The spleen in the sickling disorders: an update

  title={The spleen in the sickling disorders: an update},
  author={Rana Khatib and Raja Rabah and Sharada A. Sarnaik},
  journal={Pediatric Radiology},
In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study… 
Hematopoietic cell transplantation for sickle cell disease: updates and future directions.
  • L. Krishnamurti
  • Medicine, Biology
    Hematology. American Society of Hematology. Education Program
  • 2021
There is a need for a universal, longitudinal clinical registry to follow patients after HCT for SCD in conjunction with individuals who do not receive HCT to compare outcomes and the long-term impact of HCT on patients, families, and society.
Splenic morphological changes are accompanied by altered baseline immunity in a mouse model of sickle-cell disease.
Spleen and Peritoneal Cavity
An overview of normal splenic development and anatomy is presented, as well as a discussion of imaging techniques, anatomic variants, congenital anomalies, and the characteristic ultrasound imaging features of a broad spectrum of disorders that affect the spleen.
Carriage of encapsulated bacteria in Gabonese children with sickle cell anaemia.
  • F. Schaumburg, B. Biallas, A. Adegnika
  • Medicine, Biology
    Clinical microbiology and infection : the official publication of the European Society of Clinical Microbiology and Infectious Diseases
  • 2013
Carriage and resistance rates are similar in children with and without SCA, and data provide the basis to guide empiric therapy of invasive diseases caused by S. pneumoniae, Staph.
Current sickle cell disease management practices in Nigeria.
The care available for SCD in Nigeria is still suboptimal and there is an urgent need for concerted effort to tackle the problem, but to make a significant impact on the burden of the disease would require more focus at the primary care level.
The implementation of a web-based application to monitor daily pain levels is relevant to public health because it is likely to provide medical teams with the tools necessary to track patient pain levels more precisely and to enable healthcare providers to be more proactive with regard to pain management in patients with SCD.
Incidence of Sickle Cell Anaemia among Children Attending Maryam Abacha Women and Children Hospital, Sokoto
Improved knowledge regarding Sickle cell anaemia disease and its comprehensive care among Nigerian physicians will enhance quality of care for affected childrens and policy for regular genotype test by government and other stakeholders before marriage among Nigerians will help to prevent the disease.


[Splenic sequestration in patients with sickle cell disease. ].
Parents of infants and toddlers with sickle cell disease need to learn how to palpate the spleen in order to detect splenomegaly as early as possible and take the child to the hospital.
First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: splenic regeneration.
It is suggested that chronic HU therapy may reverse splenic dysfunction in certain patients and suggest that this drug may have efficacy beyond the elimination of pain in SCA.
Management of acute splenic sequestration crisis in sickle cell disease.
  • R. Grover, D. Wethers
  • Medicine
    Journal of the Association for Academic Minority Physicians : the official publication of the Association for Academic Minority Physicians
  • 1990
Surgery after the first episode of ASSC in the child 5 years of age and older and the choice of a year or more of long-term transfusion therapy for the child under 3 are recommended.
Acute chest syndrome in the postoperative sickle cell patient.
Infections in sickle cell anemia: pathogenesis and control.
Experience would suggest that pneumococcal vaccines may provide only modest protection in the child with SCD less than 5 years of age, and prophylactic antibiotic regimens have yet to be submitted to rigid scientific investigation.
Hyposplenism: A comprehensive review. Part I: Basic concepts and causes
In splenectomized, and functionally hyposplenic subjects, the pneumococcal conjugate vaccine is more effective, because it utilizes a T cell dependent mechanism, and should be the preferred vaccine in these circumstances.
Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.
The data indicate that BMT can correct "permanent asplenia" in SCA patients, however, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.
Complications of splenectomy.
  • C. Shatney
  • Medicine
    Acta anaesthesiologica Belgica
  • 1987
Surgeons are changing their attitudes concerning splenectomy for trauma, both accidental and iatrogenic, and nonoperative management of hemodynamically stable patients with isolated splenic injury and splenorrhaphy in patients requiring laparotomy are now firmly entrenched in the surgical armamentarium.
Hydroxyurea in Children with Sickle Cell Disease: Impact on Splenic Function and Compliance with Therapy
Hydroxyurea in children is associated with sustained excellent compliance and monitoring this compliance is uncomplicated, and splenic function in most hydroxyurea-treated children did not change over 1 year of therapy.