Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| hypersplenism |
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| Attestation |
3
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| Part of speech |
Noun
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| Grammatical label |
Countable
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| Definition |
A condition or group of conditions where the haemolytic activity (red blood cell destruction) of the spleen is increased. The spleen is often enlarged. Splenic enlargement generally is first suspected by physical examination. For children to have a palpable spleen on physical exam is not unusual and may be a normal finding. The normal palpable spleen tip is soft, smooth, and nontender. A pathologically enlarged spleen often is firmer, may have an abnormal surface or texture, and frequently is associated with other signs and symptoms of the underlying disease process. When the spleen tip has enlarged beyond 1-2 cm below the left costal margin, further evaluation should be considered. Children with splenomegaly are at increased risk of splenic injury. The American Academy of Pediatrics Committee on Sports Medicine has recommended restriction on contact sports in the presence of splenomegaly. The logic of the recommendation is that the spleen, which is exposed below the protection of the rib cage, is more susceptible to trauma. The typical therapy for hypersplenism is splenectomy. Despite the numerous causes of splenomegaly the spleen rarely is the primary site of disease. The most common mechanism of splenomegaly in children is hyperplasia of the MPS, which can be categorized as excessive antigenic stimulation (ie, infection), disorders of immunoregulation (ie, autoimmune disorders), or excessive destruction of abnormal blood cells (ie, hemolysis).
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| Definition source |
E-Medicine - Instant Access to the Minds of Medicine.
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| Context |
Splenectomy helps in prolongation of the time interval required between subsequent transfusions, when hypersplenism occurs.
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| Context source |
Khalifa, AS., et al. (1998). ‘T cell functions in infants and children with beta-thalassemia’. Acta Haematologica 79(3):153-6. (RISCEN159)
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| Synonym |
Splenomegaly
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| Subject field |
Haemopoiesis
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| Sub-field (level 1) |
Thalassemias
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| Related concept |
Splenectomy, iron overload, transfusional therapy, hypervolemia
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| it |
Ipersplenismo
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| Reliability code |
3
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