IgG4‐related disease and lymphocyte‐variant hypereosinophilic syndrome: A comparative case series

Carruthers, Mollie; Park, Su‐Jin; Slack, Graham W.; Dalal, Bakul I.; Skinnider, Brian; Schaeffer, David F.; Dutz, Jan P.; Law, Joanna K.; Donnellan, Fergal; Marquez, Vladimir; Seidman, Michael A.; Wong, P.C.; Mattman, André; Chen, Luke Y C

doi:10.1111/ejh.12842

Cited by 39 publications

(28 citation statements)

References 30 publications

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“…Whereas the most common aberrant immunophenotype is CD3−CD4+, various aberrant immunophenotypes have been described ( 64 ), and some patients have cytokine-secreting clonal T-cell populations despite an apparently normal immunophenotype. Dermatologic manifestations, including angioedema, nodules, eczematous dermatitis, and erythroderma, are common in patients with LHES ( 65 ), and aberrant T cells can often be detected in skin biopsies from affected areas ( 66 ). Patients with LHES are often glucocorticoid responsive but typically require moderately high doses ( 67 ).…”

Section: Clinical Subtype-specific Biomarkers In Hesmentioning

confidence: 99%

Clinical and Biological Markers in Hypereosinophilic Syndromes

2017

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Hypereosinophilic syndromes (HES) are rare, heterogeneous syndromes characterized by markedly elevated eosinophil counts in the blood and/or tissue and evidence of eosinophil-associated pathology. Although parasitic infections, drug hypersensitivity, and other disorders of defined etiology can present as HES (associated HES), treatment is directed at the underlying cause rather than the eosinophilia itself. A number of additional subtypes of HES have been described, based on clinical and laboratory features. These include (1) myeloid HES—a primary disorder of the myeloid lineage, (2) lymphocytic variant HES—eosinophilia driven by aberrant or clonal lymphocytes secreting eosinophil-promoting cytokines, (3) overlap HES—eosinophilia restricted to a single organ or organ system, (4) familial eosinophilia—a rare inherited form of HES, and (5) idiopathic HES. Since clinical manifestations, response to therapy, and prognosis all differ between HES subtypes, this review will focus on clinical and biological markers that serve as markers of disease activity in HES (excluding associated HES), including those that are likely to be useful only in specific clinical subtypes.

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Section: Clinical Subtype-specific Biomarkers In Hesmentioning

confidence: 99%

Clinical and Biological Markers in Hypereosinophilic Syndromes

2017

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“…Increased IgG4+ plasma cells may be seen in a number of “mimickers” of IgG4‐RD including Rosai‐Dorfman disease, multicentric Castleman's disease, and vasculitis. Increased serum IgG4, particularly mild increases in the 1.5–5 g/L range, may be seen in infection, autoimmune disease, hematologic neoplasms such as hypereosinophilic syndromes, and other conditions . In this case, extensive review of the clinical, radiologic, and pathologic features did not confirm a diagnosis of IgG4‐RD, and the IgG4 plasma cell infiltrate appears to be reactive in nature.…”

Section: Discussionmentioning

confidence: 71%

A young woman with steroid‐responsive, IgG4‐positive plasma cell‐enriched cystic lymphangioma and chylous ascites

Zaki

Mann

Carruthers

et al. 2018

Clinical Case Reports

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“…It is also likely that some of the idiopathic HES cases may be associated with an ill‐defined autoimmune disease or allergy, but an autoantibody or characteristic features of a specific entity could not be revealed. HE has been reported in patients with IgG4‐related disease, which is a relatively recently recognized entity that was not vigorously evaluated in our patients. Finally, a clonal process that is not currently recognized as a myeloid neoplasm could have accounted for some of the cases being labeled as “idiopathic”.…”

Section: Discussionmentioning

confidence: 92%

A multimodality work‐up of patients with Hypereosinophilia

Boddu

Loghavi

et al. 2018

American J Hematol

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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.

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IgG4‐related disease and lymphocyte‐variant hypereosinophilic syndrome: A comparative case series

Cited by 39 publications

References 30 publications

Clinical and Biological Markers in Hypereosinophilic Syndromes

Clinical and Biological Markers in Hypereosinophilic Syndromes

A young woman with steroid‐responsive, IgG4‐positive plasma cell‐enriched cystic lymphangioma and chylous ascites

A multimodality work‐up of patients with Hypereosinophilia

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