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.

Humans ; Pyoderma Gangrenosum ; Myeloproliferative Disorders ; Neoplasms

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

OBJECTIVETo investigate the frequency and clinical implication of JAK2 mutation in patients with myeloproliferative neoplasm(MPN)and the correlation between the mutation and thrombosis.

METHODSThe clinical and laboratory data of 107 MPN patients was retrospectively analyzed. JAK2 mutation were detected with allele-specific polymerase chain reaction (AS-PCR) and sequencing. The significance of the mutation in disease diagnosis and molecular pathogenesis and the correlation between the mutation and thrombosis was analysed.

RESULTSJAK2 mutation was detected in 71 (66.4%) and thrombosis in 34 (31.8%) of the 107 MPN patients. Thrombosis occurred in 34.8% (16/46) of polycythemia vera (PV), 32.6% (14/43) of essential thrombocythemia (ET), and 22.2% (4/18) of primany myelofibrosis (PMF) patients. The difference among the 3 groups was not significant (χ(2) = 0.96, P > 0.05). The frequency of thrombosis in JAK2(+) MPN patients (82.4%, 28/34) was higher than that in JAK2(-) MPN patients (17.6%, 6/34) (χ(2) = 5.71, P < 0.05). The frequency of thrombosis in MPN patients > 60 years was higher (41.5%, 27/65) than that in patients < 60 years (16.7%, 7/42) (χ(2) = 7.28, P < 0.01).

CONCLUSIONJAK2 V617F mutation occurs in significant percentage of Chinese patients with MPN. Patients with JAK2 mutation and older age are more succeptible to thrombosis. JAK2 mutation screening in patients with unknown thrombosis is helpful to reveal the underlying latent-MPN.