Shulkin B L, Shapiro B, Sisson J C
Division of Nuclear Medicine, University of Michigan Medical Center, Ann Arbor 48109-0028.
Am J Med. 1987 Oct;83(4):773-6. doi: 10.1016/0002-9343(87)90913-2.
Polycythemia is rarely associated with pheochromocytoma. A patient with a 22-year history of malignant pheochromocytoma is presented in whom major complications developed as a result of long-standing polycythemia, apparently due to secretion of erythropoietin by the tumors. Despite attempts to reduce tumor burden by surgery, chemotherapy, and large doses of I-131-metaiodobenzylguanidine, polycythemia persisted. Extensive venous thrombosis developed requiring hospitalization and anticoagulation. Thus, polycythemia itself may be a cause of major morbidity in patients with pheochromocytoma, and prophylactic measures may be warranted. Review of the 130 patients with benign and malignant pheochromocytoma studied since the introduction of I-131-metaiodobenzylguanidine in 1980 revealed another six patients with hematocrits over 50 but only one had a hematocrit greater than 55 and required regular phlebotomy. In contrast, anemia (hematocrit less than 35) due to variety of causes was present in 18 cases.
真性红细胞增多症很少与嗜铬细胞瘤相关。本文报告了一例有22年恶性嗜铬细胞瘤病史的患者，该患者因长期真性红细胞增多症出现了严重并发症，显然是由于肿瘤分泌促红细胞生成素所致。尽管尝试通过手术、化疗和大剂量的I-131-间碘苄胍来减轻肿瘤负荷，但真性红细胞增多症仍持续存在。患者出现广泛静脉血栓形成，需要住院并进行抗凝治疗。因此，真性红细胞增多症本身可能是嗜铬细胞瘤患者严重发病的一个原因，可能需要采取预防措施。回顾自1980年引入I-131-间碘苄胍以来研究的130例良性和恶性嗜铬细胞瘤患者，发现另外6例患者的血细胞比容超过50，但只有1例患者的血细胞比容大于55且需要定期放血。相比之下，18例患者因各种原因出现贫血（血细胞比容小于35）。
