Triple-way therapeutic approach for paraganglioma-dependent erythrocytosis: drugs and surgery plus “multi-manner” apheresis

Abstract

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.

Rapid Cytoreduction by Plateletapheresis in the Treatment of Thrombocythemia

“…TAph treatment should be done regularly or always combined with "immunomodulatory" (e.g., immunochemotherapy) and other palliative (drug) therapies. Based upon the criterion of what is the removed blood constituent-with specific "pathogenic factors or substrate"-it is possible to classify the TAph procedures into TPE and therapeutic cytapheresis (TC) [5][6][7][8][9][10][11][12][13][14][15].…”

“…Concisely, the goal of our original and innovative "multi-manner" TAph is to offer the possibility of rapid returning from a "life-threatening" (irreversible) emergency-altering simultaneously more than one blood constituent(s)-to the "district" of unbalanced, but reversible clinical condition, with a possibility for recovery of patient's hematological and overall clinical homeostasis [13][14][15].…”

Apheretic “rescue-protocol” designed for treatment of CLL associated life-threatening hemolytic crisis

“…The basic goal of "multi-manner" apheresis (MMAph) is to create an option for rapid return from some lifethreatening irreversible emergency to the reversible (although yet imbalanced) clinical condition with potentially positive clinical outcome [3][4][5]. MMAph operates by correcting of multiple blood abnormalities (structural or functional) at the same time.…”