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{{SK}}  Donath-Landsteiner syndrome;  PCH


==[[Paroxysmal cold hemoglobinuria overview|Overview]]==


'''Paroxysmal cold hemoglobinuria''' (PCH) (also known as '''Donath-Landsteiner syndrome''') is a rare disease that is associated with intravascular hemolysis and hemoglobinuria after exposure to cold. The Donath-Landsteiner antibody sticks to the red blood cells in the cold which causes a complement-mediated lysis upon rewarming.
==[[Paroxysmal cold hemoglobinuria historical perspective|Historical Perspective]]==


In people with PCH, a polyclonal [[IgG]] [[autoantibody]] binds to [[red blood cell]] surface antigens in the cold.  This can occur in a susceptible individual as blood passes through cold extremities in cold weather.  When the blood returns to the warmer central circulation, the red blood cells are lysed with [[Complement system|complement]], causing intravascular [[hemolysis]].  Hemoglobinuria and [[anemia]] can then occur.  The anemia may be mild or severe. 
==[[Paroxysmal cold hemoglobinuria classification|Classification]]==


PCH can be an after effect of an infection, when a microorganism triggers the formation of antibodies that cross-react with the P antigen on the red blood cell membrane.  Viral infections that can cause PCH include  [[measles]], [[mumps]], [[influenza]], [[Adenoviridae|adenovirus]], [[chickenpox]], [[cytomegalovirus]], and [[Epstein-Barr virus]].  Bacterial infections that can cause PCH include [[syphilis]], ''[[Haemophilus influenzae]]'' and ''[[Mycoplasma pneumoniae]]''.  PCH can also be a side effect of some vaccinations.  Chronic [[idiopathic]] (of unknown cause) PCH also occurs, but it is rare.
==[[Paroxysmal cold hemoglobinuria pathophysiology|Pathophysiology]]==


Acute PCH tends to be transient and self-limited, particularly in children.  Chronic PCH associated with syphilis resolves after the syphilis is treated with appropriate antibiotics.  Chronic idiopathic PCH is usually mild.
==[[Paroxysmal cold hemoglobinuria causes|Causes]]==


People with PCH should avoid exposure to cold temperatures.  If anemia is severe, [[blood transfusion]] may be needed.  Careful compatibility testing by the blood bank is necessary because autoantibodies may interfere with blood typing.  [[Prednisone]] may be used in individuals with PCH and severe anemia.
==[[Paroxysmal cold hemoglobinuria differential diagnosis|Differentiating Paroxysmal cold hemoglobinuria from other Diseases]]==


== Differential Diagnosis of Causes of {{PAGENAME}} ==  
==[[Paroxysmal cold hemoglobinuria epidemiology and demographics|Epidemiology and Demographics]]==


In alphabetical order: <ref>Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:77 ISBN 1591032016</ref> <ref>Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:68 ISBN 140510368X</ref>
==[[Paroxysmal cold hemoglobinuria risk factors|Risk Factors]]==


*[[Ddx:Anemia|Anemia]]
*[[Chicken Pox]]
*Congenital [[Syphillis]]
*[[Glandular fever]]
*[[Measles]]
*[[Mumps]]


==See also==
*[[Hemolytic anemia]]


==References==
==[[Paroxysmal cold hemoglobinuria screening|Screening]]==  
{{Reflist}}
 
==[[Paroxysmal cold hemoglobinuria natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
[[Paroxysmal cold hemoglobinuria history and symptoms| History and Symptoms]] | [[Paroxysmal cold hemoglobinuria physical examination | Physical Examination]] |[[Paroxysmal cold hemoglobinuria laboratory findings|Laboratory Findings]] | [[Paroxysmal cold hemoglobinuria chest x ray|Chest X Ray]] |  [[Paroxysmal cold hemoglobinuria CT|CT]] | [[Paroxysmal cold hemoglobinuria MRI|MRI]] | [[Paroxysmal cold hemoglobinuria echocardiography or ultrasound| Echocardiography or Ultrasound]] | [[Paroxysmal cold hemoglobinuria other imaging findings|Other Imaging Findings]] | [[Paroxysmal cold hemoglobinuria other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
[[Paroxysmal cold hemoglobinuria medical therapy|Medical Therapy]] | [[Paroxysmal cold hemoglobinuria surgery|Surgery]] | [[Paroxysmal cold hemoglobinuria primary prevention|Primary Prevention]] | [[Paroxysmal cold hemoglobinuria secondary prevention|Secondary Prevention]] | [[Paroxysmal cold hemoglobinuria cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Paroxysmal cold hemoglobinuria future or investigational therapies|Future or Investigational Therapies]]
==Case Studies==
[[Paroxysmal cold hemoglobinuria case study one|Case#1]]
==Related Chapters==
*[[Hemolytic anemia]]


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Latest revision as of 17:43, 21 September 2012

Paroxysmal cold hemoglobinuria

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paroxysmal cold hemoglobinuria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Donath-Landsteiner syndrome; PCH

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paroxysmal cold hemoglobinuria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination |Laboratory Findings | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case#1

Related Chapters


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