We asked, and you answered! Here are the responses from this month’s “You Make the Call” question on treatment options for a patient with erythroderma and progressive eosinophilia.
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I would request a T-cell gene rearrangement study for this patient.
Hasan Al-Yaseen, MRCP, PhD
Dubai, United Arab Emirates
I would recommend mepolizumab.
Juan José Gil Fernández, MD, PhD
Madrid, Spain
After going through the case history and other investigative reports, this looks like a lymphocytic variant of hypereosinophilic syndrome (HES). Histopathology usually shows lichenoid dermatitis without epidermotropism. Inflammatory infiltrate in the dermis is principally composed of eosinophilic cells and lymphocytes. Serum immunoglobulin E could be high as it is TH2 phenotype, and peripheral blood immunophenotyping shows atypical T-cell lymphocyte proliferation in CD4(+) and CD3(-), and clonal TCR gene rearrangement favors lymphocytic HES.
I would treat this patient with prednisolone 1 mg/kg daily along with pegylated interferon alpha.
Abdulrahman Saifudeen, MBBS, MD, MRCP
Kerala, India
Though the patient is PDGFRA negative, I would suggest trying imatinib. We have reported on a similar case with a PDGFRA-negative lymphocytic variant of hypereosinophilic syndrome (L-HES) that went into a prolonged remission with imatinib (Am J Hematol, 2011).
Another option for this patient could be mepolizumab. In the randomized trial from 2008, mepolizumab showed considerable efficacy in patients with PDGFRA-negative L-HES, though the response was somewhat weaker than the one in patients without L-HES.
Regarding interferon (IFN) alpha, my concern is the potential exacerbation of the psoriatic exanthema, a well-recognized side effect of IFN alpha.
Ioannis Kotsianidis, MD, PhD
Alexandroupolis, Greece
I think we are dealing with the lymphoid variant of hypereosinophilia, which is most likely associated with the dermatologic presentation. We may need to use PCR for the T-cell receptor, or next-generation sequencing if it’s available. Serum IgM may add value in addition to IgE.
He started on steroids with failure treatment on cyclosporine (used in first line), so interferon is a good second-line treatment. Other treatment options are mepolizumab, reslizumab, and benralizumab.
Bassam Francis Matti, MD
Baghdad, Iraq
