Dynamics of Fibrosis in Chronic Idiopathic (Primary) Myelofibrosis During Therapy: A follow-up Study on 309 Patients
Autor: | Hans Michael Kvasnicka, Volker Diehl, Annette Schmitt-Graeff, Juergen Thiele |
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Rok vydání: | 2003 |
Předmět: |
Cancer Research
medicine.medical_specialty Idiopathic myelofibrosis Biopsy Interferon alpha-2 Gastroenterology Bone Marrow Fibrosis Internal medicine medicine Humans Hydroxyurea Myelofibrosis Busulfan Retrospective Studies medicine.diagnostic_test business.industry Follow up studies Interferon-alpha Hematology medicine.disease Recombinant Proteins Surgery medicine.anatomical_structure Oncology Primary Myelofibrosis Disease Progression Observational study Bone marrow business Follow-Up Studies medicine.drug |
Zdroj: | Leukemia & Lymphoma. 44:949-953 |
ISSN: | 1029-2403 1042-8194 |
Popis: | Controversial issues in chronic idiopathic myelofibrosis (IMP) are amongst others the evolution of the disease process and the influence of therapy on the dynamics of fibrosis. For this reason, a multicenter observational study was performed on 309 patients with IMF that had a long follow-up including 822 bone marrow biopsies at a median interval of 32 months. In addition to a control group (156 patients) with symptomatic treatment, monotherapy consisted of busulfan (30 patients), hydroxyurea (52 patients), interferon (26 patients) and various combinations (48 patients). Density and quality (reticulin/collagen) of fibers was determined by a semiquantitative scoring system. Independent of therapeutic regimens at the time of the last bone marrow biopsy 67% of the patients with grades 0-2 fibrosis revealed a progression, 42% stable state and 6% regression of myelofibrosis. Because of significant differences concerning frequencies of biopsies and endpoints of examinations, individual changes in the grades of fibrosis were evaluated with regard to treatment applied at standardized intervals of 20 months. According to this calculation no relevant differences in the dynamics of myelofibrosis (progression, stable state) was detectable in the control group compared to the other therapeutic modalities. The few patients with a regression of myelofibrosis usually presented with severe hypoplasia compatible with a myelo-ablative effect by aggressive chemotherapy. In conclusion, persuasive evidence has been produced that myelofibrosis in IMF is characterized by a stepwise progression and that this process is not significantly influenced by current treatment strategies. |
Databáze: | OpenAIRE |
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