Aged ; Female ; Humans ; Myocardial Infarction ; diagnosis ; Polycythemia Vera ; complications

Deep venous thrombosis (DVT) of the upper extremity has been recently been recognized as being more common than previously reported (probably because of the increasingly frequent use of subclavian venous access). Since the initial descriptions of axillary-subclavian vein thrombosis more than 100 years ago, studies cites a 1.3% to 2.1% incidence of all DVT that occurs in the axillary or subclavian veins. Axillary-subclavian vein thrombosis is commonly associated with significant morbidity. The most serious aspect of morbidity and mortality is pulmonary embolization. Venography or digital subtraction studies are most reliable in allowing detection of thrombosis and Duplex imaging of vein is useful in following the course and assessing the effect of treatment. Early diagnosis and initiation of treatment before thrombus organization are important for successful outcome. Polycythemia vera are at an especially high risk for both thrombotic and hemorrhghic events and postoperative complications. We report a case of axillary-subclavian vein thrombosis after left subclavian vein catheterization in polycythemia vera.
Catheterization* ; Catheters* ; Early Diagnosis ; Incidence ; Mortality ; Phlebography ; Polycythemia Vera* ; Polycythemia* ; Postoperative Complications ; Subclavian Vein* ; Thrombosis* ; Upper Extremity ; Veins* ; Venous Thrombosis

Chronic Neutrophilic Leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of Philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of Polycythemia Vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91, 800/micro L (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1, 661, 000/micro L. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M: E ratio of 10: 1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no Philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40, 000/micro L.
Aged ; Aged, 80 and over ; Female ; Humans ; Leukemia, Neutrophilic, Chronic/*etiology ; Polycythemia Vera/*complications

Aged ; Humans ; Ischemic Attack, Transient ; etiology ; Male ; Polycythemia Vera ; complications ; diagnosis

OBJECTIVETo investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.

METHODSA cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.

RESULTSFatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).

CONCLUSIONMPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.

Humans ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; diagnosis ; physiopathology ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; complications ; Thrombocythemia, Essential ; complications

in the leg and the toe and was treated with angioplasty and thrombolytic therapy with phlebotomy.
Female ; Humans ; Middle Aged ; Polycythemia Vera/complications/*diagnosis/ultrasonography ; Portal Vein/*abnormalities ; Tomography, X-Ray Computed ; Venous Thrombosis/etiology

OBJECTIVETo understand the clinical feature and natural course of polycythemia vera (PV).

METHODSThe clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed.

RESULTSThere are 122 males and 63 females. The mean age was (52.7 +/- 14.1) years. The mean hemoglobin level was (208.3 +/- 21.2) g/L. Pancytosis was displayed in 74 (40%) cases, excess of red blood cells in 33 (17.8%), excess of red blood cells and granulocytes in 67 (36.2%) and excess of red blood cell and platelets in 11 (5.9%). Splenomegaly was found in 123 (66.5%) patients and hepatomegaly in 30 (16.2%). Quantitative assess of serum Epo was done in 25 patients. The level was low in 16 (64.2%) and normal in 9 (36.0%). Hematopoietic progenitor culture yields was elevated in 11 patients, endogenous erythroid colonies (EEC) formation was found in 10 cases (90.9%). Eighty two patients (44.3%) had 101 attacks of vascular thrombotic incidents, 7 patients developed myelofibrosis (MF). Secondary cancer occurred in 1 patient. Two patients died of thrombosis.

CONCLUSIONPV is an elderly adult myeloproliferative disease with a high frequency of thrombosis. EEC can be found out in PV patients. The serum Epo level is not increased in PV patients. The main sequelae of PV is MF.

Adult ; Aged ; Erythrocyte Count ; Female ; Hemoglobins ; metabolism ; Hepatomegaly ; etiology ; Humans ; Leukocyte Count ; Male ; Middle Aged ; Polycythemia Vera ; blood ; complications ; pathology ; Primary Myelofibrosis ; etiology ; Splenomegaly ; etiology ; Thrombosis ; etiology

OBJECTIVETo reassess the natural history of polycythemia vera (PV) in Chinese and evaluate the relationship between the incidence of thrombosis, post-polycythaemic myelofibrosis with myeloid metaplasia( PPMM) , leukemia transformation and the therapeutic outcome and prognostic factors.

METHODSThe clinical manifestations, laboratory parameters and treatment were retrospectively analyzed in 287 patients with PV. Univariate analysis of prognostic factors was performed using Log-rank model and multivariate analysis using COX model in term of the incidence of thrombosis, PPMM, hematologic or non hematologic cancers and mortality.

RESULTSOf the 287 patients, the median follow-up time was 46 (8-360) months. 208 thromboses were recorded in 115 patients. Twice or more thrombotic events occurred on 59 patients (51.34%). Most of these episodes occurred either at presentation or in the 2 years before diagnosis. Elder patients, prior thrombosis, poor response to therapy were associated with poor prognosis. With these three adverse prognostic factors, the patients could be separated into different risk groups. The incidence of thrombosis was higher in high risk group. 36 patients progressed to PPMM, the median time to PPMM was 80 (7-190) months. Higher WBC count, splenomegaly and treatment with alkylating agent and hydroxy-carbamide (HU) were associated with poor prognosis. 2 cases progressed to AML. 1 to lymphoma and 1 to nonhematologic cancer. 13 patients died, the cause of death was fatal thrombosis in 9 and AML in 2.

CONCLUSIONThe incidence of thromboembolism is higher and the time to myelofibrosis was shorter in Chinese PV patients than in western PV patients. The main factors that influence the survival of PV patients are thromboembolism and leukemia transformation.

Acute Disease ; Female ; Follow-Up Studies ; Humans ; Leukemia ; etiology ; Male ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; etiology ; Prognosis ; Risk Factors ; Thromboembolism ; etiology

No abstract available.
Acute Disease ; Aged ; Bone Marrow/metabolism/pathology ; Female ; Humans ; Janus Kinase 2/genetics ; Leukemia/*complications/therapy ; Mutation, Missense ; Polycythemia Vera/*diagnosis/etiology ; Real-Time Polymerase Chain Reaction ; Remission Induction

A 42-year-old Chinese man presented with left-sided chest pain and splenomegaly. Full blood count revealed erythrocytosis, while plain radiograph and computed tomography of the abdomen and pelvis revealed hepatosplenomegaly with splenic infarction. Further workup confirmed the diagnosis of polycythaemia vera. Clinical and imaging features of polycythaemia vera, as well as the potential pitfalls in image interpretation, are discussed in this article.
Adult ; Brain ; pathology ; Cerebral Infarction ; complications ; diagnosis ; Diagnostic Imaging ; methods ; Humans ; Liver ; pathology ; Male ; Middle Aged ; Polycythemia Vera ; diagnosis ; diagnostic imaging ; Radiography, Abdominal ; methods ; Seizures ; diagnosis ; Splenomegaly ; diagnosis ; Tomography, X-Ray Computed ; methods ; Venous Thrombosis ; diagnosis