Treatment of lymphocyte‐variant hypereosinophilic syndrome (L‐HES): what to consider after confirming the elusive diagnosis

Abstract

Lymphocyte-variant hypereosinophilic syndrome (L-HES) is a rare disease driven by immunophenotypically aberrant T cells producing eosinophilopoetic cytokines such as interleukin-5 (IL-5). Treatment is challenging because L-HES is relatively steroid resistant and not amenable to tyrosine kinase inhibitors. We searched the literature for clinical trials and observational studies, including case reports, of patients treated for L-HES. In all, 25 studies were selected; two were randomised controlled trials of IL-5 blockade, which included some patients with L-HES, and the rest were observational studies. Corticosteroids are often used as first-line therapy, but patients with L-HES have lower response rates than other types of HES. Treatments that reduce symptoms and steroid dependence in some patients include interferonalpha (IFN-a), anti-IL-5 monoclonal antibodies, cyclosporine and mycophenolate. These drugs target T-cell activation and proliferation, or IL-5 directly. Although effective, IFN-a and cyclosporine were commonly reported to cause side-effects resulting in discontinuation. Alemtuzumab can induce remissions, but these are generally short lived. The anti-IL-5 monoclonal antibodies mepolizumab and benralizumab are effective and well tolerated, but with a high rate of relapse once withdrawn. Hydroxyurea, methotrexate, imatinib were unsuccessful in most patients studied. More prospective clinical trials are needed for patients with L-HES.

Characteristics of patients with incidental eosinophilia admitted to a tertiary hospital in southern China

“…However, work-up for additional secondary causes of eosinophilia, such as L-HES, IgG4-RD and EGPA, received little attention from clinicians. A combination of bone marrow biopsy, cytogenetics, flow cytometry immunophenotypic analysis and T-cell clonality studies are necessary to determine the diagnosis of L‐HES [ 9 ]. When IgG4-related disease is suspected, serum protein electrophoresis (SPEP) and IgG subclasses are helpful as initial tests [ 35 ].…”

“…A variety of causes of eosinophilia have been widely recognized including asthma, dermatosis diseases, hematological malignancy, drug‐effect, parasitic infections, gastrointestinal disorders and vasculitides [ 6 , 7 ]. Recently, an increasing number of studies demonstrated that lymphocyte variant of HES subtype (L-HES), Immunoglobulin G4-related disease (IgG4-RD) and Eosinophilic granulomatosis with polyangiitis (EGPA) could be the common causes of secondary eosinophilia [ 8 , 9 ], but with inadequate evaluation. Patients with eosinophilia could exhibit with different symptoms involving multiple organs, which could be neglected by clinicians easily [ 10 ].…”

Hydrazineylidene‐3‐oxopropanal derivatives as antiviral agents for treatment of HBV and HCV: Experimental, DFT, and molecular docking studies

“…[1][2][3] The liver remains the target that can be damaged by hepatitis B and C. According to the World Health Organization (WHO, 2019), hepatitis affects around 325 million people globally, with viral hepatitis B and C causing approximately 1.4 million fatalities each year. [4,5] Hepatitis is the second leading cause of death in the world. People are 9 times more likely to get hepatitis than they are to contract HIV after tuberculosis.…”

Diagnostic spectrum of hypereosinophilia based on bone marrow pathology: 10 years' experience at a tertiary care hospital

“…Treatment of L‐HES is different from other types of HE, so it is important to distinguish. The standard treatment for L‐HES is steroids and/or pegylated interferon alpha 10,11 . Steroids, used primarily for HE, can be considered first‐line treatment for L‐HES, but are relatively steroid‐resistant and less responsive than other types of HES.…”

“…The standard treatment for L-HES is steroids and/or pegylated interferon alpha. 10,11 Steroids, used primarily for HE, can be considered first-line treatment for L-HES, but are relatively steroid-resistant and less responsive than other types of HES. Although there are further clinical trials are needed, there are many available treatments options for L-HES, including interferon-alpha (IFN-α), anti-IL-5 monoclonal antibodies, cyclosporine, and mycophenolic acid.…”