Management of polycythemia vera by endoscopic parathyroidectomy

Abstract

Polycythemia vera (PV) is a hemotologic disease. Majority of persons with PV, essential thrombocythemia and primary myelofibrosis show the Janus kinase (JAK) 2 gene– which is needed for the normal development of blood cells. PV is generally controlled with medication: hydroxyurea or pipobroman. Phlebotomy is the mainstay of therapy for PV. Association between hyperparathyroidism and PV has been very sparingly considered. Primary hyperparathyroidism may produce a growth factor, which induces pancytosis, especially in the presence of high levels of ionized calcium. It is found that parathyroid hormone levels (PTH) could influence hemopoiesis through a direct action on hemopoietic precursors. A 52-year-old female presented with complaints of fever associated with headache and generalized weakness. On evaluation, her hemoglobin value was found to be high (Hb– 18.8 g/dl) with leukocytosis. She was evaluated for polycythemia, which showed JAK-2 mutation positivity. She was started on Enteric Coated Aspirin. She was managed initially with twice-weekly venesection to keep packed cell volume <45. It was observed that she was having very high PTH– 569.9 pg/ml and low Vitamin D levels– 8.41 mg/ml. She underwent a Technetium 99M Sestamibi Scan (TcMIBI), which showed a large adenoma involving the left inferior parathyroid gland. She was taken up for endoscopic parathyroid removal through the left axillo-breast approach. Intraoperatively, PTH value had dropped from 569.9 pg/ml to 62 pg/ml. Hemoglobin level decreased to normalcy by the 2nd week of surgery without any medication or venesection. Patients with polycythemia must be screened for hyperparathyroidism. Early treatment of hyperparathyroidism by parathyroid removal will take care of polycythemia as well. Endoscopic parathyroidectomy is very feasible, safe, and well accepted by patients.