Treatment options for patients with primary myelofibrosis

نویسندگان

  • A. B. Swali
  • M. Haj
چکیده

Background Primary myelofibrosis (PMF) is a myeloproliferative disorder resulting in progressive bone marrow fibrosis. In some PMF patients, a mutation in the gene coding for janus-associated kinase 2 (JAK2) results in cell proliferation and evasion of apoptosis. PMF typically occurs in older patients, and is associated with poor prognosis and low survival, with death occurring secondary to cardiovascular disease.(1) Investigations include full blood counts, blood film analysis, and bone marrow aspiration and trephine. Treatment is largely palliative; the single curative intervention of stem-cell transplant being available only to low-risk younger patients. Potential supportive treatments include the use of JAK2 inhibitors. Method This case report studies a patient who was diagnosed with PMF at

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Primary myelofibrosis: current therapeutic options

Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to t...

متن کامل

Splenomegaly in myelofibrosis—new options for therapy and the therapeutic potential of Janus kinase 2 inhibitors

Splenomegaly is a common sign of primary myelofibrosis (PMF), post-polycythemia vera myelofibrosis (post-PV MF), and post-essential thrombocythemia myelofibrosis (post-ET MF) that is associated with bothersome symptoms, which have a significant negative impact on patients' quality of life. It may also be present in patients with advanced polycythemia vera (PV) or essential thrombocythemia (ET)....

متن کامل

Emerging therapeutic options for myelofibrosis: a Canadian perspective.

Myelofibrosis (MF) is a clonal stem cell disorder characterized by cytopenias, splenomegaly, marrow fibrosis, and systemic symptoms due to elevated inflammatory cytokines. MF is associated with decreased survival. The quality of life of patients with MF is similar to other advanced malignancies. Allogeneic hematopoietic cell transplantation is a curative treatment, but is applicable to a minori...

متن کامل

Treatment and management of myelofibrosis in the era of JAK inhibitors [Corrigendum]

Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of debilitating symptoms. Until recently, the therapeutic options for patients with MF consisted of ...

متن کامل

Myelofibrosis Associated with Romiplostim Treatment in a Patient with Immune Thrombocytopenia

Immune thrombocytopenia is characterized by antibody-mediated platelet destruction and insufficient platelet production resulting in isolated thrombocytopenia in the absence of underlying cause. Despite many treatment options, low-to-intermediate rates of remission and high rates of resistance to treatment are seen. Approximately 20% of patients do not attain a hemostatic platelet count after s...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره   شماره 

صفحات  -

تاریخ انتشار 2016