Cryofibrinogenemia: Difference between revisions

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)

@@ Line 1: / Line 1: @@
-== Definition ==
+==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
+*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 ==
+==Etiology==
-* Primary
+*Primary
-** About half eventually develop lymphoma
+**About half eventually develop [[lymphoma]]
-* Secondary
+*Secondary
-** Cancer, usually lymphoma
+**[[Cancer]], usually [[lymphoma]]
-** Infection
+**Infection
-*** Bacterial
+***Bacterial
-*** Viral, including HIV, herpesviruses, and hepatitides
+***Viral, including [[HIV]], [[herpesviruses]], and [[viral hepatitis]]
-** Autoimmune
+**Autoimmune
-*** Systemic lupus erythematosus
+***[[Systemic lupus erythematosus]]
-*** Sjögren syndrome
+***[[Sjögren syndrome]]
-*** Rheumatoid arthritis
+***[[Rheumatoid arthritis]]
+***[[Mixed connective tissue disease]]
-*** mixed CTD
-*** Polymyositis
+***[[Polymyositis]]
-*** Dermatomyositis
+***[[Dermatomyositis]]
-*** Systemic sclerosis
+***[[Systemic sclerosis]]
-*** Antiphospholipid syndrome
+***[[Antiphospholipid syndrome]]
-*** Hashimoto and Graves diseases
+***[[Hashimoto disease|Hashimoto]] and [[Graves disease|Graves diseases]]
-*** Sarcoidosis
+***[[Sarcoidosis]]
-*** Pyoderma gangrenosum
+***[[Pyoderma gangrenosum]]
-*** Psoriasis
+***[[Psoriasis]]
-*** Spondyloarthropathy
+***[[Spondyloarthropathy]]
-*** Inflammatory bowel disease
+***[[Inflammatory bowel disease]]
-** Vasculitis
+**[[Vasculitis]]
-*** ANCA-associated vasculitides
+***[[ANCA-associated vasculitis]]
-*** Giant cell arteritis
+***[[Giant cell arteritis]]
-*** Behçet disease
+***[[Behçet disease]]
-*** Polyarteritis nodosa
+***[[Polyarteritis nodosa]]
-*** Henoch-Schönlein purpura
+***[[Henoch-Schönlein purpura]]
-** Cryoglobulinemia
+**[[Cryoglobulinemia]]
-== Pathophysiology ==
+==Pathophysiology==
-* Cryofibrinogen is composed of fibrinogen, fibronectin, and fibrin, with or without albumin and immunoglobulins
+*Cryofibrinogen is composed of fibrinogen, fibronectin, and fibrin, with or without albumin and immunoglobulins
-== Clinical Presentation ==
+==Clinical Manifestations==
-* Cold intolerance
+*Cold intolerance
-* Skin findings
+*Skin findings
-** Raynaud phenomenon
+**Raynaud phenomenon
-** Purpura
+**Purpura
-** Livedo reticularis
+**Livedo reticularis
-** Skin necrosis
+**Skin necrosis
-** Acral ulcers
+**Acral ulcers
-** Gangrene
+**Gangrene
-* Arterial and venous thromboses
+*Arterial and venous thromboses
-== Investigations ==
+==Investigations==
-* Cryofibrinogen, returns as a percentage
+*Cryofibrinogen, returns as a percentage
-** Detectable in up to 7% of healthy patients
+**Detectable in up to 7% of healthy patients
-== Management ==
+==Management==
-* Mild
+*Mild
-** Cold avoidance
+**Cold avoidance
-* Moderate
+*Moderate
-** Corticosteroids
+**[[Corticosteroids]]
-** Aspirin
+**[[Aspirin]]
-** Alternatively, stanozolol 2 to 4mg BID (an anabolic steroid)
+**Alternatively, [[stanozolol]] 2 to 4mg BID (an anabolic steroid)
-* Severe
+*Severe
-** Plasmapheresis
+**[[Plasmapheresis]]
-** Immunosupressing medications
+**Immunosuppressing medications
-*** Azathrioprine
+***[[Azathrioprine]]
-*** Chlorambucil (in addition to corticosteroids)
+***[[Chlorambucil]] (in addition to [[corticosteroids]])
 [[Category:Hematology]]