Polycythemia as rare secondary direct manifestation of acromegaly: management and single-centre epidemiological data Gabriele Zoppoli • Federico Bianchi • Andrea Bruzzone • Alessandro Calvia • Caterina Oneto • Caterina Passalia • Enrico Balleari • Davide Bedognetti • Elena Ponomareva • Elena Nazzari • Lara Castelletti • Lucio Castellan • Francesco Minuto • Riccardo Ghio • Diego Ferone Published online: 19 April 2011 Springer Science+Business Media, LLC 2011 Abstract Polycythemiassociated with acromegaly is usually caused by the systemic manifestations of the dis- ease,such as sleep-apnea or concomitant erythropoietin- secreting kidney tumors. The recognition of underlying pathologies requiresa thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the dis- ease,and receding with octreotide therapy. The medical historyof 141 acromegalicpatientsfollowedby the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the liter- ature evidence for similar cases. The diagnostic workflow and 2-yearsfollow-up ofa 43-yearsold acromegalic, polycythemic man with a history of past smoking, mod- erate hypertension, and mental retardation are described. The hematological parameters of our cohortwasretro- spectively compared with those of a healthy, age/gender- related control group aswell. Therapy with octreotide LAR, 20 mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit leve hormonal setting, as well as pituitary tumor size and visua perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after2 yearsof therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges o a healthy, age/sex- related control population. In conclu- sions,polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that mustbe considered during thediagnosticwork-up ofacromegalic patients presenting with such disorder. Keywords Acromegaly Polycythemia Polycythemia Vera GH IGF-I Introduction Acromegaly is a systemic endocrine disorder due, in most cases, to GH hypersecretion by pituitary adenomas in adu [1]. Concomitant excess of GH and IGF-I produce its typica disfiguring physical manifestations, as wellas significant co-morbidities (diabetes mellitus, hypertension, polyarthri tis, sleep apnea and specific cardiovascular alterations). When inadequately treated, acromegaly impairs patients’ quality of life and leads to a 2–5 fold increase in mortality G. Zoppoli A. Bruzzone A. Calvia C. Oneto C. Passalia E. Balleari R.Ghio Department of Internal Medicine, University of Genoa, Genoa, Italy G. Zoppoli Laboratory of Molecular Pharmacology, CCR, NCI, National Institutes of Health, Bethesda, MD, USA D. Bedognetti Infectious Diseases Section, DTM, CC, National Institutes of Health, Bethesda, MD, USA F. Bianchi E.Nazzari F.Minuto D. Ferone (&) Department of Endocrine and Medical Sciences and Center of Excellence for Biomedical Research, University of Genoa, Viale Benedetto XV, 6, 16132 Genoa, Italy e-mail: ferone@unige.it D. Bedognetti National Cancer Research Institute, Genoa, Italy E. Ponomareva Department of Hematology and Stem Cell Centre, Genoa, Italy L. Castelletti L.Castellan Neuroradiology Unit, San Martino Hospital, Genoa, Italy 123 Pituitary (2012) 15:209–214 DOI 10.1007/s11102-011-0311-6