Mast cells and fibrosis in myeloproliferative neoplasms.

Mast cells and fibrosis in myeloproliferative neoplasms. - Mast cells and fibrosis in MPN

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON48689

Enrollment

Registered

2019-01-28

Start date

2019-02-01

Completion date

Unknown

Last updated

2024-04-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

itching myeloproliferative neoplasm

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: - Age > 18 years - Suspected MPN according to WHO criteria (i.e. essential thrombocytosis, polycythemia vera, primary myelofibrosis, or post-ET or post-PV myelofibrosis) - Previously proven MPN who did not yet receive pharmacotherapy. - Patient is capable of giving informed consent

Exclusion criteria

Exclusion criteria: - Age

Design outcomes

Primary

Measure	Time frame
There is no single main endpoint in this study. Instead, several parameters of mast cell load and activity will be evaluated, either separately or combined. Furthermore, these parameters will be compared between patients with/without pruritus, and before and after ruxolitinib treatment. - Is there an increase of the number of mast cells in the bone marrow of MPN patients? Compare patients with/without pruritus, and compare with patients with multiple myeloma. - What is the phenotype and function of these mast cells? Markers of aberrance (CD2, CD25, CD30) * Marker of activation (CD63) * Morphology * Does this phenotype change during treatment ruxolitinib? - Is there a relationship between the presence of the JAK2 mutation and pruritus? - Do MPN patients have higher levels of mast cell mediators in their peripheral blood and if so, do these levels decrease during treatment with ruxolitinib? Do these levels correlate with symptom severity, and specifically with the severity of pruritus? - Which transcriptional changes can be observed in single cells of the hematopoietic niche (endothelial cells, mesenchymal stromal cells, osteoblast lineage cells) as a driver of bone marrow fibrosis in MPN patients?	—

Measure

Time frame

There is no single main endpoint in this study. Instead, several parameters of mast cell load and *activity will be evaluated, either separately or combined. Furthermore, these parameters will be compared between patients with/without pruritus, and before and after ruxolitinib treatment. - Is there an increase of the number of mast cells in the bone marrow of MPN patients? Compare patients with/without pruritus, and compare with patients with multiple myeloma. - What is the phenotype and function of these mast cells? * Markers of aberrance (CD2, CD25, CD30) * Marker of activation (CD63) * Morphology * Does this phenotype change during treatment ruxolitinib? - Is there a relationship between the presence of the JAK2 mutation and pruritus? - Do MPN patients have higher levels of mast cell mediators in their peripheral blood and if so, do these levels decrease during treatment with ruxolitinib? Do these levels correlate with symptom severity, and specifically with the severity of pruritus? - Which transcriptional changes can be observed in single cells of the hematopoietic niche (endothelial cells, mesenchymal stromal cells, osteoblast lineage cells) as a driver of bone marrow fibrosis in MPN patients?

—

Secondary

Measure	Time frame
The effect of ruxolitinib and other pharmacotherapy on the aforementioned parameters.	—

Countries

Netherlands

Outcome results