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Polycythemia Vera Presenting with Generalized Chorea: A Case Report and Review of the Literature

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초록

Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by erythrocytosis. Clinical symptoms can range in severityfrom headache and tinnitus to thrombohemorrhagic complications. Neurologic symptoms are common at the onset of polycythemia;however, chorea due to PV is a rare complication. We present the case of a 77-year-old female who was referred to ourhospital because of choreic movement of the limbs, head and face. She was diagnosed with JAK2V617F mutation-positive PV. Herchorea was completely resolved by phlebotomy combined with hydroxyurea and aspirin.

Abstract

Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by erythrocytosis. Clinical symptoms can range in severityfrom headache and tinnitus to thrombohemorrhagic complications. Neurologic symptoms are common at the onset of polycythemia;however, chorea due to PV is a rare complication. We present the case of a 77-year-old female who was referred to ourhospital because of choreic movement of the limbs, head and face. She was diagnosed with JAK2V617F mutation-positive PV. Herchorea was completely resolved by phlebotomy combined with hydroxyurea and aspirin.

참고문헌 (10)

  1. Yoshinaga K, "JAK2 V617F mutation is rare in idiopathic erythrocytosis: a difference from polycythemia vera", Int J Hematol, 88, pp.82~87 (2008)
  2. Silverstein A, "Neurologic complications of polycythemia", Ann Intern Med, 57, pp.909~961 (1962)
  3. Mas JL, "Chorea and polycythaemia", J Neurol, 232, pp.169~171 (1985)
  4. Tefferi A, "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel", Blood, 110, pp.1092~1097 (2007)
  5. Berlin NI, "Diagnosis and classification of polycythemias", Semin Hematol, 12, pp.339~351 (1975)
  6. Edwards PD, "Chorea, polycythaemis, and cyanotic heart disease", J Neurol Neurosurg Psychiatry, 38, pp.729~739 (1975)
  7. Ania BJ, "Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989", Am J Hematol, 47, pp.89~93 (1989)
  8. Bruyn GW, "Chorea and polycythaemia", Eur Neurol, 23, pp.26~33 (1984)
  9. Lew J, "Hemichorea in a patient with JAK2V617F blood cells", Blood, 121, pp.1239~1240 (2013)
  10. Ahn BY, "Isolated monocular visual loss as an initial manifestation of polycythemia vera", J Neurol Sci, 258, pp.151~153 (2007)

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