Objectives: We report on a case of a 32-year-old patient with urticaria pigmentosa, the cutaneous form of mastocytosis, since the age of 13. A bone marrow biopsy revealed systemic mastocytosis disease. In 2005, after a minor trauma, the patient had an X-ray of the spine which showed a new fracture of the thoracic vertebrae 34. Further DXA examination of the bone mineral density showed osteoporosis (T-Score −2.8 lumbar vertebrae; right/left femur −1.0/−0.9). As interferon alpha is effective in mastocytosis and the pegylated form of the drug is better tolerable, the clinical response of the pegylated interferon alpha was analyzed.
Methods: A treatment with pegylated interferon alpha 2a 90 μg s.c. once a week for 6 months was initiated.
Results: No further fractures occurred and the bone mineral density increased (after 6 months T-Score 2.2 lumbar vertebrae; right/left femur −0.9/−0.7). Side effects of the treatment were minor compared to the benefit.
Conclusion: Low-dose pegylated interferon alpha therapy for 6 months in a young man with severe secondary osteoporosis due to systemic mastocytosis improved the bone mass density as well as clinical parameters. The drug appears effective in treating mastocytosis with osteoporosis and should be examined in more patients.
03 - 07 May 2008
European Society of Endocrinology