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14 Cards in this Set
- Front
- Back
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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 with pneumocc 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
confirm test |
per smear that show spherocytosis
confirm by fragility test |
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splenectomy for what patients
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moderate to severe spherocytosis or refractory to medical management
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what is more common long term complication in spherocytosis
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overwhelming sepsis with encapsulated bacteria most commonly Srept pneumonia aand risk is up to 30 years
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what is for prophylaxis of sepsis after splenectomy
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penicillin every day after vaccination for 3- 5 years( or life time long)
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what is Cooms test in sprocytosis
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negative ( different from auto im anemia wher eit is positive)
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