Cryofibrinogenemia: Difference between revisions
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− | == |
+ | ==Definition== |
− | * |
+ | *Disease caused by cold-precipitating proteins present in the plasma but not serum (i.e. associated with the clotting factors) that is associated with skin manifestations and thrombosis |
− | == |
+ | ==Etiology== |
− | * |
+ | *Primary |
− | ** |
+ | **About half eventually develop [[lymphoma]] |
− | * |
+ | *Secondary |
− | ** |
+ | **[[Cancer]], usually [[lymphoma]] |
− | ** |
+ | **Infection |
− | *** |
+ | ***Bacterial |
− | *** |
+ | ***Viral, including [[HIV]], [[herpesviruses]], and [[viral hepatitis]] |
− | ** |
+ | **Autoimmune |
− | *** |
+ | ***[[Systemic lupus erythematosus]] |
− | *** |
+ | ***[[Sjögren syndrome]] |
− | *** |
+ | ***[[Rheumatoid arthritis]] |
+ | ***[[Mixed connective tissue disease]] |
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− | *** mixed CTD |
||
− | *** |
+ | ***[[Polymyositis]] |
− | *** |
+ | ***[[Dermatomyositis]] |
− | *** |
+ | ***[[Systemic sclerosis]] |
− | *** |
+ | ***[[Antiphospholipid syndrome]] |
− | *** Hashimoto and Graves diseases |
+ | ***[[Hashimoto disease|Hashimoto]] and [[Graves disease|Graves diseases]] |
− | *** |
+ | ***[[Sarcoidosis]] |
− | *** |
+ | ***[[Pyoderma gangrenosum]] |
− | *** |
+ | ***[[Psoriasis]] |
− | *** |
+ | ***[[Spondyloarthropathy]] |
− | *** |
+ | ***[[Inflammatory bowel disease]] |
− | ** |
+ | **[[Vasculitis]] |
− | *** |
+ | ***[[ANCA-associated vasculitis]] |
− | *** |
+ | ***[[Giant cell arteritis]] |
− | *** |
+ | ***[[Behçet disease]] |
− | *** |
+ | ***[[Polyarteritis nodosa]] |
− | *** |
+ | ***[[Henoch-Schönlein purpura]] |
− | ** |
+ | **[[Cryoglobulinemia]] |
− | == |
+ | ==Pathophysiology== |
− | * |
+ | *Cryofibrinogen is composed of fibrinogen, fibronectin, and fibrin, with or without albumin and immunoglobulins |
− | == |
+ | ==Clinical Manifestations== |
− | * |
+ | *Cold intolerance |
− | * |
+ | *Skin findings |
− | ** |
+ | **Raynaud phenomenon |
− | ** |
+ | **Purpura |
− | ** |
+ | **Livedo reticularis |
− | ** |
+ | **Skin necrosis |
− | ** |
+ | **Acral ulcers |
− | ** |
+ | **Gangrene |
− | * |
+ | *Arterial and venous thromboses |
− | == |
+ | ==Investigations== |
− | * |
+ | *Cryofibrinogen, returns as a percentage |
− | ** |
+ | **Detectable in up to 7% of healthy patients |
− | == |
+ | ==Management== |
− | * |
+ | *Mild |
− | ** |
+ | **Cold avoidance |
− | * |
+ | *Moderate |
− | ** |
+ | **[[Corticosteroids]] |
− | ** |
+ | **[[Aspirin]] |
− | ** |
+ | **Alternatively, [[stanozolol]] 2 to 4mg BID (an anabolic steroid) |
− | * |
+ | *Severe |
− | ** |
+ | **[[Plasmapheresis]] |
− | ** |
+ | **Immunosuppressing medications |
− | *** |
+ | ***[[Azathrioprine]] |
− | *** |
+ | ***[[Chlorambucil]] (in addition to [[corticosteroids]]) |
[[Category:Hematology]] |
[[Category:Hematology]] |
Latest revision as of 13:50, 28 August 2020
Definition
- Disease caused by cold-precipitating proteins present in the plasma but not serum (i.e. associated with the clotting factors) that is associated with skin manifestations and thrombosis
Etiology
- Primary
- About half eventually develop lymphoma
- Secondary
- Cancer, usually lymphoma
- Infection
- Bacterial
- Viral, including HIV, herpesviruses, and viral hepatitis
- Autoimmune
- Vasculitis
- Cryoglobulinemia
Pathophysiology
- Cryofibrinogen is composed of fibrinogen, fibronectin, and fibrin, with or without albumin and immunoglobulins
Clinical Manifestations
- Cold intolerance
- Skin findings
- Raynaud phenomenon
- Purpura
- Livedo reticularis
- Skin necrosis
- Acral ulcers
- Gangrene
- Arterial and venous thromboses
Investigations
- Cryofibrinogen, returns as a percentage
- Detectable in up to 7% of healthy patients
Management
- Mild
- Cold avoidance
- Moderate
- Corticosteroids
- Aspirin
- Alternatively, stanozolol 2 to 4mg BID (an anabolic steroid)
- Severe
- Plasmapheresis
- Immunosuppressing medications
- Azathrioprine
- Chlorambucil (in addition to corticosteroids)