Cold agglutinin disease

Jump to navigation Jump to search
Cold agglutinin disease
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

WikiDoc Resources for Cold agglutinin disease

Articles

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

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

Cochrane Collaboration on Cold agglutinin disease

Bandolier on Cold agglutinin disease

TRIP on Cold agglutinin disease

Clinical Trials

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 / Govt

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

Books

Books on Cold agglutinin disease

News

Cold agglutinin disease in the news

Be alerted to news on Cold agglutinin disease

News trends on Cold agglutinin disease

Commentary

Blogs on Cold agglutinin disease

Definitions

Definitions of Cold agglutinin disease

Patient Resources / Community

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

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)

CME Programs on Cold agglutinin disease

International

Cold agglutinin disease en Espanol

Cold agglutinin disease en Francais

Business

Cold agglutinin disease in the Marketplace

Patents on Cold agglutinin disease

Experimental / Informatics

List of terms related to Cold agglutinin disease

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

Synonyms and keywords:

Overview

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.

Historical Perspective

Classification

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.

Causes

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.

Differentiating Cold Agglutinin Disease from Other Diseases

Epidemiology and Demographics

Age

Gender

Race

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

See also

References


Template:Hematologic disease Template:Hematology


Template:WikiDoc Sources