III – Disorders of the Red Cell
Membrane
Hereditary Spherocytosis (HS)
Definition:
Is a relatively common autosomal dominant
condition in which the red cell membrane is abnormally permeable , allowing the
erythrocytes to “swell –up “ into a spherical shape .
The spherocytes are readily recognizable in the
blood smear , have abnormal osmotic fragility (they are unable to swell any
further and rupture when placed in hypotonic solutions)
Are prematurely destroyed in the spleen .
Are prematurely destroyed in the spleen .
. HS results in a haemolytic anaemia of variable severity .
. Associated with intermittent jaundice and
splenomegaly .
. Exacerbation of
haemolysis may occur with infection , and crises occasionally aplastic crises
may develop .
. Chronic haemolytic"Arial","sans-serif"; mso-ascii-font-family: Calibri; mso-ascii-theme-font: minor-latin; mso-bidi-font-family: Arial; mso-bidi-language: AR-EG; mso-bidi-theme-font: minor-bidi; mso-hansi-font-family: Calibri; mso-hansi-theme-font: minor-latin;">
jaundice leads to gall stone formation .
Treatment
Haemolysis can be prevented , and the condition
effectively “ cured” by splenectomy , risks of overwhelming infection may be
reduced by avoiding splenectomy in early childhood , the administration of
polyvalent penumococcal vaccine and giving long-term prophylactic penicillin
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