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Erschienen in: Medical Oncology 2/2008

01.06.2008 | Original Paper

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

verfasst von: M. Todorovic, B. Balint, N. Suvajdzic, M. Jevtic, M. Pavlovic, M. Petrovic, M. Krstic, V. Popovic, B. Ivanovic, I. Elezovic, R. Milenkovic, M. Colovic

Erschienen in: Medical Oncology | Ausgabe 2/2008

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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.
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Metadaten
Titel
Triple-way therapeutic approach for paraganglioma-dependent erythrocytosis: drugs and surgery plus “multi-manner” apheresis
verfasst von
M. Todorovic
B. Balint
N. Suvajdzic
M. Jevtic
M. Pavlovic
M. Petrovic
M. Krstic
V. Popovic
B. Ivanovic
I. Elezovic
R. Milenkovic
M. Colovic
Publikationsdatum
01.06.2008
Verlag
Humana Press Inc
Erschienen in
Medical Oncology / Ausgabe 2/2008
Print ISSN: 1357-0560
Elektronische ISSN: 1559-131X
DOI
https://doi.org/10.1007/s12032-007-9008-0

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