No abstract available.
Down Syndrome* ; Myeloproliferative Disorders*

No abstract available.
Down Syndrome* ; Myeloproliferative Disorders*

No abstract available.
Down Syndrome* ; Myeloproliferative Disorders*

Objectives: Finding the markers/symptoms of cytology for each disease and all diseases belonged the myeloproliferative syndrome (MPS). Subjects: 55 patients in the H÷u NghÞ Hospital during 1985-1998. Results: The increased leukocytes and immature cells found in the peripheral blood of patients with MPS. However, the level and rate of these were different: 1st increased level (chronic granulocytic leukemia, 2nd increased level (essential myelofibrosis), 3th increased level(primary multi erythro was always accompanied with erythrocythmia where as the chronic granulocytic leukemia and essential myelofibrosis were accompanied with anemia. The primary thrombocythenia remains the normal erythrocyte. The thrombocythenia is not a obviously marker in the primary thrombocyte but also in the chronic granulocytic leukemia and the primary multierythrocyte. The thrombocyte found in the peripheral blood of patients with myeloproliferative syndrome. The number of marrow cells were reduced in the essential myelofibrocythemia remained at normal level. The significant increased number of marrow cells found in the most of patients with the chronic granulocytic leukemia. The obvious increased rate of germ cell of granulocyte and thrombocyte in the marrow cell picture.
Myeloproliferative Disorders ; cytology ; diagnosis

This study included 55 patients who admitted to Huu Nghi Hospital from 1985 to 1998. It is found that spleen enlargement is common in myeloproliferative conditions, with the incidence is 100% of patients who have chronic granulocytic leukemia and those have idiopathic myelosclerosis. These patients have grade II or more of spleen enlargement. This symptom is less common in patients who have polycythemia vera or essential trombocythemia, and these patients are likely to have grade I of spleen enlargement. Anemia is more likely to be found in patients with chronic granulocytic leukemia, especially in those with idiopathic myelosclerosis, but this symptom is infrequent in patients with essential trombocythemia. Patients with polycythemia vera in typical have excessive blood. Infection and hemorrhage occurred predominantly in patients with chronic granulocytic leukemia and in some cases of essential trombocythemia. Symptoms of high blood pressure, tip finger bruise and limb weakness have been found mainly in patients with polycythemia vera. In some cases with polycythemia vera, both red cell and white cell counts are increased. While patients with chronic granulocytic leukemia, polycythemia vera and idiopathic myelosclerosis expresses obvious clinical symptoms, the symptoms in patients who have essential trombocythemia are unmarked.
Myeloproliferative Disorders ; Hemorrhage ; diagnosis ; syndrome

Bone Marrow Neoplasms ; Humans ; Mutation ; Myeloproliferative Disorders

OBJECTIVETo evaluate the efficacy and safety of ruxolitinib in treatment of myeloproliferative neoplasm.

METHODSRandom clinical trials (~September 30, 2017) were identified from PubMed, Embase, Cochrane Library, Clinical Trials, CBM and Chinese Journal Full-text Database. The quality of RCT was assessed by Cochrane risk bias. Meta analysis was performed with Revman 5.3.

RESULTSRuxolitinib was efficacious in relieving splenomegaly (RR 49.12, 95% CI [15.81-152.59], P<0.001). The incidence of anemia significantly increased after ruxolitinib treatment (RR 1.71, 95% CI [1.05-2.77], P=0.16), while the thrombocytopenia (RR 1.04, 95% CI [0.50-2.16], P=0.92) and neutropenia (RR 2.46, 95% CI [0.91-6.61], P=0.07) had no statistical difference as compared with that in control group. Ischemia events had no significant difference as compared with control (RR 0.57, 95% CI [0.33-1.00], P=0.05). Infection events had no significant difference as compared with the control group (RR 1.18, 95% CI [0.79-1.78], P=0.24).

CONCLUSIONRuxolitinib is an efficacious therapeutic strategy on MPD with controlling splenomegaly. However,anemia events and bleeding events may threat its clinical safety, so more high quality RCT are needed.

Summary Sporotrichosis is a rare and chronic granulomatous subcutaneous mycotic infection caused by a dimorphic fungus, Sporothrix schenckii. We describe a patient with disseminated cutaneous sporotrichosis who was later diagnosed with myeloproliferative neoplasm and discuss the challenges and importance in diagnosing this rare condition.
Sporotrichosis ; Granulomatous Disease, Chronic ; Myeloproliferative Disorders

Humans ; Pyoderma Gangrenosum ; Myeloproliferative Disorders ; Neoplasms

Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell diseases characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased mature and immature cells in peripheral blood. As the most important discovery in recent studies of MPN, JAk2V617F mutation is considered to closely relate with the pathogenesis of MPN. The mutated JAK2 lost self-inhibition, and then, the sustained activation leads to a series of disorders in downstream signal transduction pathways, eventually resulting in malignant cell proliferation. A variety of methods have been used in quantitative/qualitative detection of JAK2V617F mutation, and researches about JAK2V617F mutation and its clinical features have also made some progress. However, it must be noted that there are still some unsolved problems, such as the role of JAk2V617F mutation in pathogenesis of MPN needs further exploration, effective targeted therapy for JAK2 is a attractive topic, and the application of JAK2V617F mutation in disease diagnosis also requires a deep research. In this review, the latest progress from different aspects is summarized briefly, including JAK2 and JAK2V617F mutation, effects of JAK2V617F mutation on the pathogenesis, clinical correlation of JAK2V617F with MPN, and targeting therapy.
Humans ; Janus Kinase 2 ; genetics ; Mutation ; Myeloproliferative Disorders ; genetics ; pathology