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Membrane defect anemia- PNH, G6PD and hereditary spherocytosis
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Membrane defect anemia- PNH, G6PD and hereditary spherocytosis.
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Hematological disorder marachedefece of rises causes
by spectrin deficiency
So the membrane becomes Rigid RBCS trapped in smallcapillaries8splee
Permeable to Na of water RBCs takeshapeofsph
Iae
1 features of hemolytic Anemia in the family history in childhoodonse
IIet
Inves
i osmotic fragility Hemolysis occurs at dilution of 0.7 N at 0.4
11 MCHC microcytosis
splenectomy npacienesa.rs
n Tiffing
Hematological disorder charae
RBCS due to sensitivity to complement Cs system
ff.si
fmembranetefearf
Iae
1 Chronic hemolyticanemia overnight Hypoventilation Co2 Acidos
ask.fi iaI g mse bzi 19hamAs it Activatesoff
1 Ham zest ve Rapid cars is serum acities
11 ToConfirm Flow cytometric Absence of CD59
complical
s
I Progress to severe AplasticAnemia 11 5 Acute leukemia
11120 Thrombosis
Treatme
BM transplant t
11 steroid 111oral Anticoagulants for thrombosis
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