A multimodality work‐up of patients with Hypereosinophilia

Abstract

The work-up of patients with hypereosinophilia (HE) is complex. Following the recently revised World Health Organization criteria, we retrospectively reviewed 125 patients who were referred to us to exclude a neoplastic cause of HE (2003-2016). The clinical laboratory work-up confirmed secondary HE in 25 (20%) patients; myeloid/lymphoid neoplasms with rearrangements of PDGFRA (n = 9) or PDGFRB (n = 2) (9%); HE associated with a well-defined myeloid neoplasm in 8 (6%); and abnormal bone marrow and/or molecular genetic abnormalities consistent with chronic eosinophilic leukemia (CEL), not otherwise specified (NOS) in 21 (17%) patients. For the remaining 60 (48%) patients, a specific diagnosis was not identified, and 56 patients had HE related findings consistent with idiopathic hypereosinophilic syndrome (HES), while 4 patients who were asymptomatic. With a median follow up of 35.3 months (range, <1-104), patients with CEL, not otherwise specified (NOS) had a median OS of 26.1 months, significantly inferior to patients with idiopathic HES (not reached, P < .01). Thus, our experience in a single tertiary cancer center shows that the work-up of HE following WHO recommendations requires a multimodality-based approach; and a correct diagnosis determines risk stratification and proper patient management. However, the causes of HE remain unknown in approximately half of referred patients, indicating the need for further studies.

IgG4-related disease: what a hematologist needs to know

“…12 Idiopathic HES and hypereosinophilia of unknown significance are diagnoses of exclusion, and account for a substantial proportion (30-50%) of diagnoses of patients evaluated for eosinophilia. 24–26 Evaluating these patients for IgG4-RD is an important and underappreciated aspect of their care. In fact, we previously published a case report with a diagnostic label of idiopathic HES, reviewed by several world experts in eosinophilia who concurred with the diagnosis, which was subsequently found to be IgG4-RD.…”

Lymphocytic variant of hypereosinophilic syndrome: A report of seven cases from a single institution

“…Reactive proliferations of eosinophils are secondary to infection, drugs, allergy, inflammatory conditions, solid tumors or lymphoproliferative neoplasms. Clonal eosinophilic proliferations are caused by various hematopoietic stem neoplasms, such as eosinophilia associated with PDGFRA, PDGFRB , FGFR1 , and PCM1‐JAK2 rearrangement, or chronic eosinophilic leukemia, not other specified (CEL‐NOS) (Hu et al, 2018; Wang, 2019). The workup of patients with eosinophilia is highly complex, which often requires a multidisciplinary approach including clinical correlation, morphological assessment in combination with immunophenotyping, cytogenetic analysis, and molecular studies.…”

“…(Wang, 2019; Wang et al, 2017). After an exhausting workup, a clear cause of HES remains unknown in a significant proportion of patients (Hu et al, 2018), and such cases would be considered to be idiopathic.…”

A Rare Translocation of t (1, 5) (q21, q32) in a Case of Myeloid Neoplasm With Eosinophilia

“…In a study of 556 patients with myeloproliferative neoplasms, the incidence of PDGFRB rearrangement was 1.8% [13]. Also, in a single center study, the incidence of PDGFRB rearrangement in hyper eosinophilia patients was 9% (2/25) [14]. As been reported, the response rate of MPN with eosinophilia and PDGFRB rearrangement was more than 95% [3].…”