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Increased Mast Cell Numbers in Bone Marrow of Patients with Myeloproliferative Neoplasms and Pruritus
Maud Hermans1, Peter Westerweel2, Thierry van den Bosch3
1Erasmus MC, University Medical Center Rotterdam, Department of Internal Medicine, Division of Allergy and Clinical Immunology, Rotterdam, the Netherlands
Abstract:
Pruritus may be a debilitating symptom in cases of myeloproliferative neoplasm (MPN). Since mast cells (MCs) play a pivotal role, we hypothesized that bone marrow (BM) MCs contribute to the occurrence of pruritus. In this exploratory study, untreated MPN patients were included. BM aspirate/biopsy/blood samples and responses to the MPN-Symptom Assessment Form were obtained. Thirty-two patients were included (essential thrombocythemia, n=19; polycythemia vera, n=7; primary myelofibrosis, n=6). Eleven had clinically significant pruritus (35%). The absolute MC count and proportion per total nucleated BM cells were significantly higher in patients with pruritus compared to those without (mean of 25.5 vs. 58.1 MCs/mm2, p=0.032 and 0.59% vs. 1.66%, p=0.041, respectively). MC immunophenotypes and serum levels of tryptase, interleukin-6, tumor necrosis factor alpha, and soluble CD117 were not correlated. Thus, this study showed that absolute and relative MC numbers are increased in cases of MPN with pruritus. However, whether MCs play a pathophysiological role in causing pruritus or are bystanders remains to be determined.
Insights
Myeloproliferative neoplasms (MPN) can cause debilitating pruritus. This study found higher bone marrow mast cell (MC) counts in MPN patients with pruritus, suggesting a potential link.
Area of Science:
- Hematology
- Oncology
- Dermatology
Background:
- Pruritus is a common and debilitating symptom in myeloproliferative neoplasms (MPN).
- Mast cells (MC) are implicated in pruritus, but their role in MPN-associated pruritus is not fully understood.
- Bone marrow (BM) mast cells are hypothesized to contribute to MPN pruritus.
Purpose of the Study:
- To investigate the association between bone marrow mast cell burden and pruritus in untreated MPN patients.
- To explore potential correlations between mast cell characteristics and pruritus severity.
Main Methods:
- An exploratory study included 32 untreated MPN patients (Essential Thrombocythemia, Polycythemia Vera, Primary Myelofibrosis).
- Bone marrow aspirate/biopsy/blood samples were analyzed for mast cell counts.
- MPN-SAF questionnaires assessed pruritus severity.
- Serum levels of tryptase, IL-6, TNF-α, and sCD117 were measured.
Main Results:
- Eleven patients (35%) reported clinically significant pruritus.
- Absolute mast cell counts (MC/mm²) and the proportion of MC among total nucleated bone marrow cells were significantly higher in patients with pruritus compared to those without (p=0.032 and p=0.041, respectively).
- No significant correlation was found between mast cell immunophenotype, serum tryptase, IL-6, TNF-α, or sCD117 levels and pruritus.
Conclusions:
- Increased absolute and relative mast cell numbers in the bone marrow are associated with pruritus in MPN patients.
- The exact pathophysiological role of mast cells in causing MPN-related pruritus requires further investigation.
- Mast cells may be involved in the development of pruritus in MPN, but their role as direct contributors or bystanders needs clarification.
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