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10 Cards in this Set
- Front
- Back
HEREDITARY SPHEROCYTOSIS
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HEREDITARY SPHEROCYTOSIS
This condition presents with the following: • Recurrent episodes • Splenomegaly • bilirubin gallstones • Elevated mean corpuscular hemoglobin concentration (MCHC) ( n 32- 36 g /dl |
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The most accurate test for hereditary spherocytosis
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The most accurate test for hereditary spherocytosis is an osmotic fragility test .
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WHAT will prevent hemolysis
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Splenectomy will prevent hemolysis since the cells are destoyed in the spleen.
Splenectomy helps in these patients |
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INHERITANCE
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AD
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clinical picture like in
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clinical picture like in autoimmune hemolytic anemia but anemia has + coombs test and dont have positive family history
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what is in def
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spectrin>>hemolysis when RBc pass trough spleen.... early splenic phasocytosis
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symptoms
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anemia
jundice splenomegly hemolysys in all ages worse after infection of exers 3 types of crisis 1. hemolytic 9 mild) 2 aplastic( severe-- caused by parvovirus) 3megaloblastic (with pregnancy) gallstones( bilitrubin) gout ankle ulcers |
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lab tests
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low Hct
elevated: MCHC LDH indirect bilirubin reticuloicytes ( like in other hemolysis) norman MCV fragility test + coombs test(-) important to disting between spherocytosis( -) and hem anemia(+) |
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tx
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splenectonmy for anemia or sign hemolysis
defer until age 6-7 because of sepsis potential after splenectony- vaccin witthpneumocc and HIB chronic folic acid replacement support cell production splenoectopmy stops the hemolysis but doesnot eliminate spherosytes maybe cholectomy of + for gallstones |
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best initial test
comfirm test |
per smear that show spherocytosis
confecm by fragility test |