Cold agglutinin disease
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| Cold agglutinin disease Classification and external resources | |
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| Cold agglutination - at body temperature, the antibodies do not attach to the red blood cells. At lower temperatures, however, the antibodies react to Ii antigens, bringing the red blood cells together, a process known as agglutination | |
| ICD-10 | D59.1 |
| ICD-9 | 283.0 |
| DiseasesDB | 2949 |
| eMedicine | med/408 |
| MeSH | D000744 |
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Cold agglutinin disease is an autoimmune disease characterized by the presence of high concentrations of circulating antibodies directed against red blood cells. It is a form of autoimmune hemolytic anemia, specifically one in which antibodies only bind red blood cells at low temperatures, typically 28-31°C.
Pathophysiology
All individuals have circulating antibodies directed against red blood cells, but their concentrations are often too low to trigger disease (titers under 1:64 at 4°C). In individuals with cold agglutinin disease, these antibodies are in much higher concentrations (titers over 1:1000 at 4°C).
At temperatures of 28-31°C, such as those encountered during winter months, and occasionally at 37°C, antibodies bind to the polysaccharide region of glycoproteins on the surface of red blood cells (typically the I antigen, i antigen, and Pr antigens). IgM-type antibodies are typically responsible for this action. Binding of antibodies to red blood cells activates the classical pathway of the complement system. If the complement response is sufficient, red blood cells are damaged by the membrane attack complex, an effector of the complement cascade. In the formation of the membrane attack complex, several complement proteins are inserted into the red blood cell membrane, forming pores that lead to membrane instability and intravascular hemolysis (destruction of the red blood cell within the blood vessels).
If the complement response is insufficient to form membrane attack complexes, then extravascular lysis will be favored over intravascular red blood cell lysis. In lieu of the membrane attack complex, complement proteins (particularly C3b and C4b) are deposited on red blood cells. This opsonization enhances the clearance of red blood cell by phagocytes in the liver, spleen, and lungs, a process termed extravascular hemolysis.
Individuals with cold agglutinin disease present with signs and symptoms of hemolytic anemia. Those with secondary agglutinin disease may also present with an underlying disease.
Etiology
There are two forms of cold agglutinin disease: primary and secondary. The primary form is by definition idiopathic, a disease for which no cause is known.
Secondary cold agglutinin disease is a result of an underlying condition. In adults, this is typically due to a lymphoproliferative disease such as lymphoma and chronic lymphoid leukemia. In children, cold agglutinin disease is often secondary to an infection, such as Mycoplasma pneumonia, mononucleosis, and HIV.
See also
References
Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289) | |||||||||||||||||||||
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| RBCs/ hemoglobinopathy |
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| Coagulation/platelets/ coagulopathy/ bleeding diathesis |
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| Monocytes/ macrophages |
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| Granulocytes |
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| See also hematological malignancy and immune disorders | |||||||||||||||||||||
WikiDoc Research Resources for Cold agglutinin disease | |
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| Articles on Cold agglutinin disease | Most recent articles on Cold agglutinin disease • Most cited articles on Cold agglutinin disease • Review articles on Cold agglutinin disease • Articles on Cold agglutinin disease in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Cold agglutinin disease | Powerpoint slides on Cold agglutinin disease • Images of Cold agglutinin disease • Photos of Cold agglutinin disease • Podcasts & MP3s on Cold agglutinin disease • Videos on Cold agglutinin disease |
| Evidence Based Medicine Regarding Cold agglutinin disease | Cochrane Collaboration on Cold agglutinin disease • Bandolier on Cold agglutinin disease • TRIP on Cold agglutinin disease |
| Cost Effectiveness of Cold agglutinin disease | Cost Effectiveness of Cold agglutinin disease |
| Clinical Trials Involving Cold agglutinin disease | Ongoing Trials on Cold agglutinin disease at Clinical Trials.gov • Trial results on Cold agglutinin disease • Clinical Trials on Cold agglutinin disease at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Cold agglutinin disease | US National Guidelines Clearinghouse on Cold agglutinin disease • NICE Guidance on Cold agglutinin disease • NHS PRODIGY Guidance • FDA on Cold agglutinin disease • CDC on Cold agglutinin disease |
| Textbook Information on Cold agglutinin disease | Books and Textbook Information on Cold agglutinin disease |
| Pharmacology Resources on Cold agglutinin disease | Dosing of Cold agglutinin disease • Drug interactions with Cold agglutinin disease • Side effects of Cold agglutinin disease • Allergic reactions to Cold agglutinin disease • Overdose information on Cold agglutinin disease • Carcinogenicity information on Cold agglutinin disease • Cold agglutinin disease in pregnancy • Pharmacokinetics of Cold agglutinin disease • |
| Genetics, Pharmacogenomics, and Proteinomics of Cold agglutinin disease | Genetics of Cold agglutinin disease • Pharmacogenomics of Cold agglutinin disease • Proteomics of Cold agglutinin disease |
| Newstories on Cold agglutinin disease | Cold agglutinin disease in the news • Be alerted to news on Cold agglutinin disease • News trends on Cold agglutinin disease |
| Commentary on Cold agglutinin disease | Blogs on Cold agglutinin disease |
| Patient Resources on Cold agglutinin disease | Patient resources on Cold agglutinin disease • Discussion groups on Cold agglutinin disease • Patient Handouts on Cold agglutinin disease • Directions to Hospitals Treating Cold agglutinin disease • Risk calculators and risk factors for Cold agglutinin disease |
| Healthcare Provider Resources on Cold agglutinin disease | Symptoms of Cold agglutinin disease • Causes & Risk Factors for Cold agglutinin disease • Diagnostic studies for Cold agglutinin disease • Treatment of Cold agglutinin disease |
| Continuing Medical Education (CME) Programs on Cold agglutinin disease | CME Programs on Cold agglutinin disease |
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| Informatics Resources on Cold agglutinin disease | List of terms related to Cold agglutinin disease |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
