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PNH aquired membrane defect (Diagnosis (Blood Test, increased: LDH,…
PNH
aquired membrane defect
Symptoms
Addominal pain-> blood clots, dec Hb, dec.NO
Diff. swallowing/muscle spasm
Erectile dysfunction ->dec Hn, dec NO, dec. vasodialation
headaches, -> vasodialation
chest pain
fatigue->anemia
TRIAD
: Thombosis, hemolitic/intravascular anemia, pancytopenia
Diagnosis
Blood Test
increased: LDH, reticulocites
decreased: Hb, haptoglobin
Urine: Hemoglobinuria
Anemia: normo- or macrocytic
Un. Bilirubin
neutrophils: normal or below
platelets: normal or below
lymphocytes: normal or kymphopenia
Bone Marrow: hyperplasia
Flow cytr. Immunophenotype: loss of CD55/59
Treatment
HEMOLITYC
Asymptomatic
Supportive care + anticoagulation, folic acid, Fe suppl.
Symptomatic
Hemolityc crisis
Supportive care/anticoagulants: prednisone, warfrin, transfusion
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Eculizimab
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at risk for infection: Neisseria meningitidis
Chronic/PAIN management
Thrombus
APLASTIC
monitor every 6 months w/flow cytrometry
Allogenic BMT
ONLY curative therapy
high rate of mortality/morbidity
Pathology
Marrow Damage
HSC - Mutations of PIG A gene. Loss of surface GPI anchor protein CD55/CD59
Erythrocyte
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Increased sensitivity to C mediated lysis
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Intravascular hemolysis
Hemoglobinuria/Ph decrease
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Anemia
Platelet
Complement classic/alter, pathway
activation, Impaired fibrynolisis
Decreased platelets= aggregation+bleeding
Hypercoagulation
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Neutrophil
Neurophil Disease
Infection
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Thrombosis
: portal v. , hepatic v. cerebral v. sagital v. + arteries
Pancytopenia