Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| splenectomy |
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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 |
An increased blood requirement due to hypersplenism accelerates iron loading, increases the risk of transfusion-mediated infection, and imposes an additional psychosocial burden on the patient and family. The majority of patients with homozygous Thalassaemia require splenectomy at some stage, but high transfusion retards or prevents hypersplenism, and splenectomy is now less frequent and is performed later than in the past. The following should be taken into account in deciding for or against splenectomy. • In Thalassaemia the evolution of hypersplenism starts with increased destruction of red blood cells. Presence of leucopenia or thrombocytopenia is an absolute indication for splenectomy, as these “classical” signs indicate very advanced hypersplenism. •An annual blood requirement of 1.5 times (or over 1.5 times) that in splenectomized patients on the same transfusion scheme, is the accepted indication for splenectomy. For patients maintained with a mean Hb of around 12 g/dL, this represents a pure red cell consumption of more than 200 mL/kg/yr. • A spleen palpable more than 6 cm below the costal margin is rarely harmful. • Splenectomy significantly increases the risk of serious infection, and should be postponed to after the fifth year of age, when possible. If splenectomy is indicated, the risk of serious subsequent infection can be reduced by the following steps. • Immunization against pneumococcal, meningococcal and haemophilus influenzae B infection, 4-6 weeks prior to splenectomy. •Prescription of prophylactic antibiotics for at least 2 years. Some doctors recommend them until the patient has left school, or even for life. As a rule, oral penicillin is prescribed in the appropriate prophylactic dose, usually 250 mg bd. • Informing patients, parents and their family doctors that the onset of a fever can mark the beginning of a serious infection in splenectomized subjects.
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| Definition source |
Cao, A., et al. (1997). Management Protocol for the treatment of Thalassemia patients. Nicosia: Thalassemia International Federation. (LISCEN01)
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| Context |
Before discussing the problem of postsplenectomy sepsis, it is important to identify the common indications for splenectomy in thalassemia intermedia. Splenectomy is generally undertaken in patients with thalassemia intermedia who experience weakness or chronic fatigue due to their anemia. In addition, patients with impaired growth or development may benefit from splenectomy. The appearance of early changes in the facial bones or the finding of cortical thinning in the long bones due to expansion of the bone marrow may warrant splenectomy as a means of raising the hemoglobin level, and perhaps decreasing the amount of erythropoietic activity. A similar rationale for splenectomy may apply to patients with massive splenomegaly or extramedullary hematopoiesis.
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| Context source |
AA.VV. (1997). ‘Thalassemia Intermedia. A Region I Conference’. The Genetic Resource Special Issue, 1997, Volume 11, Number 2 (COSCEN01)
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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 |
Hepatosplenomegaly, hypersplenism, iron overload, iron-chelation therapy, iron-chelating agent
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| it |
Splenectomia
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| Reliability code |
3
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