A population‐based study of chronic eosinophilic <scp>leukemia‐not</scp> otherwise specified in the United States

Ruan, Gordon; Smith, Caleb J.; Day, Courtney N.; Harmsen, William S.; Zblewski, Darci; Alkhateeb, Hassan B.; Begna, Kebede H.; Al‐Kali, Aref; Litzow, Mark R.; Hogan, W. J.; Gangat, Naseema; Patnaik, Mrinal M.; Pardanani, Animesh; Tefferi, Ayalew; Go, Ronald S.; Shah, Mithun Vinod

doi:10.1002/ajh.25906

Cited by 9 publications

(8 citation statements)

References 6 publications

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“…63 While not yet formally added to the category of recurrent tyrosine kinase fusion genes associated with eosinophilia, this is being evaluated in the next iteration of consensus classification criteria. 37,64,65 1 Including rare conditions (e.g. familial, hyper IgE syndrome, Omenn syndrome, episodic angioedema with eosinophilia, eosinophilia-myalgia syndrome) 2 Alternative PDGFRA fusion genes can be inferred by cytogenetically visible translocations involving 4q12.…”

Section: Clinical Presentationmentioning

confidence: 99%

World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

2021

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Disease Overview: The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary or clonal) disorders with potential for end-organ damage.Diagnosis: Hypereosinophilia (HE) has generally been defined as a peripheral blood eosinophil count greater than 1.5 Â 10 9 /L. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on morphologic review of the blood and marrow, standard cytogenetics, fluorescence in situ hybridization, next generation sequencing gene assays, and flow immunophenotyping to detect histopathologic or clonal evidence for an acute or chronic hematolymphoid neoplasm.Risk Stratification: Disease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2016 World Health Organization endorses a semi-molecular classification scheme of disease subtypes. This includes the major category "myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1 or with PCM1-JAK2", and the myeloproliferative neoplasm subtype, "chronic eosinophilic leukemia, not otherwise specified" (CEL, NOS). Lymphocyte-variant HE is an aberrant T-cell clone-driven reactive eosinophila, and idiopathic hypereosinophilic syndrome (HES) is a diagnosis of exclusion. Risk-adapted Therapy:The goal of therapy is to mitigate eosinophil-mediated organ damage. For patients with milder forms of eosinophilia (eg, < 1.5 Â 10 9 /L) without symptoms or signs of organ involvement, a watch and wait approach with close follow-up may be undertaken. Identification of rearranged PDGFRA or PDGFRB is critical because of the exquisite responsiveness of these diseases to imatinib. Corticosteroids are first-line therapy for patients with lymphocyte-variant HE and HES.Hydroxyurea and interferon-α have demonstrated efficacy as initial treatment and in steroid-refractory cases of HES. Mepolizumab, an interleukin-5 (IL-5) antagonist monoclonal antibody, was recently approved by the US Food and Drug Administration for patients with idiopathic HES. The use of the IL-5 receptor antibody benralizumab, as well as other targeted therapies such as JAK2 and FGFR1 inhibitors, is under active investigation.

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Section: Clinical Presentationmentioning

confidence: 99%

World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

2021

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“…Cardiac damage evolves through three stages; 1) an acute necrotic stage, 2) an intermediate thrombotic stage (characterized by thrombus formation on the damaged endocardium), and 3) a fibrotic stage (characterized by heart failure with reduced ejection fraction with ≤40 percent), and heart failure with preserved ejection fraction (with left ventricle ejection fraction >50 percent; known as diastolic heart failure). Management of idiopathic HES requires several considerations such as the degree of eosinophilia,end-organ dysfunction, and emergency of clinical picture [1][2][3][4][5][6][7][8][9][10][11][12][13] .…”

Section: Discussionmentioning

confidence: 99%

“…HES is a clinical picture that needs both knowledge and experience to determine its etiology and treatment 1 . Although the incidence and prevalence of HES are not known exactly, it is an extremely rare (the age-adjusted incidence rate: ~0.4 cases per 1 000 000) hematologic disorders 1,4 . Its clinical manifestations and course may vary from indolent to rapidly fatal.…”

Section: Introductionmentioning

confidence: 99%

Heart Failure Due to Idiopathic Hypereosinophilic Syndrome in Pregnancy: A Case Report and Review of the Literature

Mert

Aydın

2022

MRAJ

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“…É uma malignidade hematológica rara com níveis elevados de eosinófilos onde é caracterizada pela presença do gene de fusão FIP1L1-PDGFRA, as características diagnósticas incluem proliferação sustentada de precursores eosinofílicos com eosinofilia marcada no sangue periférico e medulo óssea (Lee et al, 2020;Morsia et al, 2020;Ruan et al, 2020).…”

Section: Leucemia Eosinofilica Crônica (Lec)unclassified

“…Devido a sua raridade, não existe consenso no tratamento, com terapêutica implementada incluindo hidroxiureia, esteroides, imatinibe, interferon e outros. Apesar desses tratamentos, duas pequenas séries de casos relataram que o LEC carregava um prognóstico ruim com sobrevida média de menos de dois anos e altas taxas de transformação leucêmica (Ruan et al, 2020).…”

Section: Leucemia Eosinofilica Crônica (Lec)unclassified

Manifestações orais nas doenças mieloproliferativas: Uma revisão de literatura

Nascimento¹,

Alves²,

Silva³

et al. 2021

RSD

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Objetivo: Abordar as manifestações causadas pelas alterações provenientes das diversas doenças mieloproliferativa na cavidade oral e as implicações na vida do paciente, de maneira a fornecer embasamento teórico que ajude no diagnóstico precoce e terapia dessas patologias. Materiais e Métodos: trata-se de uma revisão de literatura onde foi realizada uma busca nos bancos de dados relacionados às áreas da Saúde como: SCIELO, PUBMED e LILACS usando corte temporal de 2016 a 2020, com os descritores "hematologia" ou "doenças hematológicas"; "afecção" ou "manifestações orais" e "leucemia" ou "alterações hematologicas". Discussão: O presente trabalho está relacionado as alterações orais causadas pelas doenças mieloproliferativas, onde poucos são os relatos destas alterações na literatura, uma vez que não se tinha o conhecimento do acometimento da cavidade oral com as doenças, em busca de aprimorar o diagnostico diferencial este trabalho trás as principais alterações encontradas na literatura sobre as alterações bucais. Conclusão: Com isso é evidente a necessidade de acompanhamento multiprofissional dos pacientes acometidos por estas enfermidades e espera-se que esta pesquisa juntamente com pesquisas futuras, possa esclarecer as alterações provocadas pelas doenças mieloproliferativas, principalmente suas manifestações na cavidade oral, para um diagnostico diferencial e precoce. Dessa forma, concluímos que há uma relação direta das alterações clínicas na cavidade oral em indivíduos com doença mieloproliferativa.

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A population‐based study of chronic eosinophilic leukemia‐not otherwise specified in the United States

Cited by 9 publications

References 6 publications

World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

World Health Organization‐defined eosinophilic disorders: 2022 update on diagnosis, risk stratification, and management

Heart Failure Due to Idiopathic Hypereosinophilic Syndrome in Pregnancy: A Case Report and Review of the Literature

Manifestações orais nas doenças mieloproliferativas: Uma revisão de literatura

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