SHM Converge Scientific Abstract ePoster Gallery-A Case of Poorly Controlled Polycythemia Vera Leading to Chorea

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This poster presents a rare case of chorea caused by poorly controlled polycythemia vera (PV), a myeloproliferative disorder associated with erythrocytosis, thrombocytosis, and sometimes leukocytosis. Choreiform movements occur in only about 0.5–5% of PV patients and usually indicate inadequate hematologic control, but symptoms are often reversible with treatment.<br /><br />The patient was a 76-year-old woman with chronic diastolic heart failure and known PV, treated with hydroxyurea. She presented after two days of progressive shortness of breath, drooling, and involuntary rhythmic movements involving the mouth and all four extremities. She had stopped both furosemide and hydroxyurea two days earlier because of malaise. Exam showed continuous orofacial and limb movements, mild hypertension, borderline oxygen saturation, and basilar crackles. Labs showed elevated hematocrit (49.2%) and marked leukocytosis (84.6). Prior hematology records documented difficulty maintaining hematocrit control and medication adherence.<br /><br />A broad neurologic and metabolic workup was unrevealing. CT head was normal, and studies including thyroid, vitamin, infectious, and blood smear testing were negative. MRI showed hyperactive bone marrow but no stroke, mass, or other structural brain disease. Clonazepam did not improve the movements.<br /><br />Treatment focused on restoring hematologic control: home medications were restarted, hydroxyurea was increased, and phlebotomy was performed. Symptoms resolved as hematocrit improved to 40%.<br /><br />The authors emphasize that PV chorea is most often seen in elderly women and usually affects the orofacial muscles. Proposed mechanisms include hyperviscosity-related reduced basal ganglia perfusion and neurotransmitter or estrogen-related receptor sensitivity changes. The case highlights the importance of checking a complete blood count in older patients with new movement disorders, since PV chorea is rare but highly treatable and early recognition may also help prevent serious complications such as thrombosis, pulmonary embolism, and stroke.

Asset Subtitle

Giles Knowles

Meta Tag

Author List Andrea Labus, Giles Knowles, Oliver Lin

Category Clinical Vignettes

Concept Polycythemia Vera

Concept Chorea

Concept Hematocrit

Concept Leukocytosis

Concept Phlebotomy

Distinguished Non-Finalist

Presenter Organization WVU Medicine

Presenting Author Giles Knowles

Track Adult

Keywords

polycythemia vera

chorea

orofacial movements

myeloproliferative disorder

erythrocytosis

thrombocytosis

hydroxyurea

phlebotomy

basal ganglia

hematocrit control

Polycythemia Vera

Chorea

Hematocrit

Leukocytosis

Phlebotomy