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Primary myelofibrosis: current therapeutic options

View Article: PubMed Central - PubMed

ABSTRACT

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 the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach.

No MeSH data available.


Related in: MedlinePlus

Proposed treatment algorithm for primary myelofibrosis patients according to DIPSS risk groups. Therapeutic decisions take the risk group and patients’ particularities into account. The palliation of symptoms needs to be continuously pursued, independently of the therapeutic choice, and are additive to disease-modifying treatment, as indicated by the dotted lines. PMF: primary myelofibrosis; DIPSS: Dynamic International Prognostic Scoring System; Allo-HSCT: allogeneic stem cell transplantation.
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fig0005: Proposed treatment algorithm for primary myelofibrosis patients according to DIPSS risk groups. Therapeutic decisions take the risk group and patients’ particularities into account. The palliation of symptoms needs to be continuously pursued, independently of the therapeutic choice, and are additive to disease-modifying treatment, as indicated by the dotted lines. PMF: primary myelofibrosis; DIPSS: Dynamic International Prognostic Scoring System; Allo-HSCT: allogeneic stem cell transplantation.

Mentions: Asymptomatic PMF patients in the low and intermediate-1 risk groups according to DIPSS have a long expected survival and do not usually require specific treatment. If symptomatic, patients in these risk categories can be treated according to the prevailing symptom, as discussed above. Intermediate-2 and high-risk DIPSS patients have a shortened survival and should be considered for disease-modifying therapies, such as JAK inhibitors and allo-HSCT, when tolerated; the decision between these two options has to be individualized taking into account the patient's age, performance status and preferences. Combination therapies for the palliation of symptoms, regardless of the risk group and the prognosis, are beneficial and should be implemented to improve patients’ quality of life. A treatment algorithm is proposed in Figure 1.


Primary myelofibrosis: current therapeutic options
Proposed treatment algorithm for primary myelofibrosis patients according to DIPSS risk groups. Therapeutic decisions take the risk group and patients’ particularities into account. The palliation of symptoms needs to be continuously pursued, independently of the therapeutic choice, and are additive to disease-modifying treatment, as indicated by the dotted lines. PMF: primary myelofibrosis; DIPSS: Dynamic International Prognostic Scoring System; Allo-HSCT: allogeneic stem cell transplantation.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4997889&req=5

fig0005: Proposed treatment algorithm for primary myelofibrosis patients according to DIPSS risk groups. Therapeutic decisions take the risk group and patients’ particularities into account. The palliation of symptoms needs to be continuously pursued, independently of the therapeutic choice, and are additive to disease-modifying treatment, as indicated by the dotted lines. PMF: primary myelofibrosis; DIPSS: Dynamic International Prognostic Scoring System; Allo-HSCT: allogeneic stem cell transplantation.
Mentions: Asymptomatic PMF patients in the low and intermediate-1 risk groups according to DIPSS have a long expected survival and do not usually require specific treatment. If symptomatic, patients in these risk categories can be treated according to the prevailing symptom, as discussed above. Intermediate-2 and high-risk DIPSS patients have a shortened survival and should be considered for disease-modifying therapies, such as JAK inhibitors and allo-HSCT, when tolerated; the decision between these two options has to be individualized taking into account the patient's age, performance status and preferences. Combination therapies for the palliation of symptoms, regardless of the risk group and the prognosis, are beneficial and should be implemented to improve patients’ quality of life. A treatment algorithm is proposed in Figure 1.

View Article: PubMed Central - PubMed

ABSTRACT

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 the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach.

No MeSH data available.


Related in: MedlinePlus