ABSTRACT
Jauns kinase (JAK)/transducer and activator of transcription（STAT） pathway is a classical approach to study the rapid changes of the gene expression in specific target cells by a variety of extracellular signals. The JAK and STAT transfer cytokine receptor signaling plays a unique role in multiple cellular and molecular biological changes.The abnormal signal of JAK/STAT pathway will lead to the hematopoietic abnormalities.Studies had shown that the abnormal activation of JAK2/STAT signaling pathway are in many kinds of malignant hematological diseases, such as in acute lymphoblastic/myeloid leukemia, chronic myeloid leukemia, lymphoma, myelodysplastic syndromes, myeloprofilerative neoplasm, especially in the patients of myeloproliferative neoplasm(MPN) with JAK gene mutation(JAK2V617F), this mutation has an important value for MPN diagnosis. At present, the effect of the specific inhibitors of JAK2 has showed good perspective, which had been applied to clinic treatment and achieved remarkable curative effect. In this review, the JAK2/STAT signaling transduction, the JAK2 signal and hematologic malignancies, the kagulation of signaling pathway and the inhibitors of JAK2/STAT signaling pathway are summarized.
Subject(s)
Humans , Hematologic Neoplasms , Janus Kinase 2 , Mutation , STAT Transcription Factors , Signal TransductionABSTRACT
To find the relationship between myelodysplastic syndrome (MDS) and refractory monolineage cytopenia, thirteen cases of MDS with early presentation of monolineage refractory cytopenia were analyzed retrospectively. The results were as follows: (1) The percentage of 13 cases with refractory monolineage cytopenia were 5.9% of the total 219 MDS patients in the past 10 years. (2) The median time of patients with monlineage cytopenia to M DS diagnosed was 48.5 +/- 55.3 months. The median times from monolineage cytopenia to MDS diagnosed for patients with neutropenia, erythrocytopenia and thrombocytopenia were 12.5 +/- 9.5 months, 53.8 +/- 54.6 months and 59.2 +/- 65.5 months, respectively. (3) The common characteristics of 13 cases were as follows: (a) the macrocytic erythrocytes in peripheral blood and the percentage of intermediate and late erythroblast in bone marrow were increased; (b) occasionally few cells with dysplasia could be found; (c) all patients with erythrocytopenia and thrombocytopenia transformed to RA and RAS while the most of patients with neutropenia transformed to RAEB subtype; (d) autoantibody could be found in part of the patients. It is concluded that some of refractory monolineage cytopenias in essence are the early states of MDS.