The hypereosinophilic syndromes: current concepts and treatments

Abstract

SummaryThe hypereosinophilic syndromes (HES) encompass a spectrum of diseases that have increased blood eosinophils and tissue damage in common. The clinical manifestations are protean and may involve any organ system, but especially the skin. Our understanding of these diseases has drastically changed over the past 15 years, along with new classifications that characterize patients with marked eosinophilia. One HES variant, myeloproliferative, is actually chronic eosinophilic leukaemia with a unique genetic marker, FIP1L1-PDGFRA. Such patients are well-controlled by administration of the kinase inhibitor, imatinib, and remissions appear durable with continued imatinib therapy. FIP1L1-PDGFRA is expressed in several cell lineages, thus explaining increases in neutrophils and mast cells in HES. The lymphocytic HES variant is associated with T-cell clones producing interleukin-5 (IL-5) and can evolve into lymphoma. While myeloproliferative and lymphocytic HES are well established and permit elimination of the term, idiopathic, to these varieties, most HES patients do not fall into these categories and are classified as complex (using the 2006 Workshop Report). A recent study showed that a monoclonal antibody to IL-5, mepolizumab, reduced glucocorticoid therapy in HES patients who did not possess the FIP1L1-PDGFRA mutation while controlling eosinophilia and preventing recurrence or progression of tissue damage. These advances augur well for continued progress in the understanding and treatment of HES.

Leucemia eosinofílica crónica con respuesta hematológica sostenida tras tratamiento con bajas dosis de imatinib

“…Las causas de eosinofilia son de diversa naturaleza y varían en sus manifestaciones clínicas, respuestas al tratamiento y pronóstico 4 . Las más comunes obedecen a desórdenes alérgicos, infecciones, enfermedades autoinmunes, tumores sólidos y neoplasias hematológicas (Tabla 1) 5,6 . En este último grupo deben considerarse en el diagnóstico diferencial aquellas que presentan la eosinofilia como una manifestación primaria (ej.…”

A Tale of Recurrent Strokes due to Hypereosinophilic Syndrome

“…Corticosteroids are considered the first line therapy [9]. Corticosteroids remain the first line treatment for most patients with HES, with the exception of PDGFRA-associated HES which is a type of HES where FIP1L1 indeed represent chronic eosinophilic leukemia, because these patients have a molecular genetic abnormality, peculiarly an FIP1L1-PDGFRA fusion gene.…”

Approach to Treatment of Patients with Hypereosinophilic Syndrome in the Emergency Department

“…En sık görülen semptomlar: halsizlik, ateş, öksürük, solunum sıkıntısı, kas ağrıları, anjioödem, döküntü ve retinal lezyonlardır. Hastalarda anemi ve trombositopeni laboratuar bulgularındandır [2]. Bu olgu sunumunda hastanemiz aciline solunum sıkıntısı ile gelen 28 yaşında hipereozinofilik sendromlu bir olguda tanı ve tedavi yaklaşımları tartışıldı.…”