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Abdul Mannan: Antiphospholipid Syndrome in Thrombotic Storm Mode

Abdul Mannan/LinkedIn

Jun 16, 2026, 14:09

Abdul Mannan: Antiphospholipid Syndrome in Thrombotic Storm Mode

Abdul Mannan, Consultant Haematologist at Betsi Cadwaladr University Health Board, shared a post on LinkedIn:

“Most thrombosis cases give you time to think.

CAPS does not.

Catastrophic antiphospholipid syndrome, or Asherson syndrome, is what happens when APS stops behaving like a large-vessel event and turns into a thrombotic storm hitting three or more organs within days.

It accounts for roughly 1% of all APS cases.

Historically, nearly half of patients died. With current triple therapy, mortality has improved, but it still sits around 30%. Not a condition to misread.

Here is what makes it different from classic APS:

Large-vessel events, DVT, PE, or stroke, are not the main feature
Small-vessel microangiopathy drives the organ damage
Brain, lungs, kidneys, and skin are all in the firing line simultaneously

Infection and anticoagulation interruption are the two most common triggers

Diagnosis follows the Asherson Consensus Criteria:

Three or more organs involved
Onset within a week
Small-vessel occlusion on histology or imaging
Positive aPL

But here is the clinical reality: patients can die before all four boxes are ticked. Probable CAPS is a real category.

Treat it as such.

The core treatment is triple therapy:

Anticoagulation with unfractionated heparin.
High-dose glucocorticoids.
Plasma exchange and/or IVIG.
The trigger, almost always infection or a lapse in anticoagulation, has to be treated at the same time, not after.

For refractory disease, rituximab and eculizumab have both been used.

Neither has RCT-level evidence, but the International CAPS Registry has documented their use in selected cases.

One more thing: CAPS mimics TTP, HUS, DIC, and HIT. Distinguish them early. The treatments are very different.

Do not wait for a complete diagnostic picture before starting treatment.

In CAPS, time is tissue.

Have you managed a case of CAPS, or encountered a mimic that slowed you down?”

Abdul Mannan: Antiphospholipid Syndrome in Thrombotic Storm Mode