105.2 ð åèçïŒé«åž«åè / PGY OSCEïŒ
105.2.0.1 ð Cram Sheet
105.2.0.1.1 ð¥ é« yield 12
- Hemolysis markers: retic â, LDH â, indirect bili â, haptoglobin â
- G6PD: X-linked; bite cells + Heinz bodies; primaquine/dapsone/sulfa/fava
- HS: AD; spherocytes + MCHC â; splenectomy curative
- Warm AIHA: DAT IgG+; SLE/CLL/Hodgkin/drug; steroid + rituximab
- Cold AIHA: DAT C3+; lymphoplasmacytic, mycoplasma, EBV; sutimlimab (FDA 2022)
- TTP: ADAMTS13 < 10%; pentad (FAT RN); PEX + caplacizumab + rituximab
- TTP: äž transfuse platelet (worsens)
- HUS typical: STEC E. coli O157:H7; bloody diarrhea; avoid antibiotic
- aHUS: complement; eculizumab/ravulizumab
- PNH: PIG-A â CD55/CD59 def; intravascular hemolysis + thrombosis + AA
- PNH treatment: eculizumab/ravulizumab + iptacopan oral 22E
- Neisseria vaccine before complement inhibitor
105.2.0.2 â é« yield
105.2.0.2.1 Coombs Test
| DAT (direct) | IAT (indirect) | |
|---|---|---|
| Detects | Ab on RBC | Free Ab in serum |
| Use | Immune hemolysis dx | Transfusion compatibility, HDN |
105.2.0.2.2 AIHA Comparison
| Warm | Cold | |
|---|---|---|
| Temp | 37°C | 4°C |
| Ab class | IgG | IgM |
| DAT | IgG+ (± C3) | C3+ |
| Site of hemolysis | Spleen (extravascular) | Liver (extravascular) + intravascular |
| Causes | Lymphoma (CLL, Hodgkin), SLE, drug, idiopathic | Lymphoplasmacytic, Mycoplasma, EBV |
| Treatment | Steroid + rituximab | Avoid cold + rituximab + sutimlimab |
105.2.0.2.3 Hemolytic MAHA Differential
| TTP | HUS | DIC | aHUS | HELLP | |
|---|---|---|---|---|---|
| ADAMTS13 | < 10% | normal | normal | normal | normal |
| Coags | normal | normal | abnormal | normal | varies |
| Renal | mild-moderate | severe | varies | severe | varies |
| Neuro | severe | mild | varies | mild | varies |
| Trigger | autoimmune | STEC | sepsis/cancer | complement | pregnancy |
| Treatment | PEX + caplacizumab | supportive (avoid abx) | treat cause | eculizumab | deliver baby |
105.2.0.3 ð¯ èªææª¢æž¬
- Hemolysis markers 4? â Retic, LDH, indirect bili, haptoglobin
- G6PD smear? â Bite cells + Heinz bodies
- HS smear? â Spherocytes + MCHC â
- HS curative? â Splenectomy
- Warm AIHA DAT? â IgG+
- Cold AIHA DAT? â C3+
- Cold AIHA new drug? â Sutimlimab (FDA 2022)
- TTP ADAMTS13? â < 10%
- TTP treatment 3? â PEX + caplacizumab + rituximab
- TTP plt transfusion? â äž (worsens unless life-threatening bleed)
- STEC-HUS antibiotic? â é¿çš (worsens)
- aHUS treatment? â Eculizumab/ravulizumab
- PNH genetics? â PIG-A â CD55/59 deficient
- PNH new oral? â Iptacopan
- Pre-eculizumab vaccine? â Neisseria meningitidis
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