Long-term management of splenic sequestration in children with sickle cell disease

Kinney, Thomas R.; Ware, Russel E.; Schultz, William H.; Filston, Howard C.

doi:10.1016/s0022-3476(05)80529-3

Cited by 75 publications

(49 citation statements)

References 14 publications

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“…A large spleen predisposes the patient to the risks of sequestration crises, hypersplenism, infarction, and abscess formation. The splenic sequestration crises can be recurrent in about 50% patients [12][13][14][15][16][17][18]29]. In the present study, 88.6% patients had multiple splenic sequestration crises of minor type, and all the patients had significant hematological abnormality representing hypersplenism with BTdependent maintenance of hematological parameters in 95.4% patients.…”

Section: Discussionmentioning

confidence: 47%

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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Sickle cell disease displays a unique progression in the Eastern province of Saudi Arabia, where splenomegaly with hypersplenism is noted with high frequency in the adolescent and adult patients. The late persistence of splenomegaly although likely reflects the milder progression of sickle cell disease in this region; nevertheless, it predisposes the patients to increased morbidity. The present study documents the characteristic clinicopathological features of splenomegaly associated with sickle cell disease in the Al-Hassa region of Eastern province Saudi Arabia. Forty-four cases of sickle cell disease patients in whom splenectomy was performed during 1999-2003 were studied. The hemoglobinopathy profiles of the patients (age range 5-42 years) comprised sickle cell anemia (8 cases), sickle cell anemia with high fetal hemoglobin (23 cases), and sickle cell-b thalassemia (13 cases). All patients had manifestations of hypersplenism and 39 patients experienced episodes of minor-type sequestration crisis. Splenectomy was effective in ameliorating the hematological abnormalities in all cases, without any major complications in the follow-up period. The splenectomy specimens showed moderate-to-marked enlargement in most cases, with histological features of fibrocongestive splenomegaly and prominent Gandy-gamma body formations. Micro-infarcts in 27 cases and gross infarctions in 9 cases were evident. The relationship of persistent splenomegaly with higher fetal hemoglobin levels and splenic hypofunction is examined along with the significance of splenectomy in these cases. Am.

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Section: Discussionmentioning

confidence: 47%

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Chopra¹,

Al-Mulhim²,

Al-Baharani³

2005

American J Hematol

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“…In addition, when splenectomy is needed, a temporary blood transfusion programme enables the procedure to be postponed to an age when the susceptibility to pneumococcal infection is lower. Blood transfusion per se has not been proven to reduce or prevent the risk of ASSC recurrence (Kinney et al, 1990;Owusu-Ofori & Riddington, 2002). In addition, transfusions are associated with complications.…”

Section: Discussionmentioning

confidence: 99%

Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients

et al. 2012

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SummaryAcute splenic sequestration crisis (ASSC) is an unpredictable life-threatening complication of sickle cell disease (SCD) in infants. Here, our objective was to update available clinical information on ASSC. We retrospectively studied the 190 patients who were diagnosed at birth with SS or Sbeta 0 in the Paris conurbation between 2000 and 2009 and who experienced ASSC. They had 437 ASSC episodes (0AE06/patient-year). Median age at the first episode was 1AE4 years (0AE1-7) and 67% of patients had more than one episode. Age was the only factor predicting recurrence: the risk was lower when the first episode occurred after 2 years versus before 1 year of age (hazard ratio, 0AE60; 95% confidence interval, 0AE41-0AE88; P = 0AE025). A concomitant clinical event was found in 57% of episodes. The mortality rate was 0AE53%. The treatment consisted in watchful waiting without prophylactic blood transfusions or splenectomy in 103 (54%) patients and in a blood transfusion programme in 55 (29%) patients. Overall, splenectomy was performed in 71 (37%) patients, at a median age of 4AE5 years (range, 1AE9-9AE4). In conclusion, aggressive treatment may be warranted in patients experiencing ASSC before 2 years of age. Randomized controlled trials are needed to define the best treatment modalities.

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“…As opções são: observação clínica atenta, esquema de transfusões periódicas de concentrado de hemácias e esplenectomia. Os estudos clínicos sobre o SEA são retrospectivos, descritivos, não-controlados e levantam algumas questões sobre a real função esplênica após os episódios, além de vantagens e desvantagens de esplenectomia comparadas às transfusões crônicas 3,11,13,20,21 . .…”

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Acute splenic sequestration in a cohort of children with sickle cell anemia

Rezende¹,

Viana²,

Murao³

et al. 2009

J Pediatr (Rio J)

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ResumoObjetivo: Analisar o sequestro esplênico agudo (SEA) em crianças com anemia falciforme, provindas da triagem neonatal de Minas Gerais e acompanhadas pelo Hemominas de Belo Horizonte (MG). Conclusões: O SEA é um evento comum na anemia falciforme, principalmente nos 2 primeiros anos de vida, com recidiva em mais da metade dos casos. Predominou conduta conservadora na indicação da esplenectomia. Embora a letalidade tenha sido baixa, o SEA representou a segunda causa de óbito. Isso aponta para fragilidades estruturais do sistema de saúde de MG e para a necessidade de melhor capacitação profissional na abordagem do problema. Métodos J Pediatr (Rio J) AbstractObjective: To analyze acute splenic sequestration (ASS) in children with sickle cell anemia diagnosed through a newborn screening program in the state of Minas Gerais, Brazil, and followed up at the hematology center in the city of Belo Horizonte, Minas Gerais, Brazil. Results: A total of 89 patients had 173 episodes of ASS (10.2 first episodes per 100 patient-years); 75% of the first episodes occurred before 2 years of age. The estimated probability of occurrence of the first episode of ASS during the study period was 40%. Recurrence rate reached 57.3%. After the first episode, splenectomy was indicated in only 12.4% of the cases; after the second, in 60.4% of the cases. After the third episode, 41.7% of the patients remained under clinical observation. The median time between indication for splenectomy and the actual surgical procedure was 2 months. During the intervening period, 37.2% of the children suffered a new episode of ASS and one child died. Case-fatality rate was 1.1% for the first episode and 7.8% for the subsequent episodes. Among a total of 255 children, 19 died: 36.8% due to infections and 26.3% after ASS. MethodsConclusions: ASS is relatively common in sickle cell anemia, mainly in the first 2 years of life; relapse occurs in more than half of the cases. Conservative management instead of immediate splenectomy was the method of choice. Although the case-fatality rate was low, ASS was the second most common cause of death. These results disclose some fragilities of the health system in the state of Minas Gerais and the need for better professional education to approach ASS crises.J Pediatr (Rio J). 2009;85(2):163-169: Sickle cell anemia, acute splenic sequestration, children, splenectomy, newborn screening, mortality. Artigo submetido em 15.12.08, aceito em 04.02.09.

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Long-term management of splenic sequestration in children with sickle cell disease

Cited by 75 publications

References 14 publications

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Fibrocongestive splenomegaly in sickle cell disease: A distinct clinicopathological entity in the Eastern province of Saudi Arabia

Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients

Acute splenic sequestration in a cohort of children with sickle cell anemia

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