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• W2034645650 abstract "A 59-YEAR-old white man presented with acute back pain after lifting the motor of his boat and was treated initially with physical therapy and analgesics. Radiographs of the spine showed generalized osteopenia and four compression fractures and he was referred to our unit for investigation and treatment of osteoporosis. His past medical history was unremarkable; he did not smoke, he drank alcohol socially, and he was not using any medication known to influence bone metabolism. There was no family history of osteoporosis. On examination, his height was 179 cm (this reportedly was 182 cm before), his weight was 69 kg, and his blood pressure was normal. The rest of the examination revealed no abnormal findings. Laboratory investigations on admission showed an erythrocyte sedimentation rate (ESR) of 2 mm/first hour; hemoglobin, 15 mg/dl; white blood cell count (WBC), 9900/cm3 with a normal differential; and normal platelet count. Renal, liver, thyroid, adrenal, and gonadal function tests and serum protein electrophoresis were normal. Biochemical parameters pertinent to calcium and bone metabolism were all within the reference range as follows: serum calcium corrected for albumin binding, 2.32 mM (normal, 2.20-2.55 mM); phosphate, 0.90 mM (normal, 0.80-1.45 mM); alkaline phosphatase, 106 U/liter (normal, 40-120 U/liter); osteocalcin, 3.1 μg/liter (normal, <5.5 μg/liter); parathyroid hormone (PTH), 2.1 pM/liter (normal, 1.0-6.5 pM/liter); 25-hydroxyvitamin D, 94 nM (normal, 30-120 nM); urinary calcium excretion, 4.5 mmol/day (normal, 2.5-7.5 mmol/day); and hydroxyproline/creatinine ratio in urine, 14.8 μmol/mmol (normal, <30 μmol/mmol). Bone mineral density (BMD) of the lumbar spine measured by dual-energy X-ray absorptiometry (QDR 1000; Hologic, Inc., Waltham, MA, USA) was very low: 0.67 g/cm2, T score was −4.1, and Z score was −3.8; BMD of the femoral neck was 0.70 g/cm2, T score was −2.5, and Z score was −0.9. The patient was treated with 400 IU/day of cholecalciferol and 500 mg/day of calcium. Ten months later, he had a new episode of acute back pain. He did not report any episodes of flushing, tachycardia, or diarrhea. On examination, there was marked tenderness of thoracic vertebrae, increased kyphosis, and limited mobility of the spine. His height had decreased by nearly 3 cm within the last 3 months to 176.4 cm. The rest of the examination was again unremarkable. There were no skin abnormalities, in particular, no urticaria pigmentosa, lymphadenopathy, or hepatosplenomegaly. A bone marrow biopsy showed marked infiltration by mast cells of an otherwise normal bone marrow, with no evidence of a mineralization defect. This increase in mast cells was not caused by increased rate of bone turnover or secondary hyperparathyroidism, biochemically assessed. The mast cells were active as evidenced by the high urinary excretion of the histamine metabolites N-methyl histamine and N-methyl imidazole acetic acid, which were two to three times above the upper limit of the normal range. Radiographs of the spine showed a dramatic progression of the fractures and nearly all vertebrae were deformed (Fig. 1). Only L1 was intact and this was used for the measurement of spine BMD during the whole period of observation. Spine BMD had decreased by 16% within a year and BMD of the total hip and the femoral neck had decreased by 4.1% and 1.4%, respectively. The magnetic resonance imaging (MRI) of the spine showed, in addition, no signs of bone marrow pathology. T1-weighted image of an MRI of the lumbar spine after 1 year of treatment with calcium and vitamin D. The patient was treated with intravenous pamidronate (75 mg) over 5 days followed by oral pamidronate 150 mg/day for 7 consecutive years (pamidronate was prepared by the Hospital Pharmacy of the Leiden University Medical Center). As expected, treatment decreased the rate of bone turnover and was associated with a progressive increase in spine BMD by 65% after 7 years (Fig. 2); the increase in L1-L4 was 28.6%. Urinary excretion of N-methyl histamine remained elevated throughout the treatment period (Fig. 2). During the 7 years of follow-up, the patient had no further fractures (vertebral or peripheral), his height did not change (176.6 cm after 7 years), and he developed no signs or symptoms of systemic mastocytosis. (A) Urinary excretion of N-methyl histamine during 8 years of follow-up; line depicts the upper limit of the normal range; arrow indicates start of pamidronate therapy. (B) Corresponding changes of lumbar spine BMD. This patient, with no risk factors for osteoporosis, presented with severe, progressive osteoporosis of the spine as the sole clinical manifestation of mastocytosis confined to the bone marrow. The diagnosis of mastocytosis was established by bone marrow histology and the increased excretion of histamine metabolites in urine. The association of osteoporosis with bone marrow mastocytosis has been reported previously1-4 but the pathogenesis of bone destruction remains unclear and may be related to the multiple factors that are produced by mast cells and can affect bone metabolism such as, for example, histamine, heparin, proteases, and cytokines.5-8 In our series, 9% of men with idiopathic osteoporosis had infiltrations of the bone marrow with mast cells and increased urinary histamine excretion (C. Brumsen, S. Papapoulos, P. Kluin, E. Lentjes, N. Hamdy, unpublished observation, 2001). In patients with progressive osteoporosis and no evident underlying cause, the possibility of bone marrow mastocytosis should be considered and a biopsy should be taken. Therapy with intravenous pamidronate followed by oral pamidronate, with a dose used in the treatment of postmenopausal osteoporosis, induced a dramatic increase in spine BMD and prevented further fractures over 7 years of continuous administration. The increase in BMD partly may be caused by accompanying bone sclerosis that has been described in some patients with systemic mastocytosis. Previous studies have reported effective treatment of patients with systemic mastocytosis and osteoporosis with bisphosphonates.9, 10 Moreover, in our patient, skeletal protection was obtained in the presence of active mast cell disease as evidenced by the constantly high urinary excretion of histamine metabolites. Thus, pamidronate, in spite of the lack of an effect on mast cells, was effective by suppressing osteoclastic resorption, presumably the consequence of increased mast cell activity. However, during the period of observation, the patient did not develop any signs of systemic mastocytosis, suggesting that bone marrow mastocytosis may be a separate form of the disease. This is the longest reported follow-up of a patient with bone marrow mastocytosis and osteoporosis with concomitant assessment of the course of both disorders illustrating, in addition, the efficacy of bisphosphonate therapy in protecting skeletal integrity despite persistent mast cell activity." @default.
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• W2034645650 title "Osteoporosis and Bone Marrow Mastocytosis: Dissociation of Skeletal Responses and Mast Cell Activity During Long-Term Bisphosphonate Therapy" @default.
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