MPLW515L Is a Novel Somatic Activating Mutation in Myelofibrosis with Myeloid Metaplasia

MPLW515L Is a Novel Somatic Activating Mutation in Myelofibrosis with Myeloid Metaplasia

BACKGROUND The JAK2V617F allele has recently been identified in patients with polycythemia vera (PV), essential thrombocytosis (ET), and myelofibrosis with myeloid metaplasia (MF). Subsequent analysis has shown that constitutive activation of the JAK-STAT signal transduction pathway is an important pathogenetic event in these patients, and that enzymatic inhibition of JAK2V617F may be of therap...

Myelofibrosis with myeloid metaplasia (MMM), Idiopathic myelofibrosis, Agnogenic myeloid metaplasia

MMM usually presents with fatigue, weight loss, splenomegaly with or without symptoms. Anemia and various alterations of the white blood cell and/or platelet count are frequently seen at diagnosis. Thrombocytopenia-related bleeding may occur. MMM must be distinguished from myelodysplasia with fibrosis, from acute megakayoblastic leukemia and acute myelofibrosis. As the disease progresses, incre...

[Myelofibrosis with myeloid metaplasia].

Introduction Myelofibrosis with myeloid metaplasia (MMM) represents both agnogenic myeloid metaplasia (AMM) and the fibrotic stages of polycythemia vera (PV) and essential thrombocythemia (ET). The latter two conditions are also referred to as post polycythemic myelofibrosis (PPM) and post thrombocythemic myelofibrosis (PTM). MMM is currently classified under the broad category of chronic myelo...

Lenalidomide therapy in myelofibrosis with myeloid metaplasia.

We present results of 2 similarly designed but separate phase 2 studies involving single-agent lenalidomide (CC-5013, Revlimid) in a total of 68 patients with symptomatic myelofibrosis with myeloid metaplasia (MMM). Protocol treatment consisted of oral lenalidomide at 10 mg/d (5 mg/d if baseline platelet count < 100 x 10(9)/L) for 3 to 4 months with a plan to continue treatment for either 3 or ...

Experimental induction of myelofibrosis with myeloid metaplasia.