Cryofibrinogenemia - Revision history

Aidan: linkified

2020-08-28T17:50:02Z

linkified

← Older revision		Revision as of 17:50, 28 August 2020
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	== 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 Manifestations ==		==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]]		[[Category:Hematology]]

Aidan: Text replacement - "Clinical Presentation" to "Clinical Manifestations"

2020-07-20T19:36:13Z

Text replacement - "Clinical Presentation" to "Clinical Manifestations"

← Older revision		Revision as of 19:36, 20 July 2020
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	* 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

Maintenance script: Imported from text file

2020-07-04T01:17:29Z

Imported from text file

New page

== 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 hepatitides
** Autoimmune
*** Systemic lupus erythematosus
*** Sjögren syndrome
*** Rheumatoid arthritis
*** mixed CTD
*** Polymyositis
*** Dermatomyositis
*** Systemic sclerosis
*** Antiphospholipid syndrome
*** Hashimoto and Graves diseases
*** Sarcoidosis
*** Pyoderma gangrenosum
*** Psoriasis
*** Spondyloarthropathy
*** Inflammatory bowel disease
** Vasculitis
*** ANCA-associated vasculitides
*** 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 Presentation ==

* 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
** Immunosupressing medications
*** Azathrioprine
*** Chlorambucil (in addition to corticosteroids)

[[Category:Hematology]]