Differential Diagnosis of Catastrophic Antiphospholipid Syndrome
CAPS | TTP | HUS | HELLP | Malig HBP | SLE Flare | Acute DIC | |
|---|---|---|---|---|---|---|---|
| Thrombocytopenia | 60% | +++< 20,000 | ++> 20,000 | ++> 20,000 | + | +/– Immune | ++/+++ |
| Hemolytic anemia | 33% | +++ Schistos >3%–5% | +++ Schistos >3%–5% | ++ Schistos >1%–2% | +/– Few Schistos | Coombs + | +/++ Few Schistos |
| Fever | — | 30% | — | — | — | +/– | — |
| Thrombosis and/or sxs | Diffuse | CNS 66% Renal 50% | Renal 100%, N/V | Liver 100%, N/V, HA | CNS, renal sxs | Many sxs possible | None |
| PT/PTT | NI/LA + | NI/NI | NI/NI | NI/NI | NI/NI | Nl/LA + | Inc/Inc |
| Fibro/FDPs | NI/NI | NI/Inc | NI/Inc | NI/Inc | NI/NI | NI/NI | Dec/Inc |
| Other | + aPL abs | Low (5%) ADAMTS13; very high LDH (>1000) | High C5b-C9; very high LDH (>1000) | High LAEs and LDH (>600); preeclampsia 80% | Severe HBP 100% | Low complements, high anti-dsDNA abs | Low factors V and VIII |
aPL abs , Antiphospholipid antibodies; CAPS , catastrophic antiphospholipid syndrome; CNS , central nervous system; Dec , decreased; DIC , disseminated intravascular coagulation; ds , double-stranded; HBP , high blood pressure; FDP , fibrin degradation products; HUS , hemolytic uremic syndrome; Inc , increased; LA , lupus anticoagulant; LAE , liver enzymes; LDH , lactate dehydrogenase; Malig , malignant; NI, normal; N/V, nausea/vomiting; PT , prothrombin time; PTT , partial thromboplastin time; Schistos , schistocytes; SLE , systemic lupus erythematosus; sxs , symptoms; TTP , thrombotic thrombocytopenia purpura. fibro, fibrinogen.
