Cryofibrinogenemia
ICD-10 D89.1 · ICD-11 EF5Y

Treatment of Cryofibrinogenemia in the Absence of Cryoglobulinaemia and No Identifiable Secondary Cause

Clinical Scenario

This protocol addresses cryofibrinogenemia confirmed in the specific setting where cryoglobulinaemia has been excluded, no secondary underlying cause of cryofibrinogenaemia has been identified, and there is no evidence of other vaso-occlusive disease.

Absence of cryoglobulinaemia No secondary cause No other vaso-occlusive disease
Primary Clinical Goal
Healing of skin ulcers
Treatment Approach

An apheresis-based intervention is among the approaches described for this condition; the full protocol also covers what is indicated for refractory presentations. Complete options, sequencing, and all clinical details are in the structured regimen below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1093/rheumatology/kew379

Absence of cryoglobulinaemia. No secondary cause of cryofibrinogenaemia. No evidence of other vaso-occlusive disease.

In patients with secondary CF, effective management of the underlying disease is usually sufficient to achieve improvement, while approaches to treatment for essential CF in reported patients were variable and ranged from avoidance of cold exposure to aggressive immunosuppression and plasmapheresis.

Recently, healing of ulcers with colchicine and high-dose pentoxyfiline has been reported in one patient with CF who remained ulcer free for 2 years taking this combination.

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