Omalizumab Therapy for Mast Cell-Mediator Symptoms in Patients with ISM, CM, MMAS, and MCAS

Lemal, Richard; Fouquet, Guillemette; Terriou, Louis; Vaes, M.; Livideanu, Cristina Bulai; Frenzel, Laurent; Barète, Stéphane; Canioni, Danielle; Lhermitte, Ludovic; Rossignol, Julien; Arock, Michel; Dubreuil, Patrice; Lortholary, Olivier; Hermine, Olivier

doi:10.1016/j.jaip.2019.03.039

Cited by 50 publications

(57 citation statements)

References 34 publications

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“…A recent prospective study of 56 patients with a range of mast cell diseases, including 11 patients with idiopathic mast cell disorder (confirmed KIT mutation-negative), has further described the benefit of Omalizumab therapy, notably for the relief of vasomotor symptoms and recurrent anaphylaxis as well as moderate improvements in neuropsychiatric symptoms with an associated favorable safety profile. 7 In this study, the dosing schedule was at least double the dose than compared to our patient, with a minimum of 300 mg per month up to 1200 mg per month. Further cases of Omalizumab treatment in primary/monoclonal MCAS have also been summarised recently by Jagdis and Vadas 8 and Bell and Jackson.…”

Section: Commentsmentioning

confidence: 69%

Successful treatment of idiopathic mast cell activation syndrome with low‐dose Omalizumab

2019

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Objectives Idiopathic mast cell disorders, a recently defined and recognised syndrome in clinical practice, are similar to the previously termed non‐clonal mast cell disorder. Patients with idiopathic mast cell activation syndrome (MCAS) suffer all the classical signs of mast cell activation but do not have evidence of mast cell clonality. Furthermore, treatment of these patients can be limited and burdensome in those with refractory symptoms. Methods Here, we describe treatment of a patient with idiopathic MCAS utilising a single monthly subcutaneous injection of omalizumab and review the current classification and therapeutic options for clonal and non‐clonal MCAS. Results Low‐dose omalizumab treatment has successfully led to a 5‐year, sustained clinical response, controlled debilitating symptoms of mast cell activation and allowed for reintroduction and long‐term maintenance of bee venom subcutaneous immunotherapy. Conclusion Low‐dose omalizumab of 150 mg monthly should be considered for maintenance management of patients with idiopathic MCAS for its cost and quality‐of‐life benefits.

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Section: Commentsmentioning

confidence: 69%

Successful treatment of idiopathic mast cell activation syndrome with low‐dose Omalizumab

2019

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“…We also contacted two authors for further information. One responded and provided the raw database of the CEREMAST (the French References Center of Mastocytosis) retrospective cohort study published by Lemal et al The efficacy of omalizumab on each symptom was extracted individually for all patients from the latter database. The methodological quality of studies was assessed independently by two reviewers (FJ and CBL) using the Newcastle‐Ottawa Scale for cohort studies and Cochrane risk‐of‐bias tool for randomized trials when available.…”

Section: Methodsmentioning

confidence: 99%

Omalizumab in the treatment of adult patients with mastocytosis: A systematic review

Jendoubi¹,

Gaudenzio

Gallini

et al. 2020

Clin Experimental Allergy

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Background Mastocytosis is associated with mast cell (MC) mediator‐related symptoms for which limited therapies are available. Objective Our aim was to assess the efficacy and safety of omalizumab in the treatment of MC mediator‐related symptoms in adult patients with mastocytosis. Results We identified one multi‐centre retrospective cohort study (39 patients), one retrospective cohort study (13 patients), 4 case series and 10 case reports. No published controlled randomized study was identified. We included 69 patients (13 patients with cutaneous mastocytosis and 56 with systemic mastocytosis). The mean age was 48 years. Omalizumab maintenance dose was 300 mg for the majority of patients. The mean duration of treatment was 17 months. Treatment led to a tolerability of venom immunotherapy and to a complete resolution of severe reactions in all patients with post‐honeybee sting anaphylaxis. Complete resolution of idiopathic anaphylaxis episodes was noted in 84% of the patients. Complete resolution of palpitations, gastrointestinal, cutaneous, neuropsychiatric, respiratory and musculoskeletal symptoms was observed at a rate of 43%, 29%, 27%, 11%, 9% and 0%, respectively. Efficacy was maintained for the entire duration of the treatment in all but four responders. Adverse events were reported for 13 patients. Conclusions and Clinical Relevance Omalizumab appears to prevent some life‐threatening reactions associated with mastocytosis and may be a good option to treat the associated symptoms. However, the evidence relied upon is observational, uncontrolled and from a small number of patients. A randomized controlled trial is needed to better understand the place of omalizumab in mastocytosis treatment.

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“…2 Omalizumab is a safe option in mastocytosis with an overall response rate of 78.2%. 3 However, despite maximal treatment with antihistamines, montelukast, corticosteroids and omalizumab, his symptoms continued to progress. Conclusion: This case represents an uncommon subset of patients with systemic mastocytosis presenting with recurrent, refractory anaphylaxis.…”

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confidence: 99%

Systemic mastocytosis presenting as recurrent, refractory anaphylaxis

2020

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Omalizumab Therapy for Mast Cell-Mediator Symptoms in Patients with ISM, CM, MMAS, and MCAS

Cited by 50 publications

References 34 publications

Successful treatment of idiopathic mast cell activation syndrome with low‐dose Omalizumab

Successful treatment of idiopathic mast cell activation syndrome with low‐dose Omalizumab

Omalizumab in the treatment of adult patients with mastocytosis: A systematic review

Systemic mastocytosis presenting as recurrent, refractory anaphylaxis

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