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Patients who carried primary or secondary myelofibrosis from Philadelphia negative MPNs (PMF/SMF) and who are treated or are about to be treated with pegylated-interferon (mostly α2a) are eligible to this prospective study. Biological and clinical parameters will be collected from the beginning of the drug use until last news.
A non-opposition consent form need to be signed before entering this study.
In all centres interested, patients who were diagnosed for PMF/SMF can be treated with oral or sub-cutaneous drugs in order to reduce clinical symptoms and biological abnormalities belonging to these diseases.
Pegylated-interferon is one of the better weapons that can be used in these cases. These treatments are well known and used from many years in french hematological centres.
The investigators to collect clinical and biological data from patients treated with pegylated-interferon α2a (the most use) in case of PMF/SMF in order to assess efficacity and tolerance to this drug. The prescription of the product and the dose are under the responsibility of each practician.
Clinical data collected: disease and patients characteristics at the time of diagnosis and at the beginning of the drug use, spleen and liver size, presence of constitutive symptoms, transfusion needs.
Biological data collected: complete hemogram, CD34+ cells count, allele burden of JAK2V617F and calreticulin clones if DNA samples are collected.
These data will be collected avery 3 months during the two first years and every 6 months after.
The investigators also recorded adverse side effects if significative, the concomitant use of hematological drugs (cytoreductive treatments, ASE...), modalities of prescription of the drugs and the reason for stopping the drug and etiology of death if happened.
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| Measure | Description | Time Frame |
|---|---|---|
| Efficiency of the interferon in Myelofibrosis | Evaluation on the presence or not of splenomegaly and physical signs or the normalization of blood counts. | Evaluation at 1 year |
| Measure | Description | Time Frame |
|---|---|---|
| Rate of survival of patients with Myelofibrosis under interferon | Patients dead or alive at this time | Evaluation at 5 years |
| Tolerance of Interferon | Presence or not of psychiatric symptoms, cramps, hepatitic abnormalities |
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Inclusion Criteria:
Exclusion Criteria:
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Patients diagnosed for PMF or SMF and treated with pegykated interferon α2a
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| Name | Affiliation | Role |
|---|---|---|
| Jean-Christophe Ianotto, MD | CHRU de Brest | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU d'Angers | Angers | 49000 | France | |||
| Hopital du genevois |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 29217781 | Result | Ianotto JC, Chauveau A, Boyer-Perrard F, Gyan E, Laribi K, Cony-Makhoul P, Demory JL, de Renzis B, Dosquet C, Rey J, Roy L, Dupriez B, Knoops L, Legros L, Malou M, Hutin P, Ranta D, Benbrahim O, Ugo V, Lippert E, Kiladjian JJ. Benefits and pitfalls of pegylated interferon-alpha2a therapy in patients with myeloproliferative neoplasm-associated myelofibrosis: a French Intergroup of Myeloproliferative neoplasms (FIM) study. Haematologica. 2018 Mar;103(3):438-446. doi: 10.3324/haematol.2017.181297. Epub 2017 Dec 7. |
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| ID | Term |
|---|---|
| D055728 | Primary Myelofibrosis |
| ID | Term |
|---|---|
| D009196 | Myeloproliferative Disorders |
| D001855 | Bone Marrow Diseases |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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| Evaluation at 1 year |
| Annecy |
| France |
| CHRU de Brest | Brest | 29609 | France |
| Centre Hospitalier du Mans | Le Mans | France |
| Centre Hospitalier de Lens | Lens | France |
| Institut Paoli Calmette | Marseille | France |
| CH des pays de Morlaix | Morlaix | 29672 | France |
| Centre Hospitalier de Nice | Nice | France |
| AP-HP Hôpital Saint Louis | Paris | 75475 | France |
| CHIC de Cornouaille | Quimper | 29000 | France |
| Centre Hospitalier de Tours | Tours | France |
| CHU de Nancy | Vandœuvre-lès-Nancy | 54511 | France |