Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 2 cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91,800/microL (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1,661,000/microL. 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/microL.
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PMID:A case of transition of polycythemia vera to chronic neutrophilic leukemia. 1568 21

This is a case of a thrombo-occlusion occurring below an inferior vena cava (IVC) filter inserted in a patient with polycythemia vera and deep vein thrombosis. The patient was a 48-year-old man with polycythemia vera and a chief complaint of swelling, redness, and bursting pain in his right leg. After admission, contrast-enhanced computed tomography scanning demonstrated a pulmonary artery thrombus and deep vein thrombosis. We inserted a Gunther tulip vena cava filter on day 1 for the prevention of pulmonary embolism (PE), and started anticoagulation therapy based on the guideline of the Japanese Circulation Society for DVT. In addition to intravenous anticoagulants, we started therapeutic phlebotomy to improve the hypercoagulability state. On day 4, our patient complained of back pain caused by thrombo-occlusion below the IVC filter, despite the anticoagulation therapy and two therapeutic phlebotomies. From this case, we concluded it is important to lower hemoglobin level and hematocrit as early as possible for IVC-filter-insertion in patients with polycythemia vera.
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PMID:Thrombo-occlusion of inferior vena cava filter in a patient with polycythemia vera. 1978 71

Spinal subdural hematoma (SDH) is a rare condition and can be caused by several factors. Concomitant cranial and spinal SDH is even much less common. We present a 77-year-old male patient with lower back pain, paraparesis, and urinary retention following a sudden onset headache. Imaging revealed concomitant cranial and spinal SDH related to cerebral venous thrombosis (CVT) associated with hemorrhagic venous infarct. Laboratory examinations were consistent with polycythemia vera. There was no history of trauma and previous cranial surgery. Brain angiography did not reveal any evidence of arteriovenous fistula or vascular malformation. Since lower back pain occurred shortly after the headache and there was no other reasonable explanation for spinal hemorrhage, we suppose that the mechanism of spinal SDH is the migration of blood from the intracranial compartment. Therefore, this is the first report of concomitant spinal SDH and cerebral hemorrhage associated with CVT in a patient with myeloproliferative disease.
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PMID:A Case Report of Cerebral Venous Thrombosis in Polycythemia Vera Presenting with Intracranial and Spinal Subdural Hematoma. 2067 55