Autoimmune hemolytic anemia natural history, complications and prognosis: Difference between revisions

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The natural history of autoimmune hemolytic anemia begins with the hemolytic event. A diagnostic workup is typically initiated soon after the hemolytic event, and the hemolysis subsides after the start of [[corticosteroids]]. Most patients will generally achieve [[remission]] with no long-term complications. For patients who develop complications, these complications include [[infection]], [[thrombosis]], [[iron overload]], and end-organ damage from impaired oxygen delivery. The prognosis of autoimmune hemolytic anemia depends on the severity of the immune activation. The prognosis is overall favorable for most patients.
The natural history of autoimmune hemolytic anemia begins with the hemolytic event. A diagnostic workup is typically initiated soon after the hemolytic event, and the hemolysis subsides after the start of [[corticosteroids]]. Most patients will generally achieve [[remission]] with no long-term complications. For patients who develop complications, these complications include [[infection]], [[thrombosis]], [[iron overload]], and end-organ damage from impaired oxygen delivery. The prognosis of autoimmune hemolytic anemia depends on the severity of the immune activation. The prognosis is overall favorable for most patients.


==Natural History==
==Natural History, Complications, and Prognosis==
 
=== Natural History ===
* The natural history of autoimmune hemolytic anemia begins with the hemolytic event.  
* The natural history of autoimmune hemolytic anemia begins with the hemolytic event.  
* Patients usually present for evaluation after they experience symptoms of [[fatigue]], [[shortness of breath]], or decreased exercise tolerance, as the anemia becomes worse.  
* Patients usually present for evaluation after they experience symptoms of [[fatigue]], [[shortness of breath]], or decreased [[Exercise tolerance test|exercise tolerance]], as the [[anemia]] becomes worse.  
* At [[hemoglobin]] levels of less than 8 g/dl, symptoms will usually be apparent.  
* At [[hemoglobin]] levels of less than 8 g/dl, symptoms will usually be apparent.  
* After presenting with these symptoms and a diagnosis of autoimmune hemolytic anemia is made, patients are started on [[corticosteroids]].  
* After presenting with these symptoms and a diagnosis of [[autoimmune hemolytic anemia]] is made, patients are started on [[corticosteroids]].  
* A response will usually occur within 1-2 weeks, after which symptoms will likely abate. Hemoglobin will return towards normal range, and this can be maintained via a steroid taper over the next few weeks to months.  
* A response will usually occur within 1-2 weeks, after which symptoms will likely abate.
* The natural history of autoimmune hemolytic anemia is self-limited in children if treated with [[corticosteroids]]. Most children respond very well to steroids and can lead normal lives without frequent relapses or recurrences.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref> After recovery from the initial hemolytic episodes, it is unlikely that children will relapse.  
* [[Hemoglobin]] will return towards normal range, and this can be maintained via a [[steroid]] taper over the next few weeks to months.  
* In older children, autoimmune hemolytic anemia can be [[chronic]].<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
* The natural history of autoimmune hemolytic anemia is self-limited in children if treated with [[corticosteroids]].
* Most children respond very well to steroids and can lead normal lives without frequent relapses or recurrences.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
* After recovery from the initial [[hemolytic episodes]], it is unlikely that children will relapse.  
* In older children, [[autoimmune hemolytic anemia]] can be [[chronic]].<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>


==Complications==
===Complications===
*'''Infection''':  
*Infection:<ref>{{cite journal |author=Zecca M, Nobili B, Ramenghi U, ''et al.'' |title=Rituximab for the treatment of refractory autoimmune hemolytic anemia in children |journal=Blood |volume=101 |issue=10 |pages=3857–61 |year=2003 |month=May |pmid=12531800 |doi=10.1182/blood-2002-11-3547 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12531800}}</ref>  
**This is a serious concern in patients on long-term [[immunosuppressant therapy]], especially in very young children (less than two years).<ref>{{cite journal |author=Zecca M, Nobili B, Ramenghi U, ''et al.'' |title=Rituximab for the treatment of refractory autoimmune hemolytic anemia in children |journal=Blood |volume=101 |issue=10 |pages=3857–61 |year=2003 |month=May |pmid=12531800 |doi=10.1182/blood-2002-11-3547 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12531800}}</ref>   
**This is a serious concern in patients on long-term [[immunosuppressant therapy]], especially in very young children (less than two years).  
**Infections occur due to impaired [[Cell-mediated immunity|cell-mediated]] and [[humoral immunity]].   
**Infections occur due to impaired [[Cell-mediated immunity|cell-mediated]] and [[humoral immunity]].   
**Different [[immunosuppressive]] agents have different infectious complication profiles. For example, patients on [[rituximab]] can experience the rare but highly lethal infection with [[JC virus]], which causes [[progressive multifocal leukoencephalopathy]]. Severe infections can result in death.  
**Different [[immunosuppressive]] agents have different infectious complication profiles. For example, patients on [[rituximab]] can experience the rare but highly lethal infection with [[JC virus]], which causes [[progressive multifocal leukoencephalopathy]]. Severe infections can result in death.  
*'''Thrombosis''':  
*Thrombosis:<ref name="pmid26696800" />
**Patients with autoimmune hemolytic anemia are at higher risk for [[thrombosis]].  
**Patients with autoimmune hemolytic anemia are at higher risk for [[thrombosis]].  
**[[Thrombosis]] is more likely to occur in the active phase of [[hemolysis]], such as during a severe hemolytic episode.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
**[[Thrombosis]] is more likely to occur in the active phase of [[hemolysis]], such as during a severe hemolytic episode.  
**Anti-phospholipid antibodies are associated with the occurrence of thrombosis, as these antibodies bind to platelet membranes and induce thrombosis.  
**Anti-phospholipid antibodies are associated with the occurrence of [[thrombosis]], as these [[antibodies]] bind to platelet membranes and induce thrombosis.  
**Patients with autoimmune hemolytic anemia who have a known history of thrombosis or are at high risk for thrombosis from other etiologies should begin [[anticoagulation]].
**Patients with autoimmune hemolytic anemia who have a known history of thrombosis or are at high risk for thrombosis from other etiologies should begin [[anticoagulation]].
*'''Iron overload''':  
*Iron overload:  
**Autoimmune hemolytic anemia is frequently complicated by iron overload due to frequent [[red blood cell]] transfusions.  
**Autoimmune hemolytic anemia is frequently complicated by iron overload due to frequent [[red blood cell]] transfusions.  
**In patients for whom [[steroids]] do not work, [[red blood cell]] transfusions can be an important temporizing measure to maintain the hemoglobin concentration with a safe range. For example, a typical hemoglobin threshold of 7g/dl is used when considering transfusion requirements.  
**In patients for whom [[steroids]] do not work, [[red blood cell]] transfusions can be an important temporizing measure to maintain the hemoglobin concentration with a safe range. For example, a typical hemoglobin threshold of 7g/dl is used when considering transfusion requirements.  
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****Iron deposition in the heart can result in symptoms of fatigue, [[shortness of breath]], chest pain, edema, [[orthopnea]], and [[paroxysmal nocturnal dyspnea]].   
****Iron deposition in the heart can result in symptoms of fatigue, [[shortness of breath]], chest pain, edema, [[orthopnea]], and [[paroxysmal nocturnal dyspnea]].   
****Treatment involves iron chelators such as deferoxamine, deferasirox, or deferiprone.  
****Treatment involves iron chelators such as deferoxamine, deferasirox, or deferiprone.  
*'''End-organ damage from impaired oxygen delivery''': The effects of severe anemia affect a variety of organs and tissues that rely heavily on oxygen for energy metabolism. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia.  
*End-organ damage from impaired oxygen delivery: The effects of severe anemia affect a variety of organs and tissues that rely heavily on oxygen for energy metabolism. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia.  
**''[[Hypoxic brain injury]]'': This is a rare but important complication of severe anemia. Low oxygen delivery to the brain as a result of autoimmune hemolytic anemia can result in ischemic injury to neurons and supporting tissue. This is functionally similar to [[cerebrovascular accident]], or [[stroke]], which is due to atherosclerotic or embolic blockage of the cerebral circulation. Patients can experience severe symptoms such as numbness, weakness, paralysis, and [[dysarthria]], or slurred speech. Patients can have a significant neurological impairment (including deficits in motor, sensory, cortical, and cerebellar function). Overall, the likelihood of hypoxic brain injury due to autoimmune hemolytic anemia is very low.
**''[[Hypoxic brain injury]]'':  
**''[[Myocardial]] injury'': Autoimmune hemolytic anemia can result in impaired oxygen delivery to the cardiac tissue. [[Myocardial infarction]] can result from poor oxygen content in the coronary circulation, which is functionally similar to having ischemia due to blockage by an atherosclerotic plaque. Patients can experience severe symptoms such as shortness of breath, fatigue, and decreased exercise tolerance. Anemia is associated with poor outcomes.<ref name="pmid26517509">{{cite journal| author=Park S, Jung CW, Kim K, Kim SJ, Kim WS, Jang JH| title=Iron deficient erythropoiesis might play key role in development of anemia in cancer patients. | journal=Oncotarget | year= 2015 | volume= 6 | issue= 40 | pages= 42803-12 | pmid=26517509 | doi=10.18632/oncotarget.5658 | pmc=4767472 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26517509  }} </ref> Patients can have long-term complications such as congestive heart failure.
***This is a rare but important complication of severe anemia.
**''High-output cardiac failure'': Hemolytic anemia can also be associated with high-output cardiac failure, in which the [[ejection fraction]] and [[cardiac output]] are higher than normal. This is a manifestation of impaired oxygen delivery at the tissue level, for which the body compensated by increasing the circulatory output in an attempt to deliver more oxygen to tissues. High-output cardiac failure can lead to congestive heart failure.
***Low oxygen delivery to the brain as a result of autoimmune hemolytic anemia can result in ischemic injury to neurons and supporting tissue.  
***This is functionally similar to [[cerebrovascular accident]], or [[stroke]], which is due to atherosclerotic or embolic blockage of the cerebral circulation.  
***Patients can experience severe symptoms such as numbness, weakness, paralysis, and [[dysarthria]], or slurred speech.  
***Patients can have a significant neurological impairment (including deficits in motor, sensory, cortical, and cerebellar function).  
***Overall, the likelihood of hypoxic brain injury due to autoimmune hemolytic anemia is very low.
**''[[Myocardial]] injury'':  
***Autoimmune hemolytic anemia can result in impaired oxygen delivery to the cardiac tissue.  
***[[Myocardial infarction]] can result from poor oxygen content in the coronary circulation, which is functionally similar to having ischemia due to blockage by an atherosclerotic plaque.  
***Patients can experience severe symptoms such as shortness of breath, fatigue, and decreased exercise tolerance.  
***Anemia is associated with poor outcomes.<ref name="pmid26517509">{{cite journal| author=Park S, Jung CW, Kim K, Kim SJ, Kim WS, Jang JH| title=Iron deficient erythropoiesis might play key role in development of anemia in cancer patients. | journal=Oncotarget | year= 2015 | volume= 6 | issue= 40 | pages= 42803-12 | pmid=26517509 | doi=10.18632/oncotarget.5658 | pmc=4767472 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26517509  }} </ref>  
***Patients can have long-term complications such as congestive heart failure.
**''High-output cardiac failure'':  
***Hemolytic anemia can also be associated with high-output cardiac failure, in which the [[ejection fraction]] and [[cardiac output]] are higher than normal.  
***This is a manifestation of impaired oxygen delivery at the tissue level, for which the body compensated by increasing the circulatory output in an attempt to deliver more oxygen to tissues.  
***High-output cardiac failure can lead to congestive heart failure.


==Prognosis==
===Prognosis===
* The prognosis of autoimmune hemolytic anemia is variable and depends on the severity of the immune activation.  
* The prognosis of autoimmune hemolytic anemia is variable and depends on the severity of the immune activation.<ref name="pmid26696800" />
* In the early 2000s, studies reported a 10-year survival of 73%.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>  
* In the early 2000s, studies reported a 10-year survival of 73%.<ref name="pmid26696800" />  
* In the current era, the survival is likely better.  
* In the current era, the survival is likely better.<ref name="pmid26696800" />
* The mortality rate is 10-30%.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>  
* The mortality rate is 10-30%.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>  
* Patients with concurrent immune thrombocytopenia purpura have a higher mortality rate.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref> This condition is known as [[Evan's syndrome]].
* Patients with concurrent immune thrombocytopenia purpura have a higher mortality rate. This condition is known as [[Evan's syndrome]].<ref name="pmid26696800" />


===Secondary autoimmune hemolytic anemia===
====Secondary autoimmune hemolytic anemia====
The prognosis for secondary autoimmune hemolytic anemia depends on the underlying cause.  
The prognosis for secondary autoimmune hemolytic anemia depends on the underlying cause.  
*'''[[Chronic lymphocytic leukemia]]''' :
*'''Chronic lymphocytic leukemia''' :
**In patients with chronic lymphocytic leukemia ([[Chronic lymphocytic leukemia|CLL]]), for example, the hemolysis can continue for months to years if the underlying disease is high-risk. On the contrary, patients with CLL with favorable risk features may have limited hemolysis and excellent prognosis.
**In patients with chronic lymphocytic leukemia ([[Chronic lymphocytic leukemia|CLL]]), for example, the hemolysis can continue for months to years if the underlying disease is high-risk. On the contrary, patients with CLL with favorable risk features may have limited hemolysis and excellent prognosis.
*'''[[Rheumatologic conditions]]''':  
*'''Rheumatologic conditions''':  
**In patients with systemic lupus erythematosus (SLE) or other rheumatologic conditions, the severity of the hemolytic anemia corresponds with the degree of aberrant immune activation. The severity of the hemolysis parallels other autoimmune components of these diseases. The prognosis in patients with severe SLE may be dismal.
**In patients with systemic lupus erythematosus (SLE) or other rheumatologic conditions, the severity of the hemolytic anemia corresponds with the degree of aberrant immune activation. The severity of the hemolysis parallels other autoimmune components of these diseases. The prognosis in patients with severe SLE may be dismal.


===Other hemolytic anemias===
====Other hemolytic anemias====
The prognosis of other types of hemolytic anemias is variable and depends on the subtype of hemolytic anemia.
The prognosis of other types of hemolytic anemias is variable and depends on the subtype of hemolytic anemia.
*'''[[Paroxysmal cold hemoglobinuria]]''': In contrast to autoimmune hemolytic anemia, patients with paroxysmal cold hemoglobinuria have an excellent prognosis in the absence of cold weather.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref> Patients can survive for years.
*'''Paroxysmal cold hemoglobinuria''': In contrast to autoimmune hemolytic anemia, patients with paroxysmal cold hemoglobinuria have an excellent prognosis in the absence of cold weather.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref> Patients can survive for years.
*'''[[Drug-induced hemolytic anemia]]''': The prognosis for drug-related hemolysis is excellent with removal of the offending agent. Hemolysis usually resolves once the culprit drug is discontinued. After a period of weeks, the drug-dependent antibody will be eliminated, and the [[direct antiglobulin test]] will be negative. However, if the drug is not removed, hemolysis will likely continue and can contribute to severe ongoing anemia.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
*'''Drug-induced hemolytic anemia''': The prognosis for drug-related hemolysis is excellent with removal of the offending agent. Hemolysis usually resolves once the culprit drug is discontinued. After a period of weeks, the drug-dependent antibody will be eliminated, and the [[direct antiglobulin test]] will be negative. However, if the drug is not removed, hemolysis will likely continue and can contribute to severe ongoing anemia.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
*'''[[Post-infectious cold hemoglobinuria]]''': The prognosis of post-infectious cold hemoglobinuria is excellent upon clearance of the infectious agent, which is usually a virus. Hemolysis will cease after a period of weeks. The [[Donath-Landsteiner antibody]], which is a [[biphasic hemolysin]], will usually abate once the infection clears.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>
*'''Post-infectious cold hemoglobinuria''': The prognosis of post-infectious cold hemoglobinuria is excellent upon clearance of the infectious agent, which is usually a virus. Hemolysis will cease after a period of weeks. The [[Donath-Landsteiner antibody]], which is a [[biphasic hemolysin]], will usually abate once the infection clears.<ref name="pmid26696800">{{cite journal| author=Packman CH| title=The Clinical Pictures of Autoimmune Hemolytic Anemia. | journal=Transfus Med Hemother | year= 2015 | volume= 42 | issue= 5 | pages= 317-24 | pmid=26696800 | doi=10.1159/000440656 | pmc=4678314 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26696800  }} </ref>


==References==
==References==

Latest revision as of 11:58, 20 September 2018

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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]

Overview

The natural history of autoimmune hemolytic anemia begins with the hemolytic event. A diagnostic workup is typically initiated soon after the hemolytic event, and the hemolysis subsides after the start of corticosteroids. Most patients will generally achieve remission with no long-term complications. For patients who develop complications, these complications include infection, thrombosis, iron overload, and end-organ damage from impaired oxygen delivery. The prognosis of autoimmune hemolytic anemia depends on the severity of the immune activation. The prognosis is overall favorable for most patients.

Natural History, Complications, and Prognosis

Natural History

  • The natural history of autoimmune hemolytic anemia begins with the hemolytic event.
  • Patients usually present for evaluation after they experience symptoms of fatigue, shortness of breath, or decreased exercise tolerance, as the anemia becomes worse.
  • At hemoglobin levels of less than 8 g/dl, symptoms will usually be apparent.
  • After presenting with these symptoms and a diagnosis of autoimmune hemolytic anemia is made, patients are started on corticosteroids.
  • A response will usually occur within 1-2 weeks, after which symptoms will likely abate.
  • Hemoglobin will return towards normal range, and this can be maintained via a steroid taper over the next few weeks to months.
  • The natural history of autoimmune hemolytic anemia is self-limited in children if treated with corticosteroids.
  • Most children respond very well to steroids and can lead normal lives without frequent relapses or recurrences.[1]
  • After recovery from the initial hemolytic episodes, it is unlikely that children will relapse.
  • In older children, autoimmune hemolytic anemia can be chronic.[1]

Complications

  • Infection:[2]
  • Thrombosis:[1]
    • Patients with autoimmune hemolytic anemia are at higher risk for thrombosis.
    • Thrombosis is more likely to occur in the active phase of hemolysis, such as during a severe hemolytic episode.
    • Anti-phospholipid antibodies are associated with the occurrence of thrombosis, as these antibodies bind to platelet membranes and induce thrombosis.
    • Patients with autoimmune hemolytic anemia who have a known history of thrombosis or are at high risk for thrombosis from other etiologies should begin anticoagulation.
  • Iron overload:
    • Autoimmune hemolytic anemia is frequently complicated by iron overload due to frequent red blood cell transfusions.
    • In patients for whom steroids do not work, red blood cell transfusions can be an important temporizing measure to maintain the hemoglobin concentration with a safe range. For example, a typical hemoglobin threshold of 7g/dl is used when considering transfusion requirements.
    • However, a major adverse effect of frequent transfusions is iron overload, as iron can deposit in a variety of organs and result in impaired organ function. For these reasons, it is best to address the underlying cause (immune activation) of autoimmune hemolytic anemia, rather than provide transfusional support in the long-term.
  • End-organ damage from impaired oxygen delivery: The effects of severe anemia affect a variety of organs and tissues that rely heavily on oxygen for energy metabolism. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia. If hemoglobin decreases significantly, typically to values lower than 7g/dl, patients can experience impaired oxygen delivery and end-organ hypoxia.
    • Hypoxic brain injury:
      • This is a rare but important complication of severe anemia.
      • Low oxygen delivery to the brain as a result of autoimmune hemolytic anemia can result in ischemic injury to neurons and supporting tissue.
      • This is functionally similar to cerebrovascular accident, or stroke, which is due to atherosclerotic or embolic blockage of the cerebral circulation.
      • Patients can experience severe symptoms such as numbness, weakness, paralysis, and dysarthria, or slurred speech.
      • Patients can have a significant neurological impairment (including deficits in motor, sensory, cortical, and cerebellar function).
      • Overall, the likelihood of hypoxic brain injury due to autoimmune hemolytic anemia is very low.
    • Myocardial injury:
      • Autoimmune hemolytic anemia can result in impaired oxygen delivery to the cardiac tissue.
      • Myocardial infarction can result from poor oxygen content in the coronary circulation, which is functionally similar to having ischemia due to blockage by an atherosclerotic plaque.
      • Patients can experience severe symptoms such as shortness of breath, fatigue, and decreased exercise tolerance.
      • Anemia is associated with poor outcomes.[3]
      • Patients can have long-term complications such as congestive heart failure.
    • High-output cardiac failure:
      • Hemolytic anemia can also be associated with high-output cardiac failure, in which the ejection fraction and cardiac output are higher than normal.
      • This is a manifestation of impaired oxygen delivery at the tissue level, for which the body compensated by increasing the circulatory output in an attempt to deliver more oxygen to tissues.
      • High-output cardiac failure can lead to congestive heart failure.

Prognosis

  • The prognosis of autoimmune hemolytic anemia is variable and depends on the severity of the immune activation.[1]
  • In the early 2000s, studies reported a 10-year survival of 73%.[1]
  • In the current era, the survival is likely better.[1]
  • The mortality rate is 10-30%.[1]
  • Patients with concurrent immune thrombocytopenia purpura have a higher mortality rate. This condition is known as Evan's syndrome.[1]

Secondary autoimmune hemolytic anemia

The prognosis for secondary autoimmune hemolytic anemia depends on the underlying cause.

  • Chronic lymphocytic leukemia :
    • In patients with chronic lymphocytic leukemia (CLL), for example, the hemolysis can continue for months to years if the underlying disease is high-risk. On the contrary, patients with CLL with favorable risk features may have limited hemolysis and excellent prognosis.
  • Rheumatologic conditions:
    • In patients with systemic lupus erythematosus (SLE) or other rheumatologic conditions, the severity of the hemolytic anemia corresponds with the degree of aberrant immune activation. The severity of the hemolysis parallels other autoimmune components of these diseases. The prognosis in patients with severe SLE may be dismal.

Other hemolytic anemias

The prognosis of other types of hemolytic anemias is variable and depends on the subtype of hemolytic anemia.

  • Paroxysmal cold hemoglobinuria: In contrast to autoimmune hemolytic anemia, patients with paroxysmal cold hemoglobinuria have an excellent prognosis in the absence of cold weather.[1] Patients can survive for years.
  • Drug-induced hemolytic anemia: The prognosis for drug-related hemolysis is excellent with removal of the offending agent. Hemolysis usually resolves once the culprit drug is discontinued. After a period of weeks, the drug-dependent antibody will be eliminated, and the direct antiglobulin test will be negative. However, if the drug is not removed, hemolysis will likely continue and can contribute to severe ongoing anemia.[1]
  • Post-infectious cold hemoglobinuria: The prognosis of post-infectious cold hemoglobinuria is excellent upon clearance of the infectious agent, which is usually a virus. Hemolysis will cease after a period of weeks. The Donath-Landsteiner antibody, which is a biphasic hemolysin, will usually abate once the infection clears.[1]

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Packman CH (2015). "The Clinical Pictures of Autoimmune Hemolytic Anemia". Transfus Med Hemother. 42 (5): 317–24. doi:10.1159/000440656. PMC 4678314. PMID 26696800.
  2. Zecca M, Nobili B, Ramenghi U; et al. (2003). "Rituximab for the treatment of refractory autoimmune hemolytic anemia in children". Blood. 101 (10): 3857–61. doi:10.1182/blood-2002-11-3547. PMID 12531800. Unknown parameter |month= ignored (help)
  3. Park S, Jung CW, Kim K, Kim SJ, Kim WS, Jang JH (2015). "Iron deficient erythropoiesis might play key role in development of anemia in cancer patients". Oncotarget. 6 (40): 42803–12. doi:10.18632/oncotarget.5658. PMC 4767472. PMID 26517509.

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