Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| splenic sequestration |
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| Attestation |
3
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| Part of speech |
Noun syntagm
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| Grammatical label |
Countable
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| Definition |
Splenic sequestration is caused by intrasplenic trapping of red cells causing a precipitous fall in hemoglobin level and the potential for hypovolemic shock. With the recent decline in mortality from pneumococcal sepsis, SSC has become a leading cause of death in children with sickle cell disease. SSC may be defined by a decrease of at least 2 g/dL from the steady-state hemoglobin concentration, evidence of increased erythropoiesis such as a markedly elevated reticulocyte count, and an acutely enlarging spleen. SSC has been reported as early as 5 weeks of age and in adults, but in most cases first attacks occur between 3 months and 5 years of age. They are often associated with viral or bacterial infections; acute chest syndrome occurred in 20% in one series. The usual clinical manifestations are sudden weakness, pallor, tachycardia, tachypnea, and abdominal fullness. ASSC has been reported in 30% of children with sickle cell anemia in Jamaica and 7.5% of children seen at Duke University. The mortality rate for first attacks was 12% in Jamaica. Recurrent splenic sequestration crises are common, occurring in approximately 50% of those who survive the first episode, and the mortality rate in these patients may be 20%. There are no clear prognostic factors for the occurrence of ASSC, although the Hb F level at 6 months of age was somewhat lower in children who developed this complication. Although ASSC occurs most commonly among children with Hb SS, it has been reported in 5% of children with Hb SC disease at a mean age of approximately 9 years and in adults with Hb SC disease.
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| Definition source |
E-Medicine - Instant Access to the Minds of Medicine
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| Context |
There are now hints that it may be possible to predict the development of the vascular changes that lead to stroke.16 We have a much greater understanding of how to manage the chest syndrome and splenic sequestration. For the moment, it might be better to concentrate our efforts on improving the symptomatic care of patients with sickle cell anemia and continuing our studies of the factors that modify its phenotypic expression.
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| Context source |
Weatherall, D. (1992). ‘Bone marrow transplantation for thalassemia and other inherited disorders of hemoglobin’. Blood 80(6):1379-81. (RISCEN81)
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| Subject field |
Haemopoiesis
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| Sub-field (level 1) |
Thalassemias
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| Sub-field (level 2) |
Therapies
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| Related concept |
Iron overload
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| it |
Sequestro splenico
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| Reliability code |
3
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