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{{Eosinophilia}}
{{Eosinophilia}}
{{CMG}} {{AE}}
{{CMG}} {{AE}}George Leef MD


==Overview==
==Overview==
Eosinphilia refers to a group of idiopathic, neoplastic, infectious and allergic illnesses.
Eosinophilia refers to elevated eosinophils in the peripheral blood.  It is important to remember that eosinophilia is a ''symptom'', not a disease. Eosinophilia can be a manifestation of a variety of different disease processes, including neoplastic, infectious, allergic, and idiopathic processes. 
 
When the eosinophil count is markedly elevated and the patient has symptoms attributable to the eosinophils (and not explained by the underlying condition, if any), it is referred to as a [[hypereosinophilic syndrome]]. <ref name=":3">National Institute of Allergy and Infectious Disease, Hypereosinophilic Syndrome.  http://www.niaid.nih.gov/topics/EosinophilicDisorders/Pages/Hyper.aspx</ref> Most patients with eosinophilia do not have a hypereosinophilic syndrome.


==Historical Perspective==
==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*As mentioned above, eosinophilia is not a unified disease entity, but rather a manifestation of many different disease processes.
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*The [[eosinophil]] itself was discovered in 1879 <ref>Kay AB. The early history of the eosinophil. Clin Exp Allergy. 2015 Mar;45(3):575-82.</ref> as a granulocytic cell that stained with the red dye eosin.  [[Granulocyte|Granulocytes]] in general were described several decades earlier.
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
*The hypereosinophilic syndrome was first described in 1968. <ref name=":2" />
   
   
==Classification==
==Classification==
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
*Major disease categories that can cause eosinophilia: <ref name=":2">Brito-Babapulle. The Eosinophilias including the Hypereosinophilic Syndrome.  British Journal of Hematology (2003) 121(3):203-223.  </ref>
:*[group1]
*Allergy/Atopy
:*[group2]
*Infections
:*[group3]
**Parasitic: particularly associated with helminths, most protozoan parasites do not cause eosinophilia ([[Toxoplasma]] is an exception)
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
**Certain fungal organisms: Coccidiomycosis
**Viral (HIV, HTLV, Herpes)
*Malignancies
**Hematologic: primary eosinophil neoplasia, B and T cell [[Lymphoma|lymphomas]], [[Chronic myelogenous leukemia|CML]], [[Hodgkin's lymphoma|Hodgkin's Lymphoma]]
**Solid tumors: breast, lung
*[[Myelodysplastic syndrome|Myelodysplastic]] and [[Myeloproliferative disease|myeloproliferative]] disorders
*[[Mastocytosis|Systemic Mastocytosis]]
*[[Addison's disease|Addison's Disease]]
*Drug Reactions ([[DRESS syndrome|DRESS]])
*Vasculitis ([[Churg-Strauss syndrome|Churg-Strauss]])
*Idiopathic
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*Eosinophilia is defined by blood eosinophil count > 500/microL.
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*The pathogenesis is variable.  Eosinophila can be caused by a proliferative neoplasm (as in eosinophilic leukemia), or as a reaction to another process (parasitic infection, drug reaction, solid tumor) that causes elevated levels in cytokines that promote eosinophil maturation in the bone marrow, or it may be idiopathic.
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
   
   
==Causes==
==Causes==
* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
* Eosinophilia can be caused by numerous different processes listed above.  
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* There are no established causes for [disease name].
==Differentiating [disease name] from other Diseases==
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
   
   
==Differentiating eosinophilia from other Diseases==
*If the eosinophil count is elevated, eosinophilia is present by definition.  Care should be taken to discover the correct cause for the eosinophilia.
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* Eosinophilia is not a specific disease entity, epidemiologic numbers are availble for major causes of eosinophilia
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
* [[Hypereosinophilic syndrome|Hypereosinophilic syndromes]] are extremely rare, estimated at 0.36 to 6.3 people per 100000 in the US. <ref name=":3" /> Adults are most commonly affected, however children and the elderly can be affected as well.
===Age===
*Patients of all age groups may develop [disease name].
*[Disease name] is more commonly observed among patients aged [age range] years old.
*[Disease name] is more commonly observed among [elderly patients/young patients/children].
===Gender===
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
===Race===
*There is no racial predilection for [disease name].
*[Disease name] usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop [disease name].
   
   
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*Common risk factors in the development of eosinophilia are allergies and parasitic infections.  Eosinophilia is most commonly associated with allergic processes in developed countries and with parasitic infections in developing countries.
   
   
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].  
*Highly variable based on the underlying etiology.  
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*The diseases eosinophilia can be associated with have a wide range of natural histories and prognoses.  
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*An asthmatic patient with eosinophilia has a very different prognosis than a cancer patient with eosinophilia
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Hypereosinophilic syndromes have a poor prognosis and high mortality, mostly from the cardiac effects of hypereosinophilia
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].
   
   
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
*The diagnosis of eosinophilia is made when the peripheral blood eosinophil count is greater than 500 cells/microL.
:*[criterion 1]
*Hypereosinophilic syndrome usually has eosinphil counts greater than 1500 cells/microL.  Hypereosinophilic syndrome also requires the presence of organ dysfunction that is caused by the eosinophils and cannot be explained by another disease process <ref name=":3" /><ref name=":0">Cogan and Roufosse.  Clinical Management of the Hypereosinophilic Syndromes. Expert Reviews in Hematology 5(3) 275-290 (2012).</ref>.
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
   
=== Symptoms ===
=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms seen in eosinophilia are highly variable and reflect the underlying disease.
*Symptoms of [disease name] may include the following:
*Hypereosinophilia can cause symptoms due to the release of destructive compounds from eosinophils. <ref name=":3" /><ref name=":2" /><ref name=":0" /> Symptoms attributable to the elevated eosinophil count include:
:*[symptom 1]
**Dermatologic manifestations insluding rash, urticaria
:*[symptom 2]
**Muscle and joint pains
:*[symptom 3]
**Heart failure and endocardial fibrosis
:*[symptom 4]
**Cough and shortness of breath
:*[symptom 5]
**Thrombotic and embolic events
:*[symptom 6]
**Encephalopathy or neuropsychiatric disturbances
**Peripheral neuropathies
**Abdominal pain, nausea/vomiting, diarrhea, weight loss
**Any organ can be affected by hypereosinophilia and manifestations are variable
=== Physical Examination ===
=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
* Physical exam findings in eosinophilia are highly variable and reflect the underlying disease.
*Physical examination may be remarkable for:
* Physical exam findings in a hypereosinophilic syndrome are also highly variable, since the disease can affect any organ system.  Possible manifestations include:
:*[finding 1]
** Signs of volume overload related to heart failure: peripheral edema, pulmonary edema, engorged neck veins, etc.
:*[finding 2]
** Rash, skin ulcerations, lichenification
:*[finding 3]
** Altered mental status
:*[finding 4]
** Respiratory distress
:*[finding 5]
 
:*[finding 6]
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*There are no specific laboratory findings associated with eosinophilia aside from the elevated eosinophil count.
 
*Anemia and thrombocytopenia or thrombocytosis may be seen if the eosinophilia is related to a clonal proliferative process in the bone marrow. <ref name=":0" />
*A  [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*There are no imaging findings associated with eosinophilia unless it is secondary to a disease process that causes imaging findings.
*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
   
   
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*Medical treatment for eosinophilia should be directed at the underlying cause (for example treating parasitic infection or malignancy).
*Eosinophilia without evidence of organ dysfunction may be observed with monitoring for evidence of organ involvement.
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*Treatment of hypereosinophilic syndrome has a number of options including [[corticosteroids]], [[hydroxyurea]], [[interferon-alpha]], [[imatinib]] and other tyrosine kinase inhibitors, IL-5 antibodies, and other cytotoxic and immunomodulatory agents. <ref name=":3" /><ref name=":0" /> <ref name=":1">Ogbogu et al. Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy.  Journal of Allergy and Clinical Immunology (2009) 124(6):1319–1325</ref>
*[Medical therapy 1] acts by [mechanism of action 1].
 
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*Surgery may be part of the treatment for certain causes of eosinophilia, such as solid organ malignancies. However most conditions associated with eosinophilia are not treated surgically.
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
   
   
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*There are no primary preventive measures available for eosinophilia.
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].


*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].  
*Primary prevention should be targeted as specific disease entities.  


==References==
==References==
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[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Infectious disease]]

Latest revision as of 17:41, 18 September 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: George Leef MD

Overview

Eosinophilia refers to elevated eosinophils in the peripheral blood. It is important to remember that eosinophilia is a symptom, not a disease. Eosinophilia can be a manifestation of a variety of different disease processes, including neoplastic, infectious, allergic, and idiopathic processes.

When the eosinophil count is markedly elevated and the patient has symptoms attributable to the eosinophils (and not explained by the underlying condition, if any), it is referred to as a hypereosinophilic syndrome. [1] Most patients with eosinophilia do not have a hypereosinophilic syndrome.

Historical Perspective

  • As mentioned above, eosinophilia is not a unified disease entity, but rather a manifestation of many different disease processes.
  • The eosinophil itself was discovered in 1879 [2] as a granulocytic cell that stained with the red dye eosin. Granulocytes in general were described several decades earlier.
  • The hypereosinophilic syndrome was first described in 1968. [3]

Classification

Pathophysiology

  • Eosinophilia is defined by blood eosinophil count > 500/microL.
  • The pathogenesis is variable. Eosinophila can be caused by a proliferative neoplasm (as in eosinophilic leukemia), or as a reaction to another process (parasitic infection, drug reaction, solid tumor) that causes elevated levels in cytokines that promote eosinophil maturation in the bone marrow, or it may be idiopathic.

Causes

  • Eosinophilia can be caused by numerous different processes listed above.

Differentiating eosinophilia from other Diseases

  • If the eosinophil count is elevated, eosinophilia is present by definition. Care should be taken to discover the correct cause for the eosinophilia.

Epidemiology and Demographics

  • Eosinophilia is not a specific disease entity, epidemiologic numbers are availble for major causes of eosinophilia
  • Hypereosinophilic syndromes are extremely rare, estimated at 0.36 to 6.3 people per 100000 in the US. [1] Adults are most commonly affected, however children and the elderly can be affected as well.

Risk Factors

  • Common risk factors in the development of eosinophilia are allergies and parasitic infections. Eosinophilia is most commonly associated with allergic processes in developed countries and with parasitic infections in developing countries.

Natural History, Complications and Prognosis

  • Highly variable based on the underlying etiology.
  • The diseases eosinophilia can be associated with have a wide range of natural histories and prognoses.
  • An asthmatic patient with eosinophilia has a very different prognosis than a cancer patient with eosinophilia
  • Hypereosinophilic syndromes have a poor prognosis and high mortality, mostly from the cardiac effects of hypereosinophilia

Diagnosis

Diagnostic Criteria

  • The diagnosis of eosinophilia is made when the peripheral blood eosinophil count is greater than 500 cells/microL.
  • Hypereosinophilic syndrome usually has eosinphil counts greater than 1500 cells/microL. Hypereosinophilic syndrome also requires the presence of organ dysfunction that is caused by the eosinophils and cannot be explained by another disease process [1][4].

Symptoms

  • Symptoms seen in eosinophilia are highly variable and reflect the underlying disease.
  • Hypereosinophilia can cause symptoms due to the release of destructive compounds from eosinophils. [1][3][4] Symptoms attributable to the elevated eosinophil count include:
    • Dermatologic manifestations insluding rash, urticaria
    • Muscle and joint pains
    • Heart failure and endocardial fibrosis
    • Cough and shortness of breath
    • Thrombotic and embolic events
    • Encephalopathy or neuropsychiatric disturbances
    • Peripheral neuropathies
    • Abdominal pain, nausea/vomiting, diarrhea, weight loss
    • Any organ can be affected by hypereosinophilia and manifestations are variable

Physical Examination

  • Physical exam findings in eosinophilia are highly variable and reflect the underlying disease.
  • Physical exam findings in a hypereosinophilic syndrome are also highly variable, since the disease can affect any organ system. Possible manifestations include:
    • Signs of volume overload related to heart failure: peripheral edema, pulmonary edema, engorged neck veins, etc.
    • Rash, skin ulcerations, lichenification
    • Altered mental status
    • Respiratory distress

Laboratory Findings

  • There are no specific laboratory findings associated with eosinophilia aside from the elevated eosinophil count.
  • Anemia and thrombocytopenia or thrombocytosis may be seen if the eosinophilia is related to a clonal proliferative process in the bone marrow. [4]

Imaging Findings

  • There are no imaging findings associated with eosinophilia unless it is secondary to a disease process that causes imaging findings.

Treatment

Medical Therapy

  • Medical treatment for eosinophilia should be directed at the underlying cause (for example treating parasitic infection or malignancy).
  • Eosinophilia without evidence of organ dysfunction may be observed with monitoring for evidence of organ involvement.
  • Treatment of hypereosinophilic syndrome has a number of options including corticosteroids, hydroxyurea, interferon-alpha, imatinib and other tyrosine kinase inhibitors, IL-5 antibodies, and other cytotoxic and immunomodulatory agents. [1][4] [5]

Surgery

  • Surgery may be part of the treatment for certain causes of eosinophilia, such as solid organ malignancies. However most conditions associated with eosinophilia are not treated surgically.

Prevention

  • There are no primary preventive measures available for eosinophilia.
  • Primary prevention should be targeted as specific disease entities.

References

  1. 1.0 1.1 1.2 1.3 1.4 National Institute of Allergy and Infectious Disease, Hypereosinophilic Syndrome. http://www.niaid.nih.gov/topics/EosinophilicDisorders/Pages/Hyper.aspx
  2. Kay AB. The early history of the eosinophil. Clin Exp Allergy. 2015 Mar;45(3):575-82.
  3. 3.0 3.1 3.2 Brito-Babapulle. The Eosinophilias including the Hypereosinophilic Syndrome. British Journal of Hematology (2003) 121(3):203-223.
  4. 4.0 4.1 4.2 4.3 Cogan and Roufosse. Clinical Management of the Hypereosinophilic Syndromes. Expert Reviews in Hematology 5(3) 275-290 (2012).
  5. Ogbogu et al. Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy. Journal of Allergy and Clinical Immunology (2009) 124(6):1319–1325