No ring sideroblasts are present. Scattered mature mast cells are present (2%). [pathologyoutlines.com]

Hypereosinophilic syndrome usually presents with fever, weight loss, fatigue, and rash. A rash is present in over 50% of patients, but is non-specific in appearance. Most commonly, red swollen itchy nodules (lumps) have been described. [dermnetnz.org]

A follow-up examination at 6 months revealed no motor or sensory impairment, with the patient presenting a normal blood eosinophil count. [elsevier.es]

Angiolymphoid hyperplasia with eosinophilia: Presents with eosinophilia and papules, plaques, and nodules on the head and neck. Kimura disease presents with eosinophilia and large subcutaneous masses on the head or neck. Typically seen in Asian men. [unboundmedicine.com]

He presented with cushingoid facies, knee arthritis, violet streaks on the abdomen and darkened skin with erythematous and papular lesions on his face, limbs and trunk (Figure 1). He had no kidney injury, hepatomegaly or splenomegaly. [htct.com.br]

• Splenomegaly

  FDA-regulated Device Product: No Product Manufactured in and Exported from the U.S.: No Additional relevant MeSH terms: Layout table for MeSH terms Hepatomegaly Hypereosinophilic Syndrome Eosinophilia Syndrome Splenomegaly Disease Pathologic Processes [clinicaltrials.gov]

  People with PDGFRA-associated chronic eosinophilic leukemia can also have an enlarged spleen (splenomegaly) and elevated levels of certain chemicals called vitamin B12 and tryptase in the blood. [medlineplus.gov]

  CHIC2 site that causes the FIP1L1/PDGFRA-associated fusion gene (which has tyrosine kinase activity that can transform hematopoietic cells).Patients often have Anemia Elevated serum vitamin B12 levels Hypogranular or vacuolated eosinophils Myelofibrosis Splenomegaly [msdmanuals.com]

  It was clear from that time that a subgroup of patients with HES had clinical and hematological characteristics of myeloproliferative disease, such as splenomegaly, anemia and myelofibrosis. [ctajournal.biomedcentral.com]

  He had no kidney injury, hepatomegaly or splenomegaly. Ceftriaxone and azithromycin were prescribed to treat communitary pneumonia and the prednisone dosage was reduced to 15mg/day. [htct.com.br]

• Palpable Spleen

  Complete response (CR) = normalization of white blood cell (WBC) count; absolute eosinophil count in blood; and % eosinophils in blood, without increased blasts or eosinophils in bone marrow, and with a non-palpable spleen and/ or normal spleen size by [clinicaltrials.gov]

• Anemia

  Morphologically, RBCs show normochromic, normocytic anemia with mild anisopoikilocytosis. WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. [pathologyoutlines.com]

  Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell count greater than 100,000 cells/cm3, abnormal marrow and/or basophils, elevated serum levels of vitamin B12, serum tryptase [wikidoc.org]

  Repeat testing may be indicated for eosinophilia (typically considered >500 cells/µL) but may not be required if absolute counts exceed 1,500 cells/µL, especially in a symptomatic patient (anemia and thrombocytopenia detected on CBC may indicate a neoplasm [arupconsult.com]

  […] myeloproliferative variant is often associated with a small interstitial deletion in chromosome 4 at the CHIC2 site that causes the FIP1L1/PDGFRA-associated fusion gene (which has tyrosine kinase activity that can transform hematopoietic cells).Patients often have Anemia [msdmanuals.com]

  It was clear from that time that a subgroup of patients with HES had clinical and hematological characteristics of myeloproliferative disease, such as splenomegaly, anemia and myelofibrosis. [ctajournal.biomedcentral.com]

• Cough

  Common and early HES symptoms include fatigue, cough, shortness of breath, myalgia, skin rash, and fever. Symptoms of hypereosinophilia correlate with areas of the body affected by high levels of eosinophils. [icliniq.com]

  Acute eosinophilic pneumonia is an idiopathic disease that presents with fever, cough, dyspnea, and hypoxemia occurring for days to weeks, typically in males and in individuals who have recently started to smoke tobacco. [unboundmedicine.com]

  Symptoms Symptoms of eosinophilia are generally related to the underlying condition, and may include: • Wheezing • Breathlessness • Coughing • Abdominal pain • Diarrhea • Fever • Rashes • Weight loss • Night sweats • Enlarged lymph nodes • Numbness and [cancer.columbia.edu]

  Symptoms Early symptoms of HES may include fatigue, cough, breathlessness, muscle pain, rash and fever. Causes Some varieties of hypereosinophilic syndrome tend to run in families. [beaconhealthsystem.org]

• Dyspnea

  During the hospital stay, the dyspnea worsening and a new thorax CT was performed, which revealed a 7.0cm diameter cavity in the right hemithorax. [htct.com.br]

  Any organ or tissue may be affected and myalgia, arthritis, dyspnea, pruritus, skin lesions and other symptoms may develop.33 Gotlib J. [scielo.br]

  Acute eosinophilic pneumonia is an idiopathic disease that presents with fever, cough, dyspnea, and hypoxemia occurring for days to weeks, typically in males and in individuals who have recently started to smoke tobacco. [unboundmedicine.com]

  Common manifestations include brain or lung hypoxia (eg, encephalopathy, dyspnea or respiratory failure). 1. [msdmanuals.com]

  The accumulation of eosinophils in the lungs can cause recurrent upper respiratory tract infections, coughing, and dyspnea. Symptoms of HES are based on the affected area and include: Skin - Eczema, thickening of the skin, hives. [icliniq.com]

• Thrombosis

  […] of 43yo F with thrombi filling both ventricles cause completely unknown Endomyocardial Fibrosis With Ventricular Thrombosis: Gross fixed tissue good color horizontal section of ventricles endocardial fibrosis thrombus and focal myocardial fibrosis are [wikidoc.org]

  However, idiopathic HS does not meet these diagnostic criteria.2 From a neurological viewpoint, idiopathic HS is characterised by involvement of both the central nervous system (venous thrombosis and encephalopathy) and the peripheral nervous system [elsevier.es]

  Association of Thrombosis With Hypereosinophilic Syndrome in Patients With Genetic Alterations. JAMA Netw Open. 2021 Aug 2. 4 (8):e2119812. [QxMD MEDLINE Link]. [Full Text]. Klion A. [emedicine.medscape.com]

  […] other cells including lymphocytes, mast cells, and fibroblasts.1 When appropriately targeted, granule release helps control pathogens, while inappropriate release has been implicated in the development of allergic inflammation, fibrosis, vasculitis, and thrombosis [emjreviews.com]

• Hepatosplenomegaly

  Some possible symptoms a patient may present with include: Cardiomyopathy[5] Skin lesions[5] Thromboembolic disease[5] Pulmonary disease[5] Neuropathy[5] Hepatosplenomegaly[5] Reduced ventricular size[5] Atopic eczema Diagnosis[edit] Numerous techniques [en.wikipedia.org]

• Hepatomegaly

  White Cells Blasts More Than 5 Percent of Bone Marrow Nucleated Cells Blasts More Than 5 Percent of Peripheral Blood White Cells Blasts Under 20 Percent of Bone Marrow Nucleated Cells Chronic Eosinophilic Leukemia, Not Otherwise Specified Eosinophilia Hepatomegaly [clinicaltrials.gov]

  He had no kidney injury, hepatomegaly or splenomegaly. Ceftriaxone and azithromycin were prescribed to treat communitary pneumonia and the prednisone dosage was reduced to 15mg/day. [htct.com.br]

  He had no kidney injury, hepatomegaly or splenomegaly. Figure 1 A and B before Imatinib. Ceftriaxone and azithromycin were prescribed to treat communitary pneumonia and the prednisone dosage was reduced to 15 mg/day. [scielo.br]

  Myeloid Hypereosinophilic Syndrome Patients with M-HES are characterized by hepatomegaly, splenomegaly, circulating myeloid precursors, increased serum vitamin B12 and/or tryptase levels, anemia, thrombocytopenia, hematologic diseases (myeloid fibrosis [mdpi.com]

  Classification Myeloproliferative forms of HES • Clinical Hepatomegaly, Splenomegaly • Laboratory - Circulating myeloid precursors, Increased serum vitamin B12 or Tryptase, Anemia, Thrombocytopenia - Hematologic (myeloid fibrosis, left shift in maturation [slideshare.net]

• Ulcer

  This time there were pustules and ulcers of the oral mucosa, and on the elbows and back (Figure 3). [htct.com.br]

  Hypereosinophilic syndrome Idiopathic hypereosinophilic syndrome (HES), an extremely high eosinophil count of unknown origin Lymphatic filariasis (a parasitic infection) Ovarian cancer Parasitic infection Primary immunodeficiency Trichinosis (a roundworm infection) Ulcerative [mayoclinic.org]

  Skin manifestations vary greatly: pruritus, skin rashes, nodules, mucous ulcerations, and oedema. [marih.fr]

  The nature of skin symptoms is highly variable, with some having cutaneous erythema or eczema while others may have more severe symptoms such as urticaria, angioedema, or even ulceration. [emjreviews.com]

• Angioedema

  Episodic angioedema with eosinophilia (Gleich syndrome) is a rare disease that leads to recurrent attacks of fever, angioedema, urticaria, weight gain, and blood eosinophilia without other organ damage. [unboundmedicine.com]

  The nature of skin symptoms is highly variable, with some having cutaneous erythema or eczema while others may have more severe symptoms such as urticaria, angioedema, or even ulceration. [emjreviews.com]

  For example, in one study, serial skin biopsies from patients with episodic angioedema with eosinophilia demonstrated EGP staining only when symptoms were present (15, 34). [frontiersin.org]

  Patients more often have Angioedema, skin abnormalities, or both Circulating immune complexes (sometimes with serum sickness) Hypergammaglobulinemia (especially high IgE) Patients with the lymphoproliferative variant also more often respond favorably [msdmanuals.com]

  Episodic hypereosinophilia is known as Gleich’ syndrome or episodic angioedema with eosinophilia (EAE), a rare disease characterized by recurrent angioedema accompanied by hypereosinophilia, with cyclical variable clinical pattern. [ctajournal.biomedcentral.com]

• Urticaria

  It can also resemble urticaria (hives). An enlarged liver and spleen is often present indicating liver and spleen involvement. The lungs, kidneys, heart and nervous system can be affected. [dermnetnz.org]

  Episodic angioedema with eosinophilia (Gleich syndrome) is a rare disease that leads to recurrent attacks of fever, angioedema, urticaria, weight gain, and blood eosinophilia without other organ damage. [unboundmedicine.com]

  The nature of skin symptoms is highly variable, with some having cutaneous erythema or eczema while others may have more severe symptoms such as urticaria, angioedema, or even ulceration. [emjreviews.com]

  […] most common, followed by pulmonary, gastrointestinal, cardiac and lastly neurologic 6% of patients presented initially with incidentally detected asymptomatic hypereosinophilia Dermatologic: angioedema, dermographism, eczema, erythroderma, pruritis, urticaria [pathologyoutlines.com]

  Features that indicate a favorable prognosis in hypereosinophilic syndrome include the following: Angioedema Urticaria Elevated serum IgE level Sustained response to corticosteroids Early diagnosis and intensive management The presence of features that [emedicine.medscape.com]

Testing for Secondary/Reactive Eosinophilia A broad range of conditions can result in secondary/reactive eosinophilia, and a clinically driven workup is needed to determine cause. [arupconsult.com]

Specifically, lung involvement is evaluated using pulmonary function testing, bronchoscopy, and serologic tests.1 Once any secondary causes are excluded, the workup should evaluate for primary (clonal) eosinophilia. [consultant360.com]

As an additional workup, no lymphocyte clonality was found by flow cytometry. [htct.com.br]

[…] splenomegaly and lymphadenopathy Liver, CNS, muscle, pulmonary and nasal sinus involvement occur more commonly than in eosinophilic leukemia (Haematologica 2014;99:e148) Etiology Cause is unknown Diagrams / tables Images hosted on other servers: Algorithm workup [pathologyoutlines.com]

• Neutrophilia

  WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. Occasional circulating immature granulocytic precursors, e.g. metamyelocytes and myelocytes, are noted. [pathologyoutlines.com]

• Anisopoikilocytosis

  Morphologically, RBCs show normochromic, normocytic anemia with mild anisopoikilocytosis. WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. [pathologyoutlines.com]

• Normocytic Anemia

  Morphologically, RBCs show normochromic, normocytic anemia with mild anisopoikilocytosis. WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. [pathologyoutlines.com]

• Hypercellular Bone Marrow

  Another myelogram showed hypercellular bone marrow with 78% of cells being granulocytes (27% eosinophils and 51% eosinophil precursors) of hypoblastic erythroid lineage and hypercellular megakaryocyte lineage, presenting monolobulation and polylobulation [htct.com.br]

Experimental: Treatment (ruxolitinib) Patients receive ruxolitinib PO BID on days 1-28. [clinicaltrials.gov]

Specific treatment depends on your symptoms, the severity of your condition and the cause of your HES. [mayoclinic.org]

Future treatments may involve the JAK1/2 inhibitor ruxolitinib (and other JAK inhibitors). [consultant360.com]

Cardiovascular complications of HES represent the worst prognosis, responsible for a high rate of fatal outcome. [ctajournal.biomedcentral.com]

Prognosis is generally poor, and the 3 year survival rate of patients with hypereosinophilic syndrome is approximately 12% Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell [wikidoc.org]

Prognosis Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. Blast transformation may occur after many years. [emedicine.medscape.com]

Outlook / Prognosis What can I expect if I have hypereosinophilic syndrome? Your prognosis, or anticipated outcome, depends on your specific situation. [my.clevelandclinic.org]

Prognosis The prognosis in HES depends on the organ systems involved, disease severity and response to therapy. Outcomes can vary greatly from one person to the next. There is no cure. If HES is left untreated, the disease may be fatal. [apfed.org]

Clinically there are no identifiable etiologies of reactive eosinophilia such as allergic reactions, parasitic infections, autoimmune diseases and medications. [pathologyoutlines.com]

Identifying the etiology is essential for treatment decisions, and treating the underlying disorder often will resolve the eosinophilia. [arupconsult.com]

Eosinophilia of any etiology can cause the same types of tissue damage. [msdmanuals.com]

[…] eosinophilic disorder–was approximately 0.4 cases per 1,000,000 according to the International Classification of Disease for Oncology (version 3), and coding of 9964/3, the Surveillance, Epidemiology and End Results (SEER) database from 2004 to 2015.1 Etiology [consultant360.com]

Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. [1, 2, 3, 4, 5, 6, 7] HES is classified into primary (clonal) HES, reactive HES, and (when the etiology [emedicine.medscape.com]

Causes of reactive eosinophilia have a significant regional influence worldwide and regional large epidemiologic studies would be needed for a global evaluation of the problem. [ctajournal.biomedcentral.com]

[…] may be treated with the monoclonal antibody mepolizumab currently being developed to treat the disease.[7] If this becomes successful, it may be possible for corticosteroids to be eradicated and thus reduce the amount of side effects encountered.[7] Epidemiology [en.wikipedia.org]

Eosinophil granule proteins, including eMBP1, have various and numerous toxic effects on tissues and organs, and their detection has important pathophysiologic and treatment implications. [arupconsult.com]

The pathophysiology and clinical presentation of eosinophilic disorders are highly heterogeneous and disease outcome may vary from asymptomatic or mild, to severe and fatal, with variable time course patterns. [ctajournal.biomedcentral.com]

The role of eosinophils as secretory cells and mediators of the cytotoxic response seems to explain the pathophysiology of peripheral neuropathy, particularly major basic protein and eosinophil-derived neurotoxin, which are responsible for neuropathic [elsevier.es]

Their development depends entirely on which organ systems are involved in the disease process (see Presentation and Pathophysiology). [emedicine.medscape.com]

Such a complex clinical picture causes difficulties in evaluation of eosinophilic syndromes clinically, which results in potentially severe disease outcomes.5 Conclusion The pathophysiologic mechanisms of eosinophilic disorders are well characterized. [consultant360.com]

Clinical trials Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition. [mayoclinic.org]

Prevention[edit] There are no primary preventive measures available for hypereosinophilic syndrome.[6] There are no effective measures for the primary prevention of hypereosinophilic syndrome. [wikidoc.org]

X-rays, to check the condition of your lungs CT scan, to detect problems in the chest, abdomen and pelvis Echocardiogram or MRI, to assess heart function Treatment Treatment for hypereosinophilic syndrome is aimed at reducing your eosinophil count to prevent [beaconhealthsystem.org]

Treatment Treatment goals include decreasing blood eosinophil numbers, preventing organ damage, and slowing disease progression. [apfed.org]