subtotal splenectomy is not of sufficient magnitude to compensate for a transient arrest in erythropoiesis.…”

Section: Discussionmentioning

confidence: 99%

Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis

Bader-Meunier

Gauthier

Archambaud

et al. 2001

Blood

145

112

View full text Add to dashboard Cite

Clinical manifestations of hereditary spherocytosis (HS) can be abrogated by splenectomy. However, concerns exist regarding exposure of patients to a lifelong risk for overwhelming infections and, to a lesser extent, to vascular complications after total splenectomy. In the search for alternative treatment modalities, we assessed, in a previous pilot study, the potential usefulness of subtotal splenectomy in a small population of patients. During a mean follow-up period of 3.5 years, subtotal splenectomy was shown to be effective in decreasing the hemolytic rate, while maintaining the phagocytic function of the spleen. In the current study, we evaluated the clinical and biologic features of 40 patients with HS who underwent subtotal splenectomy and were monitored for periods ranging from 1 to 14 years. The beneficial effect of subtotal splenectomy included a sustained decrease in hemolytic rate and a continued maintenance of phagocytic function of the splenic remnant. However, mild-to-moderate hemolysis was persistent and accounted for secondary gallstone formation and aplastic crisis in a small subset of patients. Surprisingly, regrowth of the remnant spleen did not seem to have a major impact on the beneficial outcomes of these individuals. Our results suggest that subtotal splenectomy appears to be a reasonable treatment option for management of patients with HS, especially young children.

show abstract

“…Thus in most HS patients, splenectomy cures the anemia, and decreases the incidence of cholelithiasis. 37 Even severe HS patients experience marked improvement in their anemia postsplenectomy. 12 Early complications of splenectomy include local infection, bleeding, and pancreatitis due to injury to the tail of the pancreas during surgery.…”

Section: Hereditary Spherocytosismentioning

confidence: 99%

Abnormalities of the Erythrocyte Membrane

Gallagher

2013

Pediatric Clinics of North America

View full text Add to dashboard Cite

Synopsis Primary abnormalities of the erythrocyte membrane, including the hereditary spherocytosis and hereditary elliptocytosis syndromes, are an important group of inherited hemolytic anemias. Classified by distinctive morphology on peripheral blood smear, these disorders are characterized by clinical, laboratory, and genetic heterogeneity. Among this group, hereditary spherocytosis patients are more likely to experience symptomatic anemia. Treatment of hereditary spherocytosis with splenectomy is curative in most patients. Once considered routine, growing recognition of the longterm risks of splenectomy, including cardiovascular disease, thrombotic disorders, and pulmonary hypertension, as well as the emergence of penicillin-resistant pneumococci, a concern for infection in overwhelming postsplenectomy infection, have led to re-evaluation of the role of splenectomy. Current management guidelines acknowledge these important considerations when entertaining splenectomy and recommend detailed discussion between health care providers, patient, and family. The hereditary elliptocytosis syndromes are the most common primary disorders of erythrocyte membrane proteins. However, most elliptocytosis patients are asymptomatic and do not require therapy.

show abstract

Red-Blood-Cell Survival After Splenectomy in Congenital Spherocytosis

Cited by 25 publications

References 8 publications

Hereditary Spherocytosis: Evaluation of 68 Children

Hereditary Spherocytosis: Evaluation of 68 Children

Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis

Abnormalities of the Erythrocyte Membrane

Contact Info

Product

Resources

About