Endocrine Abstracts

Introduction: Acromegaly and Waldenstrom’s macroglobulinaemia are two rare diseases (about 3–4 and 10 cases/million per year respectively), the association between them is even more infrequent, one case being reported in the available literature.

Case report: We present the case of a 74-years-old female (I.I.) suffering with both diseases. In 1997 acromegaly diagnosed by dysmorphic syndrome, than in 2004 hormonal/imagistical investigations (GH at baseline: 2.5 ng/ml, suppressed GH with OGTT: 2.3–2.9–11.6–7.5 ng/ml; 12/15 mm pituitary adenoma on MRI) confirmed the diagnosis. She refused any therapeutical approaches. Cardiovascular diseases (hypertension, left ventricular hypertrophy, major left bundle branch block, left ventricle failure NYHA III) and osteoarticular involvement (osteoporosis with right hip fracture and multiple vertebral fractures) have slowly progressed in time. In 2008 Waldenström’s disease was diagnosed and chemotherapy was administered (cyclophosphamide, vincristine, corticosteroids), the outcome being favourable. Affirmatively the dysmorphic syndrome has not aggravated during the last 7 years, but in May 2011 a sudden severe aggravation of the general status, weight loss, tongue-mandibular hypertrophy, malocclusion, swallowing and respiratory disturbances developed within 6 weeks. Surprisingly, hormonal results (GH at baseline: 3.3 ng/ml, GH after OGTT: 1.58–1.58–1.76–1.24 ng/ml; PRL: 72.3 ng/ml), IGF1: 114 ng/ml (101–267) and pituitary CT (11 mm adenoma) did not showed the reactivation of acromegaly. In August–September 2011 a severe gastroparesis, malabsorption syndrome and cachexia developed.

Conclusion: We present a particular course of acromegaly: a very slow progression without any specific treatment, than a rapid aggravation of facial dysmorphic signs. The favourable laboratory/imagistic evolution of acromegaly, confirmed by the involution of adenoma on CT, the slightly elevated GH and normal IGF1, may be attributed to a partial apoplexy in the adenoma or eventually to the medications used for the haematological disease. The discordance between evolutive clinical signs and non-evolutive laboratory/imagistic results needs supplementary investigations (directed towards a local viscerocranial/buccal process).

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.