Abstract
Gleich’s syndrome (GS) is characterized by recurrent episodes of angioedema, increase in body weight, fever, hypereosinophilia, and elevated serum IgM. The exact etiology remains unclear. Currently, the only treatment strategy is the administration of high dose of steroids during the acute phases. We report the case of a 37-year-old man suffering from GS with recurrent episodes of angioedema, fever, hypereosinophilia [6,000/mm3 (45%)], and high eosinophil cationic protein (ECP) (>200 μg/l), treated with oral steroids during the acute phase (prednisone 50–75 mg/day), the dose of maintenance being 25 mg/day. No monoclonal components were identified, and genetic tests exclude mutations including Bcr/Abl, JAK2 V617F, c-KIT D816V, and FIP1L1-PDGFRA. Using Luminex technology, we observed higher serum levels of interleukin (IL)-5, CCL2, and CCL11 during the acute exacerbations in comparison with the clinical remission phases though CCL11 did not achieve statistical significance. The flow-cytometric analysis identified a CD3+ CD8− lymphocyte population with high frequency of IL-4-, IL-5-, and IL-13-producing cells. No clinical benefit was observed after therapeutic strategies with imatinib, interferon-α, cyclosporine-A, and azathioprine. Due to high IL-5 serum levels, an intravenous treatment with anti-IL-5 monoclonal antibody mepolizumab (750 mg every 4 weeks) was started. A reduction in the rate of exacerbation phases/year (10 ± 3 vs 2 ± 1; p < 0.005), in the eosinophils count both in percentage (28.8 ± 12.8 vs 9.8 ± 3.9; p < 0.001) and absolute value (2,737 ± 1,946 vs 782 ± 333; p < 0.001) were observed as well as the ECP serum levels (132.7 ± 62.7 vs 21 ± 14.2 μg/l; p < 0.05). The daily dose of prednisone was significantly reduced (25 vs 7.5 mg). Any adverse effects were recorded. To the best of our knowledge, this case is the first report of the disease successfully treated with mepolizumab, and it could represent a novel therapeutic strategy in GS.
Highlights
We report the case of a 37-year-old man with a history starting at age 15 of recurrent episodes of angioedema of the head, trunk, and limbs, associated with myalgias, fever, hypereosinophilia [during an acute phase eosinophils had risen to 6,000/mm3 corresponding to 45% of circulating white cells and the eosinophil cationic protein (ECP) had risen to >200 μg/l], and characterized by oliguriaAnti-IL5 in Gleich’s syndrome (GS) and weight gain (5–7 kg)
The demonstration of high serum levels of IL-5 in our patient during the exacerbation phases of the disease is in agreement with the therapeutic choice of using an anti-IL-5 antibody
A clonal expansion of CD4+ T cells has been shown in a proportion of patients suffering from GS [8], the production of high amounts of IL-5 can be sustained by a non-clonal T cell population, as in our case of GS
Summary
We report the case of a 37-year-old man with a history starting at age 15 of recurrent episodes of angioedema of the head, trunk, and limbs, associated with myalgias, fever, hypereosinophilia [during an acute phase eosinophils had risen to 6,000/mm corresponding to 45% of circulating white cells and the eosinophil cationic protein (ECP) had risen to >200 μg/l], and characterized by oliguria. The high serum levels of IL-5 shown in the patient and the role of this cytokine in eosinophil differentiation and survival, after obtaining written informed consent from the patient and the local Ethical Committee approval, in April 2011 we started intravenous treatment with anti-IL-5 monoclonal antibody mepolizumab (kindly supplied from GlaxoSmithKline) 750 mg every 4 weeks. Besides a reduction of the frequency of clinical attacks, the treatment with mepolizumab allowed a significant decrease of the severity of each exacerbation in terms of mean weight gain (5.3 ± 1 vs 1.3 ± 0.4, p < 0.0001) and in the daily maintenance dose of prednisone (25 vs 7.5 mg). A statistically significant reduction of ECP serum levels (132.7 ± 62.7 vs 21 ± 14.2 μg/l; p < 0.05) measured in samples collected at exacerbations before mepolizumab treatment and during the patients’ visits at follow-up after the beginning of therapy. The patient has provided the written informed consent for the publication
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