Todorovic M, Balint B, Suvajdzic N, Jevtic M, Pavlovic M, Petrovic M, Krstic M, Popovic V, Ivanovic B, Elezovic I, Milenkovic R, Colovic M
Institute of Hematology, Clinical Center of Serbia, Belgrade, Serbia.
Med Oncol. 2008;25(2):148-53. doi: 10.1007/s12032-007-9008-0. Epub 2007 Sep 25.
Paragangliomas are tumors arising from the extra-adrenal paragangliar neural crest cells. The sympathoadrenal neuroendocrine system consists of extra-adrenal paragangliar cellular layer along the paravertebral and para-aortic axis, and the adrenal medullae. Paraganglioma should be included in the differential diagnosis of secondary erythrocytosis due to its possible ectopic erythropoietin (EPO) secretion. Thus, in this report we present a 24-year-old female patient with onset of unregulated ectopic EPO secretion, and consecutive erythrocytosis followed by hypertension, secondary to paraganglioma of multifocal retroperitoneal localization. Clinical, laboratory, and radiological investigations confirmed both an elevated EPO level and the presence of multiple paraganglioma. This paraneoplastic-mediated medical condition with high risk of cellular hyperviscosity syndrome (CHVS) requires prompt diagnosis and rapid therapeutic interventions. Initially, simple phlebotomy procedures were used; following that, tumors were surgically removed. In the course of the disease, this tumor relapsed, and urgent apheresis, as a treatment of life-threatening state, was used. The therapy performed resulted in a rapid blood viscosity depletion and a significant (P < 0.01) serum EPO reduction, as well as the general clinical benefit. Therefore, we conclude that the use of our own "multi-manner" apheresis (erythrocythapheresis plus plasma exchange), for long-time interval (until further causative therapy), effectively cross-bridged the possible hazards of EPO-dependent CHVS.
副神经节瘤是起源于肾上腺外副神经节神经嵴细胞的肿瘤。交感肾上腺神经内分泌系统由沿椎旁和主动脉旁轴的肾上腺外副神经节细胞层以及肾上腺髓质组成。由于副神经节瘤可能异位分泌促红细胞生成素（EPO），因此在继发性红细胞增多症的鉴别诊断中应考虑到它。在此报告中，我们介绍了一名24岁女性患者，该患者因多灶性腹膜后定位的副神经节瘤继发出现不受调节的异位EPO分泌、连续性红细胞增多症，随后出现高血压。临床、实验室及影像学检查证实EPO水平升高且存在多发副神经节瘤。这种具有细胞高黏滞综合征（CHVS）高风险的副肿瘤介导的病症需要迅速诊断和快速治疗干预。最初采用简单的放血程序；随后，手术切除肿瘤。在疾病过程中，该肿瘤复发，于是采用紧急血液分离术作为对危及生命状态的治疗方法。所实施的治疗导致血液黏度迅速降低、血清EPO显著降低（P < 0.01），并带来总体临床获益。因此，我们得出结论，使用我们自己的“多方式”血液分离术（红细胞单采术加血浆置换），在较长时间间隔内（直至进一步的病因治疗），有效地跨越了EPO依赖性CHVS的可能风险。
