Ptch2 loss drives myeloproliferation and myeloproliferative neoplasm progression

Ptch2 loss drives myeloproliferation and myeloproliferative neoplasm progression

JAK2V617F(+) myeloproliferative neoplasms (MPNs) frequently progress into leukemias, but the factors driving this process are not understood. Here, we find excess Hedgehog (HH) ligand secretion and loss of PTCH2 in myeloproliferative disease, which drives canonical and noncanonical HH-signaling. Interestingly, Ptch2(-/-) mice mimic dual pathway activation and develop a MPN-phenotype with leukoc...

JAK2V617F somatic mutation in the general population: myeloproliferative neoplasm development and progression rate.

Clinical significance of the JAK2V617F mutation in patients with a myeloproliferative neoplasm has been the target of intensive research in recent years. However, there is considerably uncertainty about prognosis in JAK2V617F positive individuals without overt signs of myeloproliferative disease. In this study, we tested the hypothesis that increased JAK2V617F somatic mutation burden is associa...

Hypothetical Atopic Dermatitis-Myeloproliferative Neoplasm Syndrome

Atopic dermatitis (AD) is a chronic inflammatory skin disease. Myeloproliferative neoplasms (MPNs) are hematopoietic malignancies caused by uncontrolled proliferation of hematopoietic stem/progenitor cells. Recent studies have described several mutant mice exhibiting both AD-like skin inflammation and MPN. Common pathways for skin inflammation encompass overexpression of thymic stromal lymphopo...

Low-dose methotrexate in myeloproliferative neoplasm models.

Association of primary myeloperoxidase deficiency and myeloproliferative neoplasm.

We report a 63-year-old woman with myeloproliferative neoplasm (MPN), who presented with inflammatory involvement of the skin. The white cell count was 31,800/μL and the platelet count was 709×10/μL. The positivity of neutrophil alkaline phosphatase was 100% and the score was 281. The bone marrow was hypercellular with enlarged megakaryocytes with mature cytoplasm and multilobulated nuclei (Pic...