• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.41-43
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• 2015

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1053-1055
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• 2016

Background: Polycythemia rubra vera (PV), being a primary polycythemia, is caused by neoplastic proliferation of erythroid, megakaryocytic and granulocytic lineages which result in panmyelosis. PV patients have a somatic acquired mutation in the Janus kinase (JAK2) pathway, rendering cell proliferation independent of the normal regulatory mechanisms that regulate erythropoiesis. The rational of this study was to determine the prevalence of the JAK-2 V617F mutation in Pakistani patients with PV. Materials and Methods: In this cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. Patients were diagnosed based on WHO criteria for PV. All were screened for G-T point mutation (V617F) in the JAK2 gene on chromosome 9 by an allele specific PCR. Results: The mean age was $53.4{\pm}9.31years$ (range 36-72) and the male to female ratio was 2:1. The frequency of JAK2 V617F positivity in our PV patients was found to be 92.3%. Overall 30.7% of patients were asymptomatic and remaining 69.3% presented with symptomatic disease. The mean hemoglobin was $18.1{\pm}1.9g/dl$ with the mean hematocrit of $55.6{\pm}8.3%$. The mean total leukocyte count was $12.8{\pm}7.1{\times}10^9/l$ and the platelet count was $511{\pm}341.9{\times}10^9/l$. A positive correlation of JAK2 V617F mutation was established with high TLC count (P=0.01). No correlation of JAK2 V617F could be established with age or gender (P>0.05). Conclusions: The JAK2 V617F mutation frequency in our PV patients was similar to those reported internationally. Screening for the mutation in all suspected PV cases could be beneficial in differentiating patients with reactive and clonal erythrocytosis.

• Obstetrics & gynecology science
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• v.61 no.6
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• pp.655-661
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• 2018

Objective There is concern regarding the safety of delayed cord clamping (DCC) in babies born at less than 34 weeks' gestation. Therefore, the primary objective of this study was to compare the rates of hyperbilirubinemia and polycythemia during initial 7 days in infants born at less than 34 weeks' gestation and randomized to receive DCC by 120 seconds or early cord clamping (ECC) within less than 30 seconds. Methods One hundred pregnant women were randomly subjected to DCC or ECC at the time of birth in a tertiary referral hospital setting. Blood samples were obtained from each newborn at 48 hours and 7 days for hematocrit measurement. Serum bilirubin levels were estimated once the infant had clinically significant jaundice or at 72 hours. For the statistical analysis, the ${\chi}^2$ test, Student's t-test, or Wilcoxon rank sum test was used. Results The hematocrit was significantly higher in the DCC group than in the ECC group (P<0.001). None of the babies had polycythemia. Mean total serum bilirubin level was 6.6 mg/dL in the DCC group and 8.7 mg/dL in the ECC group (P<0.001). There was no increased risk of hyperbilirubinemia in the DCC group. Conclusion DCC benefits preterm neonates with no significant adverse effects.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.178-183
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• 2022

Polycythemia vera (PV) is a rare myeloproliferative disease that causes elevated absolute red blood cell (RBC) mass due to uncontrolled RBC production. Moreover, this condition has been associated with a high risk of ischemic stroke and large vessel stenosis or occlusion, with many studies reporting cerebral infarction in PV patients. Despite these findings, there have been no reports on the vessel wall MRI (VW-MRI) findings of the narrowed vessels in PV-associated ischemic stroke patients. To the best of our knowledge, this is the first report in English regarding the carotid VW-MRI findings of a 30-year-old male diagnosed with PV after being hospitalized due to stroke.

• 대한핵의학회:학술대회논문집
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• 1979.05a
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• pp.20-20
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• 1979

• Tuberculosis and Respiratory Diseases
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• v.64 no.3
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• pp.224-229
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• 2008

Erdheim-Chester disease (ECD) is a rare disease that is characterized by multi-organ involvement of foamy histiocytes. It causes systemic inflammation, and also demonstrates various clinical manifestations and has a poor prognosis. We encountered a case of ECD in a patient that had been treated for underlying polycythemia vera. As far as we know, this is the first reported case worldwide where ECD developed in association with polycythemia vera. A 59-year-old man visited our hospital due to pleuric pain at the right side of the chest. Pleural tissue that was obtained following a thoracoscopic biopsy showed non-Langerhan's cell histiocytosis, suggesting the presence of ECD. The histiocytes stained positively for CD68, but were negative for S-100 and CD1a. The patient also complained of pain at both hips and the right shoulder area. An X-ray and magnetic resonance image demonstrated that the lesion showed sclerosis and osteolysis in both the proximal femur and right humerus. Treatment was started with predinisolone, and subsequently cyclophosphamide was added. ECD is a very rare multi-systemic disease, and its cause and therapeutic options have not yet been defined. ECD has a poor prognosis. Therefore, we believe that additional case studies are needed prior to the determination of a novel therapy for ECD.

• The Korean Journal of Nuclear Medicine
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• v.11 no.1
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• pp.59-70
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• 1977

Eight patients with polycythemia vera were diagnosed from 1965 through 1977 among the patients of Seoul National University Hospital. In our series there were 4 men and 4 women with average age of 54 years at the time of diagnosis. Symptoms of facial plethora, headache, pruritus and dizziness were the most frequent manifestations of disease in order of frequency. At inital examination 8 of 8 patients had facial plethora, 6 of 8 patients splenomegaly and 4 if 8 patients hepatomegaly. The laboratory data revealed mean Red cell volume of 55+10ml/kg which was elevated in all cases. Leukocytosis was observed in 5 of 8 patients and thrombocytosis 2 of 8 patients. Leukocyte alkaline phosphatase was increased in all cases. Bone marrow aspiration disclosed typical panmyelosis in 5 of 8 patients. In one patient there was diffuse myelofibrosis on bone marrow biopsy. The combined diseases included 2 patient with hypertension and 1 patient with thrombosis of aorta and left renal artery. The patients were treated with phlebotomy alone in 2 patients, chemotherapy with busulfan in 1 patient and $^{32}P$ in 5 patients with favorable results. During the mean 29.9 months follow up period, there were development of iron deficiency in 3 patients, hyperuricemia in 2 patients and thrombosis in 1 patient. The occurrence of acute leukemia of myelofibrosis was not observed in the course of disease.

• Journal of Chest Surgery
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• v.29 no.6
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• pp.660-663
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• 1996

Pulmonary arteriovenous fistula is relatively rare disease, and the most common types are congenital in origin. The clinical presentation of pulmonary arteriovenous fistula ranges from incidental finding on a chest roentgenogram to polycythemia, cyanosis, congestive heart failure, and major neurologic deficits caused by paradoxical embolism. Recently we have experienced a case of asymptomatic, well-localized pulmonary arteriovenous fistula In a 13 years-old female patient, which was successfully treated by thoracoscopic lobectomy.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.12
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• pp.5013-5018
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• 2015

Background: Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell disorders characterized by proliferation of one or more myeloid lineages. Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical Philadelphia chromosome (Ph)-negative MPN that have a Janus Kinase 2 (JAK2) mutation, especially JAK2V617F in the majority of patients. The major complications of Ph-negative MPNs are thrombosis, hemorrhage, and leukemic transformation. Objective: To study clinical manifestations including symptoms, signs, laboratory findings, and JAK2V617F mutations of Ph-negative MPN (PV, ET and PMF) as well as their complications. Materials and Methods: All Ph-negative MPN (PV, ET and PMF) patients who attended the Hematology Clinic at Maharaj Nakorn Chiang Mai Hospital from January, 1 2003 through December, 31 2013 were retrospectively reviewed for demographic data, clinical characteristics, complete blood count, JAK2V617F mutation analysis, treatment, and complications. Results: One hundred and fifty seven patients were included in the study. They were classified as PV, ET and PMF for 68, 83 and 6 with median ages of 60, 61, and 68 years, respectively. JAK2V617F mutations were detected in 88%, 69%, and 100% of PV, ET and PMF patients. PV had the highest incidence of thrombosis (PV 29%, ET 14%, and PMF 0%) that occurred in both arterial and venous sites whereas PMF had the highest incidence of bleeding (PMF 17%, ET 11%, and PV 7%). During follow up, there was one ET patient that transformed to acute leukemia and five cases that developed thrombosis (three ET and two PV patients). No secondary myelofibrosis and death cases were encountered. Conclusions: Ph-negative MPNs have various clinical manifestations. JAK2V617F mutations are present in the majority of PV, ET, and PMF patients. This study confirmed that thrombosis and bleeding are the most significant complications in patients with Ph-negative MPN.

• Nuclear Medicine and Molecular Imaging
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• v.41 no.1
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• pp.66-67
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• 2007

POEMS syndrome is a rare disorder, also known as Crow-Fukase, PEP or Takatsuki syndrome. The acronym, POEMS, represents polyneuropathy, organomegaly, endocrinopathy, M protein and skin change. However, there are associated features not included in the acronym such as sclerotic bone lesions, Castleman disease, papilledema, thromobocytosis, peripheral edema, ascites, effusion, polycythemia, fatigue and clubbing. In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit and that reveal as osteosclerotic myeloma. Several cases of $^{18}F-FDG$ PET in multiple myeloma involvements were reported, but there was no previous literature that reported FDG PET findings in POEMS syndrome. We describe here a 66-year-old patient with POEMS syndrome who underwent $^{18}F-FDG$ PET/CT image.