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| | '''For patient information, click [[Hypereosinophilic syndrome (patient information)|here]]''' |
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| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} | | | Name = {{PAGENAME}} | |
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| OMIM = 607685 | | | OMIM = 607685 | |
| MedlinePlus = | | | MedlinePlus = | |
| eMedicineSubj = med | | | eMedicineSubj = | |
| eMedicineTopic = 1076 | | | eMedicineTopic = | |
| eMedicine_mult = {{eMedicine2|derm|920}} | | | eMedicine_mult = | |
| MeshID = D017681 | | | MeshID = D017681 | |
| }} | | }} |
| {{Search infobox}} | | {{Hypereosinophilic syndrome }} |
| {{CMG}} | | {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} |
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| '''Associate Editor-In-Chief:''' {{CZ}}
| | ==[[Hypereosinophilic syndrome overview|Overview]]== |
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| {{Editor Help}}
| | ==[[Hypereosinophilic syndrome classification|Classification]]== |
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| ==Overview== | | ==[[Hypereosinophilic syndrome historical perspective|Historical Perspective]]== |
| The '''hypereosinophilic syndrome''' is a disease process characterized by a persistently elevated [[eosinophil]] count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause after a careful workup, with evidence of involvement of either the [[heart]], [[nervous system]], or [[bone marrow]].
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| ==Classification== | | ==Pathophysiology== |
| There are two forms of the hypereosinophilic syndrome: '''Endomyocardial fibrosis''' and '''[[Loeffler's endocarditis]]'''.
| | [[Hypereosinophilic syndrome pathophysiology#Pathophysiology|Pathophysiology]] | [[Hypereosinophilic syndrome pathophysiology#Relationship to chronic eosinophilic leukemia|Relationship to chronic eosinophilic leukemia]] | [[Hypereosinophilic syndrome pathophysiology#Microscopic pathology|Microscopic pathology]] |
| * Endomyocardial fibrosis (also known as Davies disease) is seen in Africa and South America.
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| * Loeffler's endocarditis does not have any geographic predisposition.
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| ==Presentation== | | ==[[Hypereosinophilic syndrome epidemiology and demographics|Epidemiology & Demographics]]== |
| In both forms of the hypereosinophilic syndrome, the eosinophilia causes infiltration of the [[myocardium]] of the [[heart]], which leads to fibrotic thickening of portions of the heart. The portions of the heart most affected by this disease are the apex of the [[left ventricle|left]] and [[right ventricle|right]] ventricles, but fibrotic infiltrations may also involve the mitral or tricuspid valves. Because of the infiltrative nature of the disease process, the cavity of the ventricles of the heart diminish in size, causing an obliterative [[cardiomyopathy]] and [[restrictive cardiomyopathy|restriction]] to the inflow of blood in to the chambers of the heart. Ventricular mural thrombi may develop.
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| ==Relationship to chronic eosinophilic leukemia== | | ==[[Hypereosinophilic syndrome epidemiology and demographics|Risk Factors]]== |
| [[Chronic eosinophilic leukemia]] (CEL) is a [[myeloproliferative]] disease which shares many common characteristics with hypereosinophilic syndrome. Many cases of CEL have a characteristic gene rearrangement , ''FIP1L1/PDGFRA'', caused by a sub-microscopic deletion of ~800 thousand base pairs of DNA on [[chromosome 4]].<ref name="pmid12660384">{{cite journal |author=Cools J, DeAngelo DJ, Gotlib J, ''et al'' |title=A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome |journal=N. Engl. J. Med. |volume=348 |issue=13 |pages=1201-14 |year=2003 |pmid=12660384 |doi=10.1056/NEJMoa025217}}</ref> The ''FIP1L1/PDGFRA'' fusion gene causes constitutive activation of the platelet derived growth factor receptor - alpha (PDGFRA). ''FIP1L1/PDGFRA''-positive patients respond well to treatment with [[tyrosine kinase]] inhibitor drugs, such as [[imatinib mesylate]] (Gleevec® or Glivec®).
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| ==Pathological Findings== | | ==[[Hypereosinophilic syndrome screening|Screening]]== |
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| Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. [http://www.peir.net © PEIR, University of Alabama at Birmingham, Department of Pathology]
| | ==[[Hypereosinophilic syndrome causes|Causes]]== |
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| | ==[[Hypereosinophilic syndrome differential diagnosis|Differentiating Hypereosinophilic syndrome from other Diseases]]== |
| <gallery heights="175" widths="175">
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| Image:7131.jpg|Endomyocardial Fibrosis With Ventricular Thrombosis: Gross fixed tissue good color view from right atrium into right ventricle showing top of thrombus in right ventricle strange case of 43yo F with thrombi filling both ventricles cause completely unknown
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| Image:7132.jpg|Endomyocardial Fibrosis With Ventricular Thrombosis: Gross fixed tissue good color horizontal section of ventricles endocardial fibrosis thrombus and focal myocardial fibrosis are evident strange case with no evident cause
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| | ==[[Hypereosinophilic syndrome natural history|Natural History, Complications & Prognosis]]== |
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| | ==Diagnosis== |
| | [[Hypereosinophilic syndrome history and symptoms|History & Symptoms]] | [[Hypereosinophilic syndrome physical examination|Physical Examination]] | [[Hypereosinophilic syndrome staging|Staging]] | [[Hypereosinophilic syndrome laboratory tests|Lab Tests]] | [[Hypereosinophilic syndrome electrocardiogram|Electrocardiogram]] | [[Hypereosinophilic syndrome chest x ray|Chest X Ray]] | [[Hypereosinophilic syndrome CT|CT]] | [[Hypereosinophilic syndrome MRI|MRI]] | [[Hypereosinophilic syndrome echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Hypereosinophilic syndrome other imaging findings|Other Imaging Findings]] | [[Hypereosinophilic syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| | ==Treatment== |
| <gallery heights="175" widths="175">
| | [[Hypereosinophilic syndrome medical therapy|Medical Therapy]] | [[Hypereosinophilic syndrome surgery|Surgery]] | [[Hypereosinophilic syndrome primary prevention|Primary Prevention]] | [[Hypereosinophilic syndrome secondary prevention|Secondary Prevention]] |
| Image:7133.jpg|Endomyocardial Fibrosis With Ventricular Thrombosis: Gross fixed tissue color not to good horizontal section of ventricles showing thrombus filling both which is quite old and in left ventricle endocardial fibrosis and focal myocardial fibrosis cause unknown heart wt 550 gm
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| Image:7134.jpg|Endomyocardial Fibrosis With Ventricular Thrombosis: Gross fixed tissue not to bad color four horizontal sections of ventricles showing lumens filled with thrombus mostly old and on left endocardial fibrosis 43 year old female cause completely unknown ventricles had slowing filled with thrombus
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| | ==Resources== |
| | * {{DermNet|systemic/hypereosinophilic}} |
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| | {{Hematological malignancy histology}} |
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| Image:404930.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME Blood smear from a 50-year-old male with a 3-year history of hypereosinophilic syndrome with progressive bone marrow failure and multiple organ involvement. The three eosinophils show marked hypogranulation. (Wright-Giemsa stain)
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| Image:404931.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME Bone marrow smear from the patient in A showing a marked increase in eosinophils at all stages of maturation. Some of the granules in immature eosinophils have a basophilic color and two of the eosinophil myelocytes have a diminished number of granules. (Wright-Giemsa stain)
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| Image:404932.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME Bone marrow biopsy showing marked hypercellularity due primarily to an increase in eosinophils and precursors. Megakaryocytes are markedly reduced. Increased reticulin fibers are present. Despite intensive chemotherapy there was a progressive increase in marrow fibrosis and number of blasts. The patient died of central nervous system failure 17 months after this specimen was obtained. (Hematoxylin and eosin stain)
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| Image:404933.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME WITH MYELOFIBROSIS Bone marrow biopsy. The marrow is markedly fibrotic. (Hematoxylin and eosin stain)
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| Image:404934.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME WITH MYELOFIBROSIS High magnification of the specimen on the left. Blasts and immature cells are scattered throughout the fibrotic tissue. (Hematoxylin and eosin stain)
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| Image:404935.jpg|BONE MARROW: HYPEREOSINOPHILIC SYNDROME: ULTRASTRUCTURE Electron micrograph of an abnormal eosinophil from the specimen in figure 274B. There is a decreased number of granules. The majority of the granules present are homogeneous in contrast to normal eosinophil granules which have a dense core surrounded by a less dense capsule. (Uranyl acetate-lead citrate stains, X20,000)
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| Image:405204.jpg|BONE MARROW: MAST CELL LEUKEMIA AND EOSINOPHILIA A blood smear from a young woman with mast cell leukemia and marked eosinophilia. There are five eosinophils, one neutrophil, and a large atypical-appearing mast cell with relatively sparse small granules and a slightly lobulated nucleus. This patient had eosinophilia as a presenting feature and was initially thought to have a hypereosinophilic syndrome. Eosinophilia is observed in approximately one third of patients with systemic mast cell disease. (Wright-Giemsa stain)
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| ==References==
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| <references/>
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| ==External links==
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| * {{DermNet|systemic/hypereosinophilic}}
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| {{Hematological malignancy histology}}
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| {{SIB}}
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| [[es:Síndrome hipereosinofílico]] | | [[es:Síndrome hipereosinofílico]] |
| [[pl:Zespół hipereozynofilowy]] | | [[pl:Zespół hipereozynofilowy]] |
| [[zh:嗜酸性粒细胞增多症]] | | [[zh:嗜酸性粒细胞增多症]] |
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
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| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| | [[Category:Types of cancer]] |