Lymphocyte-variant hypereosinophilic syndrome presenting as chronic dermatitis and responding to mycophenolic acid

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

“…Another group has recently reported prolonged steroid-free remission in response to peg-IFN-α in 4/5 patients with CD3 − CD4 + T cell associated L-HES ranging from 10 to 95 months, with significant reductions in abnormal T cell counts (32). However, the fifth patient described in more detail as a case report (33) experienced disease recurrence while on therapy within months, as observed in two patients in our series with recombinant IFN-α.…”

“…Among these agents, cyclosporin is the only drug reported by others to have some efficacy in L-HES (3/5 patients in the French series) ( 5 ). A recent single case report has shown a response to mycophenolic acid in a patient who had not responded to mycophenolate mofetil or peg-IFN-α ( 33 ). Alemtuzumab, a monoclonal anti-CD52 antibody able to target eosinophils in addition to the majority of leukocytes including lymphocytes, has been used anecdotally to treat CD3 − CD4 + HES ( 21 , 36 ) with an observed decrease in the CD3 − CD4 + T cell count in one case.…”

“…Among these agents, cyclosporin is the only drug reported by others to have some efficacy in L-HES (3/5 patients in the French series) (5). A recent single case report has shown a response to mycophenolic acid in a patient who had not responded to mycophenolate mofetil or peg-IFN-α (33). Alemtuzumab, a monoclonal anti-CD52 antibody able to target BOX 1 | Lessons learned on CD3-CD4+ T cell associated L-HES from this study.…”

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

“…A 2018 case report demonstrated complete and sustained clinical remission in a patient with L-HES and cutaneous T-cell lymphoma on MMF 500 mg twice daily, while a 2019 case report details successful clinical remission and peripheral eosinophil normalisation in a patient treated with mycophenolic acid 720 mg twice daily. 14,35 This patient had initially responded well to MMF, but was switched to mycophenolic acid due to better gastrointestinal tolerability. Lef evre et al 3 also reported one patient in their series who received and responded to mycophenolate.…”

Pegylated interferon alpha 2a is an effective and well‐tolerated treatment option for lymphocyte‐variant hypereosinophilic syndrome

“…Flow cytometry was performed on peripheral blood to identify the abnormal T‐cells including the three most commonly recognized L‐HES subtypes (normal range as % of total lymphocytes): CD3 − 4 + (≤2%), CD3 + CD4 + CD7 − (≤10%) and CD3 + CD4 − CD8 − (≤15%). Two of the subjects have been previously published as individual case reports (L3 and L8; Cheung et al , ; Dou et al , ); our flow cytometry and T‐cell clonality methods have been described in a previous publication (Carruthers et al , ). Response to treatment was defined as control of symptoms and eosinophils <1·0 × 10 9 /l (Khoury et al , ).…”