Intravascular Hemolysis¶
- Paraxsomal Nocturnal Hematuria(Chromosome X)
- G6PD (chromosome X)
- Auto-immune Hemolytic Anemia
Paroxysmal Nocturnal Hematuria¶
Paroxysmal - Some Night Nocturnal - at sleep
- Acquired X chromosome Deficiency of Gpi attachment protein
- Gpi attaches CD55(Decay activating Factor), CD59 (membrane inhibitor Reactive lysis) ➡️ deactivates Complement
- At night Shallow Breathing ➡️ Respiratory Acidois ➡️ Complement Actovation ➡️ Destruction of RBC,WBC and Platelets
- *M/C death thrombosis of Hepatic and CNS(Damaged platelets activating THrombosis)
- AML risk (acquired Mutation can cause Cancerous Mutation)
Labs¶
- Hemoglobunemia
- Hemoglubunria
- Hemosiderinuria(renal cells with absorbed iron necrosis)
Diagnosis¶
- Flow cytometry, FLARER to COnfirm
- Gel card test to screen
Treatment¶
- Eculizumab
G6PD¶
- X linked Recessive Disorder
- Reduction in RBC life
- Ocurs only on Predisposing Fctors that increse Free Radical injury
- free radical injury ➡️ HEinz body(Aggregation of Hb) ➡️ MAcrophages eat membrane ➡️ bite cells
LAbs¶
- Methamoglobin reduction test(Methamgobulin is reduced to normal Rbc in NADPH presence)
- NADPH glows in UV
Auto-immune hemolytic Anemai¶
||IgG|IgM| |—|—|—| ||Warn temperature|Cold temperture| ||P-antigen|I-Antigen | ||SLE,CLL,drugs like Penicillin|Mycoplasma Pneumoia,Mono-nucleosis|