Abstract
Polycythemia associated with acromegaly is usually caused by the systemic manifestations of the disease, such as sleep-apnea or concomitant erythropoietin-secreting kidney tumors. The recognition of underlying pathologies requires a thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the disease, and receding with octreotide therapy. The medical history of 141 acromegalic patients followed by the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the literature evidence for similar cases. The diagnostic workflow and 2-years follow-up of a 43-years old acromegalic, polycythemic man with a history of past smoking, moderate hypertension, and mental retardation are described. The hematological parameters of our cohort was retrospectively compared with those of a healthy, age/gender-related control group as well. Therapy with octreotide LAR, 20 mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit level, hormonal setting, as well as pituitary tumor size and visual perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after 2 years of therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges of a healthy, age/sex- related control population. In conclusions, polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that must be considered during the diagnostic work-up of acromegalic patients presenting with such disorder.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Melmed S (2006) Medical progress: acromegaly. N Engl J Med 355:2558–2573
Colao A, Ferone D, Marzullo P, Lombardi G (2004) Systemic complications of acromegaly: epidemiology, pathogenesis, and management. Endocr Rev 25:102–152
Colao A, Ferone D, Marzullo P, Panza N, Pivonello R, Orio F Jr, Grande G, Bevilacqua N, Lombardi G (2002) Lymphocyte subset pattern in acromegaly. J Endocrinol Invest 25:125–128
Grellier P, Chanson P, Casadevall N, Abboud S, Schaison G (1996) Remission of polycythemia Vera after surgical cure of acromegaly. Ann Intern Med 124:495–496
Fauci AS et al (2008) Harrison’s principles of internal medicine/editors, Anthony S. Fauci… [et al.], 17th edn. McGraw-Hill Medical, New York
Cirmena G, Aliano S, Fugazza G, Bruzzone R, Garuti A, Bocciardi R, Bacigalupo A, Ravazzolo R, Ballestrero A, Sessarego M (2008) A bcr-jak2 fusion gene as the result of a t(9;22)(p24;q11) in a patient with acute myeloid leukemia. Cancer Genet Cytogenet 183:105–108
Piaggio G, Rosti V, Corselli M, Bertolotti F, Bergamaschi G, Pozzi S, Imperiale D, Chiavarina B, Bonetti E, Novara F, Sessarego M, Villani L, Garuti A, Massa M, Ghio R, Campanelli R, Bacigalupo A, Pecci A, Viarengo G, Zuffardi O, Frassoni F, Barosi G (2009) Endothelial colony-forming cells from patients with chronic myeloproliferative disorders lack the disease-specific molecular clonality marker. Blood 114:3127–3130
Wu H, Liu X, Jaenisch R, Lodish HF (1995) Generation of committed erythroid bfu-e and cfu-e progenitors does not require erythropoietin or the erythropoietin receptor. Cell 83:59–67
Melmed S, Colao A, Barkan A, Molitch M, Grossman AB, Kleinberg D, Clemmons D, Chanson P, Laws E, Schlechte J, Vance ML, Ho K, Giustina A (2009) Guidelines for acromegaly management: an update. J Clin Endocrinol Metab 94:1509–1517
Isidori AM, Giannetta E, Lenzi A (2008) Male hypogonadism. Pituitary 11:171–180
Daly AF, Petrossians P, Beckers A (2005) An overview of the epidemiology and genetics of acromegaly. J Endocrinol Invest 28:67–69
Johansson P (2006) Epidemiology of the myeloproliferative disorders polycythemia Vera and essential thrombocythemia. Semin Thromb Hemost 32:171–173
Tefferi A, Vardiman JW (2008) Classification and diagnosis of myeloproliferative neoplasms: the 2008 world health organization criteria and point-of-care diagnostic algorithms. Leukemia 22:14–22
Cats A, Dullaart RP, Kleibeuker JH, Kuipers F, Sluiter WJ, Hardonk MJ, de Vries EG (1996) Increased epithelial cell proliferation in the colon of patients with acromegaly. Cancer Res 56:523–526
Kelley KW, Arkins S, Minshall C, Liu Q, Dantzer R (1996) Growth hormone, growth factors and hematopoiesis. Horm Res 45:38–45
Garrett RW, Emerson SG (2008) The role of parathyroid hormone and insulin-like growth factors in hematopoietic niches: physiology and pharmacology. Mol Cell Endocrinol 288:6–10
Petri A, Ferraris M, Binotti M, Tardivo I, Bona G, Miniero R (2007) Growth hormone and erythropoiesis. Review of the literature. Minerva Pediatr 59:787–800
Bergamaschi S, Giavoli C, Ferrante E, Lania A, Rusconi R, Spada A, Beck-Peccoz P (2006) Growth hormone replacement therapy in growth hormone deficient children and adults: effects on hemochrome. J Endocrinol Invest 29:399–404
Argetsinger LS, Campbell GS, Yang X, Witthuhn BA, Silvennoinen O, Ihle JN, Carter-Su C (1993) Identification of jak2 as a growth hormone receptor-associated tyrosine kinase. Cell 74:237–244
Tucci A, Bonadonna S, Cattaneo C, Ungari M, Giustina A, Guiseppe R (2003) Transformation of a mgus to overt multiple myeloma: the possible role of a pituitary macroadenoma secreting high levels of insulin-like growth factor 1 (igf-1). Leuk Lymphoma 44:543–545
Oomen SP, van Hennik PB, Antonissen C, Lichtenauer-Kaligis EG, Hofland LJ, Lamberts SW, Lowenberg B, Touw IP (2002) Somatostatin is a selective chemoattractant for primitive (cd34(+)) hematopoietic progenitor cells. Exp Hematol 30:116–125
Acknowledgments
This study was partially supported by grant from Italian Minister of Instruction, University and Research [PRIN 2008 n. 2008LFK7J5_004]. G.Z.’s Ph.D. Fellowship was sponsored by the University of Genoa, Genoa, Italy.
Conflict of interest
The Authors report no conflict of interest in the present manuscript.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Zoppoli, G., Bianchi, F., Bruzzone, A. et al. Polycythemia as rare secondary direct manifestation of acromegaly: management and single-centre epidemiological data. Pituitary 15, 209–214 (2012). https://doi.org/10.1007/s11102-011-0311-6
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-011-0311-6