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Hypereosinophilic Syndrome (hypereosinophilic + syndrome)

Distribution by Scientific Domains

Medical Sciences	100%

Distribution within Medical Sciences

Hematology	26%
Dermatology	23%

Kinds of Hypereosinophilic Syndrome

idiopathic hypereosinophilic syndrome

Selected Abstracts

Evaluation of Cardiac Involvement in Hypereosinophilic Syndrome: Complementary Roles of Transthoracic, Transesophageal, and Contrast Echocardiography

ECHOCARDIOGRAPHY, Issue 8 2006
Rajesh Shah M.D.
Hypereosinophilic syndrome is a rare but important systemic disease with multiple clinical presentations. Approximately 40% of these cases have cardiac involvement. Echocardiography is the most easily available and versatile imaging modality in assessing cardiac involvement in this disease process. As described and reviewed in this case, it may be the first imaging modality to raise suspicion of this disease entity. Hence, clinicians interpreting echocardiograms and caring for patients need to be aware of the manifestations and complementary roles of various echo techniques in delineating cardiac involvement. Furthermore, the importance of a thorough history and laboratory review prior to echocardiography may provide valuable clues which may otherwise be missed. [source]

Hypereosinophilic Syndrome Presenting with Biventricular Cardiac Thrombi

ECHOCARDIOGRAPHY, Issue 6 2010
Ankur Lodha M.D.
Hypereosinophilic syndrome is a rare condition characterized by idiopathic eosinophilia with organ system involvement. Cardiac involvement portends a less favorable prognosis as it can be complicated by development of heart failure, valvular dysfunction, and restrictive cardiomyopathy. We present a rare case of hypereosinophilic syndrome with FIP1L1/PDGFRA fusion in a 50-year-old male associated with thrombus in left and right ventricle. (Echocardiography 2010;27:E57-E59) [source]

Evaluation of Cardiac Involvement in Hypereosinophilic Syndrome: Complementary Roles of Transthoracic, Transesophageal, and Contrast Echocardiography

Loeffler endocarditis: What have we learned?

AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2007
Juan Benezet-Mazuecos
Loeffler endocarditis, eosinophilic endomyocardial disease or fibroplastic endocarditis appears to be a subcategory of the Hypereosinophilic syndrome in which the heart is predominantly involved. It is an uncommon myocardial disease, thought to be secondary to eosinophils damage, characterized by fibrous thickening of the endocardium of one or both ventricles, leading to apical obliteration and multiple cardiovascular complications. Despite all the efforts, the ultimate responsible mechanisms of this entity remain unresolved. Many theories have been raised trying to explain this phenomenon, but nowadays the enigma in relation to the different patterns of evolution continues. In this concise review we discuss the different pathophysiologic theories postulated and the management of the cardiovascular complications. Perhaps it will serve to assist in recognition of patients with the same condition around the world. Am. J. Hematol. 82:861,862, 2007. © 2007 Wiley-Liss, Inc. [source]

Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2009
K. Ito
Summary Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES. [source]

Hypereosinophilic Syndrome Presenting with Biventricular Cardiac Thrombi

Peripheral T-cell lymphoma associated consecutively with hemophagocytic lymphohistiocytosis and hypereosinophilic syndrome

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2003
Antonio Gutiérrez
Abstract:, Both hemophagocytic lymphohistiocytosis and hypereosinophilic syndrome have been associated with hematologic neoplasms and are respectively related to an overproduction of the cytokines Thelper 1 (Th1) and Th2 by tumor cells or reactive cells. To our knowledge, this is the first time a case of a peripheral T-cell lymphoma consecutively associated with both paraneoplastic conditions has been reported. Importantly, in this case when the lymphoma exclusively involved the bone marrow, severe paraneoplastic systemic damage, a CD8+ suppressor/cytotoxic phenotype and a hypereosinophilia not related to high levels of interleukin (IL)-5 was found. Interestingly, progression of the lymphoma coincided with an increase in the serum levels of several Th2 cytokines and IL-2, a decrease in tumor necrosis factor and granulocyte-macrophage colony-stimulating factor levels and the onset of a hypereosinophilic syndrome. [source]

Mucocutaneous Splendore-Hoeppli phenomenon

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 11 2008
Mahmoud R. Hussein
Splendore-Hoeppli phenomenon (asteroid bodies) is the in vivo formation of intensely eosinophilic material (radiate, star-like, asteroid or club-shaped configurations) around microorganisms (fungi, bacteria and parasites) or biologically inert substances. This study presents a literature review concerning Splendore-Hoeppli reaction in the mucocutaneous diseases. It examines the histopathological features, nature and differential diagnosis of this reaction. It also discusses the mucocutaneous infections and the non-infective diseases associated with it. Available studies indicate that several mucocutaneous infections can generate Splendore-Hoeppli reaction. The fungal infections include sporotrichosis, pityrosporum folliculitis, zygomycosis, candidiasis, aspergillosis and blastomycosis. The bacterial infections include botryomycosis, nocardiosis and actinomycosis. The parasitic conditions include orbital pythiosis, strongyloidiasis, schistosomiasis and cutaneous larva migrans. In addition, Splendore-Hoeppli reaction may be seen with non-infective pathology such as hypereosinophilic syndrome and allergic conjunctival granulomas. The Splendore-Hoeppli reaction material comprises antigen-antibody complex, tissue debris and fibrin. Although the exact nature of this reaction is unknown, it is thought to be a localized immunological response to an antigen-antibody precipitate related to fungi, parasites, bacteria or inert materials. The characteristic formation of the peribacterial or perifungal Splendore-Hoeppli reaction probably prevents phagocytosis and intracellular killing of the insulting agent leading to chronicity of infection. To conclude, Splendore-Hoeppli reaction is a tell tale of a spectrum of infections and reactive conditions. The molecular pathways involved in the development of this reaction are open for future investigations. [source]

Development of Löffler's endocarditis in FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome despite continuous imatinib mesylate therapy and continuous complete remission,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2010
Jan Václavík
First page of article [source]

A rat model of hypereosinophilic syndrome

PATHOLOGY INTERNATIONAL, Issue 2 2001
Kenji Sano
Hypereosinophilia-occurring rats without chemical and antigen treatment have been maintained in our laboratory. The rat, Matsumoto Eosinophilia Shinshu (mes), showed hypereosinophilia at the age of 9 weeks or older and developed eosinophil-related inflammatory lesions in many organs. These lesions included: aortitis, granulomatous lesion in the mesenteric lymph node, inflammatory fibroid polyp of the stomach and pulmonary vasculitis with septal infiltration. These lesions were involved with cellular infiltration of eosinophils and macrophages, and deposition of eosinophilic crystals which immunohistologically showed major basic protein and eosinophilic peroxidase derived from eosinophilic lysosomal constituents. Although the distribution of lesions in mes is a little different from that of hypereosinophilic syndrome (HES) in humans, in that endomyocardial fibrosis appears in HES while aortitis appears in mes, mes is probably comparable with HES. The present paper describes the pathological aspects of the lesions in mes and discusses the pathogenesis of tissue injury related to eosinophilic infiltration. [source]

Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis

PEDIATRIC TRANSPLANTATION, Issue 5 2010
Yohei Yamada
Yamada Y, Hoshino K, Shimojima N, Shinoda M, Obara H, Kawachi S, Fuchimoto Y, Tanabe M, Kitagawa Y, Morikawa Y. Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis. Pediatr Transplantation 2010: 14:e49,e53. © 2009 John Wiley & Sons A/S. Abstract:, Idiopathic HES is characterlized by prolonged eosinophilia without an identifiable underlying cause and multiple-organ dysfunction. We report a case of a LDLT for a 12-yr-old Japanese girl with BA accompanied by HES. Histological examination of the resected liver showed biliary cirrhosis with dense eosinophilic infiltration of portal tracts and the lobules of the liver. She developed portal vein thrombosis on post-operative day 10 and the histopathological findings of the thrombus revealed dense eosinophilic deposition, suggesting that HES might have influenced the formation of this thrombus. Liver graft biopsies also demonstrated the presence of activated eosinophilils with biliary damage. Blood chemistry findings suggested liver dysfunction as a result of the eosinophilic infiltrations. Prednisolone treatment improved the liver dysfunction. Four years after LDLT, she remains clinically well on prednisolone at 0.3 mg/kg/day, with an eosinophil count ranging from 10 to 15%. A literature review has not shown any previous reports of HES with BA. This case demonstrates the possibility of an association between eosinophilic infiltration and liver dysfunction during follow-up for BA and after LDLT. [source]

Selective priming of peripheral blood eosinophils in patients with idiopathic hypereosinophilic syndrome,

APMIS, Issue 11 2006
MARIA LAMPINEN
The idiopathic hypereosinophilic syndrome (HES) is characterised by blood eosinophilia associated with organ involvement. Elevated numbers of blood neutrophils have been observed during episodes of active HES. However, an increased responsiveness of eosinophils to chemotactic and chemokinetic stimuli may explain the selective eosinophil infiltration of the tissue. We have studied the migratory responses of blood eosinophils and neutrophils from 9 patients with HES and from 13 healthy control subjects. Chemokinetic and chemotactic responses to factors acting on both cell types were analysed by means of a modification of the Boyden chamber technique. We found increased migratory responses of the eosinophils, but not of the neutrophils, from the patients with HES. Increased blood neutrophil counts in three of the patients did not coincide with alterations of the neutrophil migratory responses. Our finding of increased migratory responses of eosinophils from patients with HES towards non-specific chemoattractants suggests selective priming of eosinophils in this disease. Interleukin (IL)-5 has previously been shown to prime eosinophils for migratory responses, and successful anti-IL-5 therapy of patients with HES indicates an important role for this cytokine in the development of hypereosinophilia. [source]

Isolated symptomatic cutaneous disease in hypereosinophilic syndrome

AUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2010
Veronica Preda
ABSTRACT A 41-year-old Phillipino man presented with a 3-year history of a relapsing and remitting generalized chronic pruritic erythematous papular and plaque-like eruption. Investigations showed a persistently elevated eosinophil count. His disease was limited to cutaneous involvement with an absence of demonstrable internal organ involvement, despite extensive investigations and multidisciplinary review. Other causes of eosinophilia were excluded. A diagnosis of idiopathic hypereosinophilic syndrome was made. Our patient's presentation raises a number of issues related to hypereosinophilic syndrome. In particular, relating to managing hypereosinophilic syndrome and the challenge of minimizing therapy side-effects. Our case highlights the considerable morbidity of untreated isolated cutaneous disease, for which he was hospitalized with suicidal ideations. In a minority of reports, skin involvement is the only manifestation of hypereosinophilic syndrome. [source]

In this issue , August 2009

AUSTRALIAN VETERINARY JOURNAL, Issue 8 2009
A Jackson Editor in Chief
Post-weaning mortality of Merino sheep , Transcutaneous ultrasound over the right flank for pregnancy diagnosis in cows , Right flank transcutaneous vs transrectal ultrasound for pregnancy diagnosis in cows , Ultrasound of soft tissue tumours in dogs , Idiopathic hypereosinophilic syndrome in a Rottweiler , Toxicity from accidental oral dosing of a topical endectocide , Scrub-itch mite in an endangered wallaby , Cerebellar cortical degeneration in a koala [source]

Clinical remission of idiopathic hypereosinophilic syndrome in a Rottweiler

AUSTRALIAN VETERINARY JOURNAL, Issue 8 2009
FE James
Idiopathic hypereosinophilic syndrome (HES) is a rare syndrome for which Rottweilers appear to over-represent the canine cases. A 6-month-old female entire Rottweiler presented with seizures following a traumatic incident. The dog was identified as having a marked, sustained eosinophilia and investigations did not identify an underlying cause. Concurrently, the dog had chronic eosinophilic hepatitis with impaired liver function and mesenteric eosinophilic lymphadenitis. The dog went on to have spontaneous resolution of HES and normal liver function was subsequently documented. To date, the dog is still alive, more than 4 years after initial presentation. The diagnosis of idiopathic HES in Rottweilers may not carry a poor prognosis and the condition may spontaneously resolve, even in cases with documented organ damage. [source]

Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2000
K-A. Jang
Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES. [source]

Alemtuzumab in the reversal of encephalopathy associated with hypereosinophilic syndrome

BRITISH JOURNAL OF HAEMATOLOGY, Issue 5 2009
Guilherme Fleury Perini
No abstract is available for this article. [source]

Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome with abnormal T cells: a case report

BRITISH JOURNAL OF HAEMATOLOGY, Issue 5 2004
Vincenzo Pitini
No abstract is available for this article. [source]

Triptolide abrogates oncogene FIP1L1-PDGFR, addiction and induces apoptosis in hypereosinophilic syndrome

CANCER SCIENCE, Issue 11 2009
Yanli Jin
The pathogenesis of hypereosinophilic syndrome (HES) in some patients is highly dependent on FIP1-Like-1 (FIP1L1),platelet-derived growth factor receptor alpha (PDGFR,), which can generate sustained activation signaling to maintain a cell malignant phenotype. HES usually shows good response to the tyrosine kinase inhibitor imatinib, but mutations in FIP1L1-PDGFR, (e.g. T674I) can confer acquired resistance to imatinib. An alternative therapeutic strategy other than with tyrosine kinase inhibitors is needed to overcome acquired drug resistance. We hypothesized that switching off the crucial chimeric oncoprotein FIP1L1-PDGFR, on which HES cells depend, should have deleterious effects on the cancer cells. We used low concentrations of triptolide, a transcription inhibitor, to shut down the expression of FIP1L1-PDGFR,. EOL-1 cells and BaF3 cells expressing wild-type or T674I FIP1L1-PDGFR, were treated with triptolide, and signaling pathways, cell cycling, and apoptosis were analyzed by RT-PCR, immunoblotting, and flow cytometry, respectively. The results revealed that at nanomolar concentrations triptolide decreased the levels of mRNA and protein of FIP1L1-PDGFR, and the growth of the neoplastic cells, regardless of the mutational status of PDGFR,. Triptolide also downregulated the signaling molecules Stat3, Akt, and Erk1/2, which are downstream from PDGFR,, and induced G1 cell-cycle arrest. Triptolide time- and dose-dependently induced apoptosis by decreasing the anti-apoptotic proteins Mcl-1 and Bcl-XL,triggering the intrinsic apoptotic pathway. In conclusion, triptolide has potent activity against malignant cells in HES bearing FIP1L1-PDGFR,, regardless of its mutational status that confer acquired resistance to imatinib. Our results suggest that triptolide may be a promising agent in the treatment of HES. (Cancer Sci 2009; 00: 000,000) [source]

Treatment responsive dementia in advanced idiopathic hypereosinophilic syndrome

ACTA NEUROPSYCHIATRICA, Issue 2 2009
Witold So
No abstract is available for this article. [source]

A short low-dose imatinib trial allows rapid identification of responsive patients in hypereosinophilic syndromes

BRITISH JOURNAL OF HAEMATOLOGY, Issue 5 2009
Tamara Intermesoli
Summary Although imatinib may be effective in hypereosinophilic syndromes, the exact response kinetics are not known. Imatinib was administered at 100,400 mg/d each week in a 12-week response-oriented schedule, targeting a complete clinical and haematological remission (CR). CR was achieved in 11/23 patients (6/6 with FIP1L1 - PDGRFA rearrangement and 5/17 without, P = 0·006), most after 2 weeks of 100 mg/d imatinib. The maximum imatinib dose had no effect in early unresponsive patients. Low-dose, short-course imatinib may represent a rational choice for identifying responsive cases, both within and outside the pre-defined FIP1L1 rearrangement subset. [source]