Chokri Ben Lamine: CAPS After Autologous SCT – Diagnostic Criteria and Key Mimics
Chokri Ben Lamine, Hematologist at King Faisal Specialist Hospital and Research Center, shared a post on X:
“Catastrophic antiphospholipid syndrome (CAPS) after autologous SCT
Suspect with rapid multiorgan thrombosis/ischemia with or without thrombocytopenia and hemolysis. Post-transplant organ failure or a positive aPL test alone does not establish CAPS!
Definite CAPS: all 4 classification criteria
- Involvement of at least 3 organs/systems/tissues.
- Manifestations develop simultaneously or within 1 week.
- Histology confirms small-vessel thrombosis in ≥1 organ/tissue.
- Antiphospholipid antibodies (aPL) detected.
Probable CAPS includes compatible presentations lacking biopsy confirmation or involving only 2 organs; other defined combinations exist. Classification criteria support clinical judgment—do not delay urgent treatment for biopsy or repeat serology.
Immediate workup
- aPL panel: lupus anticoagulant – dRVVT and LA-sensitive aPTT with confirmatory testing; anticardiolipin IgG/IgM; anti-β₂-glycoprotein I IgG/IgM.
- Draw before anticoagulation/plasma exchange if feasible without delaying care. Anticoagulants can distort LA testing; acute infection may produce transient aPL.
- Repeat aPL at least 12 weeks later to establish persistence. Historical CAPS criteria used at least 6 weeks.
- CBC, smear/schistocytes, reticulocytes, LDH, bilirubin, haptoglobin with or without DAT.
- PT/INR, aPTT, fibrinogen, D-dimer; creatinine/electrolytes, liver profile, urinalysis and urine protein/creatinine.
- Blood cultures and targeted infection testing; review drugs, heparin exposure and prior thrombosis/APS.
Document organ involvement
- Renal: BP, AKI, proteinuria.
- Pulmonary: oxygenation; CT pulmonary angiography when indicated.
- Neurological: CT/MRI for focal deficits, seizures or encephalopathy.
- Cardiac: ECG, troponin, echocardiography.
- Doppler/CT angiography according to suspected thrombosis.
- Biopsy an accessible affected site only when safe.
Critical post-auto-SCT mimics
- TA-TMA: hypertension, proteinuria, increased LDH, schistocytes with or without AKI; consider soluble C5b-9 where available.
- TTP: urgent ADAMTS13 activity/inhibitor—sample before plasma therapy; activity less than 10% strongly supports TTP.
- Sepsis/DIC, HIT when clinically plausible, sinusoidal obstruction syndrome, drug-related or complement-mediated TMA.
Exam mnemonic: ‘3–1–H–A’
- 3 organs
- 1 week
- Histology
- aPL
Strong suspicion – urgent hematology/ICU/rheumatology review. CAPS usually requires therapeutic heparin plus glucocorticoids plus plasma exchange and/or IVIG, individualized for bleeding risk, while treating the trigger.”
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