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'''For patient information click [[Autoimmune hemolytic anemia (patient information)|here]]'''
__NOTOC__
 
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = Autoimmune hemolytic anemia |
   Name          = Autoimmune hemolytic anemia |
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   MeshID        = D000744 |
   MeshID        = D000744 |
}}
}}
{{SI}}
{{Autoimmune hemolytic anemia}}
{{CMG}}
 
'''For patient information click [[Autoimmune hemolytic anemia (patient information)|here]]'''
 
{{CMG}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]; {{shyam}}, [[User:Irfan Dotani|Irfan Dotani]] [3]
 
{{SK}} AIHA; hemolytic anemia with autoimmune cause.
==[[Autoimmune hemolytic anemia overview|Overview]]==


==Overview==
==[[Autoimmune hemolytic anemia historical perspective|Historical Perspective]]==


'''Autoimmune hemolytic anemia''' (AIHA) is a type of [[hemolytic anemia]] where the body's immune system attacks its own [[red blood cells]] (RBCs), leading to their destruction ([[hemolysis]]).  [[Antibodies]] and associated [[complement system]] components become fixed onto the [[Red blood cell|RBC]] surface.    These [[antibodies]] can be detected with the [[Coombs test#Direct Coombs test|direct antiglobulin test]], also known as the direct [[Coombs test]].  AIHA can also be induced by several drugs including methyl-dopa and fluarabine.
==[[Autoimmune hemolytic anemia classification|Classification]]==


[[Autoimmunity]] must not be confused with [[alloimmunity]].
==[[Autoimmune hemolytic anemia pathophysiology|Pathophysiology]]==


==Classification==
==[[Autoimmune hemolytic anemia causes|Causes]]==
Haemolysis can be intravascular or extravascular.


'''Intravascular haemolysis'''<br>
==[[Autoimmune hemolytic anemia differential diagnosis|Differentiating Autoimmune hemolytic anemia from other Diseases]]==
Red blood cell lysis occurs in the circulation as a result of activation of the [[complement system]] cascade.
'''Extravascular haemolysis'''<br>
Red Blood Cells that are coated with antibodies are specifically recognised in the [[reticuloendothelial system]] and destroyed by [[macrophages]].


==Subtypes==
==[[Autoimmune hemolytic anemia epidemiology and demographics|Epidemiology and Demographics]]==


*'''[[Warm antibody autoimmune hemolytic anemia]]'''
==[[Autoimmune hemolytic anemia risk factors|Risk Factors]]==
:*Idiopathic
:*[[Systemic lupus erythematosus]]
:*[[Evans' syndrome]] (antiplatelet antibodies and haemolytic antibodies)
:*[[Chronic lymphocytic leukemia]]
:*Drugs ([[methyldopa]])


*'''Cold antibody autoimmune hemolytic anemia'''
==[[Autoimmune hemolytic anemia screening|Screening]]==
:*[[Idiopathic cold hemagglutinin syndrome]]
:*[[Infectious mononucleosis]]
:*[[Paroxysmal cold hemoglobinuria]] (rare)
:*[[Lymphoma]]


*'''Mixed-type autoimmune hemolytic anemia'''
==[[Autoimmune hemolytic anemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
==Causes==
The causes of AIHA are poorly understood. The disease may be primary, or secondary to another underlying illness. The primary illness is [[idiopathic]] (the two terms being used synonymously). Idiopathic AIHA accounts for approximately 50% of cases.<ref>{{cite journal |author=Gupta S, Szerszen A, Nakhl F, ''et al.'' |title=Severe refractory autoimmune hemolytic anemia with both warm and cold autoantibodies that responded completely to a single cycle of rituximab: a case report |journal=J Med Case Reports |volume=5 |pages=156 |year=2011 |pmid=21504611 |pmc=3096571 |doi=10.1186/1752-1947-5-156 |url=http://www.jmedicalcasereports.com/content/5//156}}</ref> Secondary AIHA can result from many other illnesses. Warm and cold type AIHA each have their own more common secondary causes. The most common causes of secondary warm-type AIHA include lymphoproliferative disorders (e.g. [[chronic lymphocytic leukemia]], [[lymphoma]]) and other autoimmune disorders (e.g. [[systemic lupus erythematosis]], [[rheumatoid arthritis]], [[scleroderma]], [[ulcerative colitis]]). Less common causes of warm-type AIHA include neoplasms other than lymphoid, and infection. Secondary cold type AIHA is also primarily caused by lymphoproliferative disorders, but is also commonly caused by infection, especially by mycoplasma, viral pneumonia, infectious mononucleosis and other respiratory infections. Less commonly, it can be caused by concomitant autoimmune disorders.<ref name=Sokol81/>


Drug-induced AIHA, though rare, can be caused by a number of drugs, including [[methyldopa|α-methyldopa]] and [[penicillin]]. This is a [[type II hypersensitivity|type II immune response]] in which the drug binds to [[macromolecule|macromolecules]] on the surface of the RBCs and acts as an antigen. Antibodies are produced against the RBCs, which leads to complement activation. Complement fragments, such as C3a, C4a and C5a, activate granular leukocytes (e.g. neutrophils), while other components of the system (C6, C7, C8, C9) can either form the membrane attack complex (MAC) or can bind the antibody, aiding phagocytosis by [[macrophage|macrophages]] (C3b). This is one type of "penicillin allergy".
==Diagnosis==
 
[[Autoimmune hemolytic anemia diagnostic study of choice|Diagnostic Study of Choice]] | [[Autoimmune hemolytic anemia history and symptoms|History and Symptoms ]] | [[Autoimmune hemolytic anemia physical examination|Physical Examination]] | [[Autoimmune hemolytic anemia laboratory findings|Laboratory Findings]] | [[Autoimmune hemolytic anemia electrocardiogram|Electrocardiogram]] | [[Autoimmune hemolytic anemia chest x-ray|X-ray]] | [[Autoimmune hemolytic anemia echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Autoimmune hemolytic anemia CT|CT]] | [[Autoimmune hemolytic anemia MRI|MRI]] | [[Autoimmune hemolytic anemia other imaging findings|Other Imaging Findings]] | [[Autoimmune hemolytic anemia other diagnostic studies|Other Diagnostic Studies]]


==Laboratory findings==
*Positive direct [[Coombs test]]
*[[Anaemia]]
==Treatment==
==Treatment==
Much literature exists regarding the treatment of AIHA. Efficacy of treatment depends on the correct diagnosis of either warm or cold type AIHA.
[[Autoimmune hemolytic anemia medical therapy|Medical Therapy]] | [[Autoimmune hemolytic anemia surgery |Surgery]] | [[Autoimmune hemolytic anemia primary prevention|Primary Prevention]] | [[Autoimmune hemolytic anemia secondary prevention|Secondary Prevention]] | [[Autoimmune hemolytic anemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] |  [[Autoimmune hemolytic anemia future or investigational therapies|Future or Investigational Therapies]]


Warm type AIHA is usually a more insidious disease, not treatable by simply removing the underlying cause. First line therapy for this is usually with [[corticosteroid|corticosteroids]], such as [[prednisolone]]. Following this, other immunosuppressants are considered, such as [[rituximab]], [[danazol]], [[cyclophosphamide]], [[azathioprine]] or [[cyclosporine]].
==Case Studies==


Cold agglutinin disease is treated by avoiding the cold or sometimes with rituximab. Removal of the underlying cause is also important.
[[Autoimmune hemolytic anemia case study one|Case #1]]


Paroxysmal cold hematuria is treated by removing the underlying cause, such as infection.
==Related Chapters==
 
==Related chapters==
*[[Haematology]]
*[[Haematology]]
*[[Haemolytic anaemia]]
*[[Haemolytic anaemia]]
{{WH}}
{{WS}}


{{Hematology}}
{{Hematology}}
{{SIB}}
 


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{{WikiDoc Sources}}
{{WikiDoc Sources}}


[[Category:Mature chapter]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Autoimmune diseases]]
[[Category:Coagulation system]]
[[Category:Blood disorders]]
[[Category:Hematology]]
[[Category:Hematology]]

Latest revision as of 11:52, 20 September 2018

Autoimmune hemolytic anemia
ICD-10 D59.0-D59.1
ICD-9 283.0
MedlinePlus 000579
MeSH D000744

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Diagnostic Study of Choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2], Irfan Dotani [3]

Synonyms and keywords: AIHA; hemolytic anemia with autoimmune cause.

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Autoimmune hemolytic anemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-ray | Echocardiography and Ultrasound | CT | MRI | 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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