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{{SK}}  Donath-Landsteiner syndrome;  PCH  
{{SK}}  Donath-Landsteiner syndrome;  PCH  


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


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 historical perspective|Historical Perspective]]==


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 classification|Classification]]==


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 pathophysiology|Pathophysiology]]==


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 causes|Causes]]==


== Differential Diagnosis of Causes of {{PAGENAME}} ==  
==[[Paroxysmal cold hemoglobinuria differential diagnosis|Differentiating Paroxysmal cold hemoglobinuria from other Diseases]]==


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 epidemiology and demographics|Epidemiology and Demographics]]==


*[[Ddx:Anemia|Anemia]]
==[[Paroxysmal cold hemoglobinuria risk factors|Risk Factors]]==
*[[Chicken Pox]]
*Congenital [[Syphillis]]
*[[Glandular fever]]
*[[Measles]]
*[[Mumps]]  


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


==References==
 
{{Reflist}}
==[[Paroxysmal cold hemoglobinuria screening|Screening]]==
 
==[[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 ultrasound|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 suregry|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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Revision as of 17:42, 21 September 2012

Paroxysmal cold hemoglobinuria

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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 | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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Case#1

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