On the other hand, cardiac tamponade is a very rare presentation of IHES, and few anecdotal cases exist in literature. We report on IHES in a patient who presented with cardiac tamponade. [ncbi.nlm.nih.gov]

• Splenomegaly

  The most common clinical manifestations were splenomegaly and cardiac involvement. Corticosteroids (CS) as monotherapy were initiated in all study patients. [ncbi.nlm.nih.gov]

  Patients often have Splenomegaly Thrombocytopenia Anemia Elevated serum vitamin B12 levels Hypogranular or vacuolated eosinophils Myelofibrosis Patients with the myeloproliferative subtype often develop endomyocardial fibrosis and may rarely develop acute [merckmanuals.com]

  Gastrointestinal tract - tract inflammation, infarction of the gut secondary to emboli, splenomegaly, ascites, hepatitis, pancreatitis. Neurological - acute cerebrovascular accident, confusion, ataxia, peripheral neuropathy. [patient.info]

  Classification and characteristics of HES a HES variants Characteristics Parameters Sub types Myeloproliferative Clinical Hepatomegaly Splenomegaly M-HES: Features of myeloproliferative disease without proof of clonality Blood Myeloid precursors Anaemia [academic.oup.com]

  In NIH series_ hematological involvement was seen in all, pulmonary in 40%, skin in 56%, neurological in 64%, splenomegaly in 45%, hepatomegaly in 35%, cardiovascular in 54% and ocular involvement in 18%(1,8). [indianpediatrics.net]

• Fatigue

  A 17-year-old man was referred for dyspnea, fatigue, and fever. Idiopathic hypereosinophilic syndrome was diagnosed. Transthoracic echocardiography demonstrated multiple intracardiac thrombi in the left ventricular apex. [ncbi.nlm.nih.gov]

  It usually presents with fever, weight loss, fatigue, and rash. An enlarged liver and spleen and liver is often present. The lungs, kidneys, heart, and nervous system can also be affected. [checkorphan.org]

  Early clinical features include fatigue, diarrhea, and rash. Prognosis is generally poor, and the 3-year survival rate of patients with hypereosinophilic syndrome is approximately 12%. [wikidoc.org]

  These symptoms include: Skin rashes such as urticaria or angioedema Dizziness Memory loss or confusion Cough Shortness of breath Fatigue Fever Mouth sores Diagnosis Symptoms of HES are also common in many other medical problems, making an initial diagnosis [aaaai.org]

  Generalised symptoms - fatigue, aches and pains, fever, night sweats and pruritus. Diarrhoea is common, as are abdominal pain and nausea. [patient.info]

• Constitutional Symptom

  Such digestive complications are observed in all forms of HES, including those with unknown pathogenesis. • Constitutional symptoms include weakness, fatigue, anorexia, fever, night sweats, weight loss, arthralgia and myalgia. [ojrd.biomedcentral.com]

• Abdominal Pain

  A 64-year-old man had dysphagia, swelling of the oral mucosa and the posterior cervical muscles, accompanied by an abdominal pain and diarrhea. Peripheral blood cell count showed marked eosinophilia. [ncbi.nlm.nih.gov]

  Generalised symptoms - fatigue, aches and pains, fever, night sweats and pruritus. Diarrhoea is common, as are abdominal pain and nausea. [patient.info]

  The common gastrointestinal symptoms in a case of HES include abdominal pain, diarrhea, nausea, and vomiting. [ehj.eg.net]

  […] regulatory disorder of the immune system, parasitosis, food and drug allergies, malignancy, tuberculosis, and chronic vesicle irritation. [1] The most common presenting symptoms include frequency, dysuria, gross or microscopic hematuria, suprapubic and abdominal [sjkdt.org]

  Other allergic-type symptoms may include abdominal pain and difficulty breathing. Over 90% of patients with systemic mastocytosis carry a mutation in the c-KIT gene. [leukaemia.org.au]

• Nausea

  Alcohol intolerance with abdominal pain, flushing, and nausea. Organ involvement in HES [ 6 ] This can be manifold and the following are some examples: Blood - thrombocytopenia, hypercoagulability. [patient.info]

  The common gastrointestinal symptoms in a case of HES include abdominal pain, diarrhea, nausea, and vomiting. [ehj.eg.net]

  Lymphadenopathy Splenomegaly Thromboembolic phenomena Thrombocytopenia Neurologic > 50% Cerebral emboli with focal deficits Diffuse encephalopathy with altered behavior and cognitive function and spasticity Peripheral neuropathy GI > 40% Abdominal cramps Diarrhea Nausea [merckmanuals.com]

  Electrolyte disturbances Malignant hypercalcaemia Few reports of hypercalcemia related to idiopathic HES have been described [ 28–30 ] It is often a symptomatic (general fatigue, loss of appetite, nausea, and difficulty falling asleep) malignant (11.7 [academic.oup.com]

  • Gastrointestinal symptoms may include abdominal pain, diarrhea, nausea and vomiting. [ojrd.biomedcentral.com]

• Skin Disease

  He also had erythema elevatum diutinum, a rare skin disease of the vasculitic type. No cardiomyopathy was present. Pleural effusions, skin lesions, and blood eosinophilia disappeared with prolonged corticosteroid treatment. [erj.ersjournals.com]

  […] tissue disorders – Scleroderma, polyarteritis nodosa Skin diseases – Dermatitis herpetiformis Inflammatory bowel disease Sarcoidosis Addison disease Clonal eosinophilia Clonal eosinophilia is diagnosed by bone marrow histology, cytogenetics, and molecular [emedicine.medscape.com]

  […] tissue disease such as rheumatoid arthritis, infectious disease such as scabies, HIV (human immunodeficiency virus), HTLV (human T cell lymphotropic virus), and allergic bronchopulmonary aspergillosis, skin disease such as psoriasis, endocrine disease [ojrd.biomedcentral.com]

• Arthralgia

  • Constitutional symptoms include weakness, fatigue, anorexia, fever, night sweats, weight loss, arthralgia and myalgia. Biological findings HES patients are also heterogeneous with regard to associated biological features. [ojrd.biomedcentral.com]

• Headache

  Although he had hypereosinophilia for more than 6 months, he was asymptomatic until the sudden onset of vomiting and headache due to superior sagittal sinus thrombosis. [ncbi.nlm.nih.gov]

  Case Report Presenting complaints A 7-year-old boy presented with complaints of intermittent fever, headache, body ache, itchy papular skin rashes with cervical and axillary lymphadenopathy since last 2 years [Figure 1]. [amhsjournal.org]

Her workup during admission revealed absolute eosinophil count of 17,447 and bone marrow biopsy showed eosinophilia with no lymphoproliferative process. [ncbi.nlm.nih.gov]

If this workup is unrevealing, idiopathic HES should be considered. [hematologyandoncology.net]

An initial workup revealed normal urine analysis and a negative urine culture. A complete blood count (CBC) showed significant eosinophilia (34%), with an absolute esosinophil count of 4700. [sjkdt.org]

Imatinib mesylate has shown some promise in the treatment of HES. [ncbi.nlm.nih.gov]

Idiopathic hypereosinophilic syndrome remains a serious condition with a poor prognosis for most patients. [ncbi.nlm.nih.gov]

Eosinophilic cholangiopathy is a rare cholestatic disorder of unknown etiology. [ncbi.nlm.nih.gov]

The data on patients' characteristics, epidemiology, clinical features, diagnosis, treatment and outcome were collected and analysed. [ncbi.nlm.nih.gov]

Hypereosinophilic syndromes: Epidemiology, clinical manifestations, and diagnosis. http://www.uptodate.com/home. Accessed Feb. 9, 2017. Weller PF, et al. Approach to the patient with unexplained eosinophilia. http://www.uptodate.com/home. [mayoclinic.org]

Epidemiology [ 4 ] HES is rare in adults and tends to be under-diagnosed. It is even rarer in children. [ 5 ] There are no formal patient registers and lack of published data makes estimates of prevalence difficult. [patient.info]

[…] 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]

Epidemiology Surveillance, Epidemiology, and End Results (SEER) database • Prevalence of HES or chronic eosinophilic leukemia in the United States is between 0.3 and 6.3 cases per 100,000 person-years Crane MM, Chang CM, Kobayashi MG, Weller PF. [slideshare.net]

Little is known about the pathophysiology and potential triggers of this condition. Approximately 40-70% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. [ncbi.nlm.nih.gov]

The other possibility is that the skin changes emerge in coincidence with HES. 1 Institute for Pathophysiology, School of Medicine, Zaloška cesta 4, 1000 Ljubljana; 2 Department for Interstitial Pulmonary Disease, Institute of Pulmonary Medicine and Pathology [journals.lww.com]

The idiopathic hypereosinophilic syndrome: Clinical, pathophysiologic and therapeutic considerations. Ann Int Med. 1982;97:78–92. Google Scholar 11. Smit AJ, van Essen LH, de Vries EG. [link.springer.com]

Pathophysiology Many cases of clonal HES harbor the FIP1L1-PDGFRA fusion gene. FIP1L1-PDGFRA is a fusion protein generated by an interstitial deletion on chromosome 4. [cancertherapyadvisor.com]

These findings suggest that earlier treatment and a sufficient dose of corticosteroids may prevent the lesional expansion in acute myelitis. [ncbi.nlm.nih.gov]

Prevention - Idiopathic hypereosinophilic syndrome Information not available. [checkorphan.org]

Prevention 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]

Medications known as antihistamines are used to prevent and reduce allergic reactions. [leukaemia.org.au]