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Hypereosinophilia

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https://www.readbyqxmd.com/read/28209946/pdgfr%C3%A1%C2%B5-rearranged-myeloid-neoplasm-with-marked-eosinophilia-in-a-37-year-old-man-and-a-literature-review
#1
Mirela Andrei, Andrei Bandarchuk, Cherif Abdelmalek, Ajay Kundra, Vladimir Gotlieb, Jen Chin Wang
BACKGROUND PDGFRᵝ-positive myeloid neoplasms are rare. Marked leukocytosis (over 100×10⁹/L) with marked eosinophilia (over 10%) has been rarely described in myeloid neoplasms associated with PDGFRᵝ rearrangement. CASE REPORT We report a case of 37-year-old man with myeloid neoplasm associated with PDGFRᵝ rearrangement who presented with marked eosinophilia of 13.3% and leukocytosis with WBC count of 189×10⁹/L. He was found to have PDGFRᵝ locus rearrangement at 5q32-33 by fluorescent in situ hybridization (FISH)...
February 17, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28185879/ctla4-ig-effectively-controls-immune-activation-and-inflammatory-disease-in-a-novel-murine-model-of-leaky-scid
#2
Stéphanie Humblet-Baron, Susann Schönefeldt, Josselyn Garcia-Perez, Frédéric Baron, Emanuela Pasciuto, Adrian Liston
BACKGROUND: Severe combined immunodeficiency (SCID) can be caused by loss-of-function mutations in genes involved in the DNA recombination machinery, such as RAG1, RAG2 or DCLRE1C. Defective DNA recombination causes a developmental block in T cells and B cells, resulting in high susceptibility to infections. Hypomorphic mutations in the same genes can also give a partial loss of T cells, in a spectrum including leaky SCID (LS) and Omenn syndrome (OS). These patients not only develop life-threatening infections due to immunodeficiency, but also develop inflammatory/autoimmune conditions due to the presence of autoreactive T cells...
February 6, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28174010/a-case-of-t-cell-lymphoproliferative-disorder-associated-with-hypereosinophilia-with-excellent-response-to-mycophenolate-mofetil
#3
Amr Hanbali, Marwan Shaheen, Feras Alfraih, Wafa Al-Otaibi, Riad El Fakih, Tarek Owaidah, Syed Ahmed
Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×10(9)/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level...
February 1, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28168065/rapid-change-in-mental-status-in-a-patient-with-hypereosinophilia
#4
Hanyin Wang, John K Erban
We present the case of a 48-year-old female with acute onset altered mental status, who was found to have eosinophilia, elevated troponin, and embolic strokes. Extensive testing for autoimmune, infectious, and coronary artery etiologies was unremarkable. After a cardiac MRI revealed focal myocardial hyperenhancement, the patient underwent an endomyocardial biopsy with findings consistent with eosinophilic myocarditis. The patient was diagnosed of idiopathic hypereosinophilic syndrome and started on prednisone and apixaban...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28124651/aspirin-exacerbated-respiratory-disease-prevalence-diagnosis-treatment-and-considerations-for-the-future
#5
Joshua L Kennedy, Ashley N Stoner, Larry Borish
Aspirin-exacerbated respiratory disease (AERD) is a late onset condition characterized by the Samter triad (aspirin sensitivity [as well as sensitivity to any nonselective cyclooxygenase inhibitor], nasal polyps, asthma) and additional features, including eosinophilic chronic rhinosinusitis, hypereosinophilia, anosmia, frequent absence of atopy, and, intolerance to ingestion of red wine and other alcoholic beverages. The diagnosis is rare, and, because of this, it is also often missed by physicians. However, it is highly overexpressed in patients with severe asthma (and severe chronic rhinosinusitis with nasal polyps), which makes its recognition essential...
November 1, 2016: American Journal of Rhinology & Allergy
https://www.readbyqxmd.com/read/28111898/concurrent-myeloid-sarcoma-atypical-teratoid-rhabdoid-tumor-and-hypereosinophilia-in-an-infant-with-a-germline-smarcb1-mutation
#6
Jonathan L Metts, Sunita I Park, Bruno P Soares, Cindy Fong, Jaclyn A Biegel, Kelly C Goldsmith
We report a 1-year-old female child presenting with hypereosinophilia who was found to have concurrent myeloid sarcoma and a central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT). She was later found to have a germline mutation in SMARCB1. Concurrent hematologic malignancy and CNS AT/RT have not previously been described in the context of a SMARCB1 loss-of-function germline mutation.
January 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28111307/jak1-gain-of-function-causes-an-autosomal-dominant-immune-dysregulatory-and-hypereosinophilic-syndrome
#7
Kate L Del Bel, Robert J Ragotte, Aabida Saferali, Susan Lee, Suzanne M Vercauteren, Sara A Mostafavi, Richard A Schreiber, Julie S Prendiville, Min S Phang, Jess Halperin, Nicholas Au, John M Dean, Emily Jewels, Anne K Junker, Paul C Rogers, Michael Seear, Margaret L McKinnon, Stuart E Turvey
Germline JAK1 gain-of-function mutations cause autosomal dominant immune dysregulation and hypereosinophilia with eosinophilic infiltration of the gastrointestinal tract, massive hepatosplenomegaly and severe atopic dermatitis that can be successfully treated with ruxolitinib, an oral JAK1/2 inhibitor.
January 19, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28065613/anca-negative-churg-strauss-syndrome-presenting-as-acute-multiple-cerebral-infarcts-a-case-report
#8
Klearchos Psychogios, Ilias Evmorfiadis, Spyros Dragomanovits, Athanasios Stavridis, Konstantinos Takis, Loukas Kaklamanis, Stathis Pantelis
Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28052805/hypereosinophilic-syndrome
#9
Heung R Noh, Gil G Magpantay
BACKGROUND: Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterized by persistent eosinophilia associated with multiple organ damage. OBJECTIVE: To increase awareness of the rare but potentially life-threatening disease and to provide a brief overview of the clinical presentation, diagnosis, and management of HES. METHODS: Pertinent data from the patient's clinical course was incorporated with salient articles on the topic...
January 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28028030/myeloid-neoplasms-with-eosinophilia
#10
REVIEW
Andreas Reiter, Jason Gotlib
Molecular diagnostics has generated substantial dividends in dissecting the genetic basis of myeloid neoplasms with eosinophilia. The family of diseases generated by dysregulated fusion tyrosine kinase (TK) genes is recognized by the World Health Organization (WHO) category, "Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2" In addition to myeloproliferative neoplasms (MPN), these patients can present with myelodysplastic syndrome/MPN, as well as de novo or secondary mixed-phenotype leukemias or lymphomas...
February 9, 2017: Blood
https://www.readbyqxmd.com/read/28018451/eosinophilic-gastroenteritis-in-an-18-year-old-male-with-prolonged-nephrotic-syndrome
#11
Da Min Choi, Jung Eun Pyun, Hyung Eun Yim, Kee Hwan Yoo, Jung Ok Shim, Eun Jung Lee, Nam Hee Won
Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27955822/febuxostat-associated-eosinophilic-polymyositis-in-marginal-zone-lymphoma
#12
Georges Chahine, Khalil Saleh, Claude Ghorra, Nathalie Khoury, Nadine Khalife, Fouad Fayad
Febuxostat is an orally administered selective inhibitor of xanthine oxidase approved for the treatment of gout and prevention of tumor lysis syndrome. It is a relatively safe medication. Hypersensitivity reactions associated with the use of febuxostat are quite rare with only one reported case of DRESS syndrome. Recently, two case reports of rhabdomyolysis following the initiation of febuxostat were published. We hereby present the first case of rhabdomyolysis with hypereosinophilia following the administration of febuxostat to a 50-year-old patient newly diagnosed with marginal zone lymphoma...
December 7, 2016: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/27883350/loeffler-endocarditis-in-young-woman-a-case-report
#13
Natalia Y Osovska, Natalia V Kuzminova, Irina I Knyazkova
Loeffler endocarditis is a rare acquired endocardial and myocardial disease characterized by a sharp decrease in the compliance of either or both ventricles with an acute diastolic dysfunction and massive mural thrombosis. This disease is presented in the classification of cardiomyopathies and is a variant of restrictive cardiomyopathy. Today Loeffler endocarditis is considered as a manifestation of hypereosinophilic syndrome with predominant heart involvement. The life-time diagnosis of myocardial injury due to eosinophilic infiltration is rare, or it is diagnosed at the stage of necrotizing endomyocarditis, when the treatment is no longer effective...
November 25, 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/27846662/-the-lymphoid-variant-of-hes-l-hes-as-differential-diagnose-of-severe-asthma-in-childhood
#14
T Leu, S Rauthe, C Wirth, H-U Simon, V Kunzmann, H Hebestreit, S Kunzmann
Based on a case report an overview on the differential diagnostic considerations with respect to blood hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in childhood is given. A 13-year-old boy was admitted for the clarification of an asthma. In the blood count an increased HE with 3 500/µl (30%) was found along with elevated total serum IgE and IL-5 level (2 000 IU/ml and 17 pg/ml). Lung function showed an obstruction (FEV1 38%). Radiologically the picture of bronchiectasis and mucus pluggine appeared...
November 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27811459/kimura-s-disease-a-case-presentation-of-postauricular-swelling
#15
A Rajesh, T Prasanth, V C Naga Sirisha, Mds Azmi
Kimura's disease (KD) is a rare chronic inflammatory disease of subcutaneous tissues and occurs predominantly in head and neck region. It is seen primarily in young Asian males. Typical clinical presentations are painless subcutaneous masses, regional lymph node enlargement, blood and tissue hypereosinophilia, and increased serum IgE levels. Here, we present a case of a 27-year-old female who presented with unilateral single nodular swelling in the right postauricular region. The diagnosis of KD was done based on characteristic histopathologic finding in conjunction with peripheral eosinophilia and increase in serum IgE levels...
November 2016: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/27805245/active-schistosomiasis-severe-hypereosinophilia-and-rapid-progression-of-chronic-endomyocardial-fibrosis
#16
A O Mocumbi, C Goncalves, A Damasceno, C Carrilho
Endomyocardial fibrosis (EMF) is a neglected restrictive cardiomyopathy of unknown aetiology and unclear natural history, which causes premature deaths in endemic areas. We present the case of a 13-year-old boy from a highly endemic area, presenting with concurrent signs of chronic EMF and severe hypereosinophilia associated with active schistosomal cystitis. We discuss the possible role of this parasitic infection in determining the progression of EMF in endemic areas for both conditions.
October 23, 2016: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/27728633/miliary-tuberculosis-with-hypereosinophilia
#17
Bharathkanth Reddy M
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27727077/comprehensive-evaluation-of-cardiac-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-egpa-with-cardiac-magnetic-resonance
#18
Alberto Francesco Cereda, Patrizia Pedrotti, Lucio De Capitani, Cristina Giannattasio, Alberto Roghi
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by hypereosinophilia. EGPA typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally systemic vasculitis. Cardiac involvement is the most important predictor of mortality; it occurs in approximately 15-60% of EGPA patients, a significant proportion of whom are asymptomatic and have normal electrocardiogram (ECG) and echocardiogram...
October 7, 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27721930/eosinophilic-ascites-a-diagnostic-and-therapeutic-challenge
#19
Shefali Agrawal, Sandeep Vohra, Sangeeta Rawat, Vikas Kashyap
Eosinophilic gastroenteritis (EGE) is a rare condition characterized by eosinophilic infiltration of the gastrointestinal tract. Depending on the dominant layer of infiltration it is classified into three types namely, mucosal, muscularis and subserosal. The most uncommon variant is the subserosal type characterized by primarily subserosal disease, eosinophilic ascites and peripheral hypereosinophilia. The clinical features are non-specific with history of atopic predisposition and allergy. Endoscopic biopsy is frequently non-diagnostic due to an uninvolved gastrointestinal mucosa rendering its diagnosis a challenge...
September 27, 2016: World Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/27709778/liver-fibrosis-with-hypereosinophilia-causing-transient-abnormal-myelopoiesis
#20
Shunsuke Minakata, Naoki Sakata, Norihisa Wada, Yuhei Konishi, Satoshi Marutani, Takuji Enya, Hidenori Nakagawa, Hiroshi Wada, Tsukasa Takemura
Transient abnormal myelopoesis is mostly self-resolving and has a good prognosis, but some patients subsequently die of liver fibrosis. We report the case of an infant with Down syndrome who developed life-threatening liver fibrosis at the same time as the blasts were about to disappear. This patient also had a marked increase in eosinophils, which were possibly harboring a GATA1 mutation and were expressing a high level of platelet-derived growth factor-B mRNA; these may have been involved in the development of liver fibrosis...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
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