Osteoporose bei systemischer Mastozytose: Eine Kohortenstude mit 43 Patienten
Osteoporosis caused by systemic mastocytosis: A retrospective overview involving 43 consecutive patients.
by Niels Schmidt
Date of Examination:2022-11-23
Date of issue:2022-11-16
Advisor:Prof. Dr. Heide Siggelkow
Referee:PD Dr. Daniel Hoffmann
Referee:Prof. Dr. Margarete Schön
Persistent Address:
http://dx.doi.org/10.53846/goediss-9542
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Abstract
English
Introduction Mastocytosis is a group of rare diseases with a clonal proliferation of mast cells in one or more organs. Systemic mastocytosis has been described as the second most frequent cause of secondary osteoporosis (after steroid-induced loss of bone mass) at 1.25%. The purpose of this study was to review measures of diagnosis, clinical symptoms, organ involvement and long-term follow-up after therapy with bisphosphonates. Materials and Methods This retrospective, monocentric observational study was conducted in an osteological center during the period between 2005 - 2015. From a total of 7,722 osteoporosis patients treated in hospital, 1,374 were selected for a bone biopsy according to clearly defined criteria (severity of compression and wedge fractures during a short period of time causing unusual pain). The diagnosis of systemic mastocytosis was made by an experienced osteopathologist. All patients with mastocytosis were treated with 5 mg zoledronate per year. 13 of the patients (8 men and 5 women) with systemic mastocytosis were tracked for up to 7 years. Outcome and Results In the period 2005-2015, 7,722 patients with osteoporosis were treated. A bone biopsy was performed in 1,374 patients, (n= 415 m= male, n=959 w= female). Mastocytosis was diagnosed in 43 (3.1%) with bone biopsy (m:24 (5.8%); (w:19 (2%)). Patients with mastocytosis were 54.4 ± 12.3 (m) years and 57.4 ± 11.75 (w) years, respectively. Urticaria pigmentosa was present in 45.83% (m, n=11) and 15.79% (w, n=3) patients, respectively. Bone density (DEXA-T score) at the lumbar spine was -2.59 ± 1.08 (m) and -2.65 ± 1.16 (w), respectively. Total bone density (T score) at the hip was -1.36 ± 0.76 (m) and -1.83 ± 1.13 (w), respectively. In all cases, vertebral fractures were present at the initial diagnosis of mastocytosis: 4.3 ± 3.5 (m) and 4.6 ± 3.8 (w), respectively. Regarding the bone remodeling markers, elevated deoxypyridinoline/crea secretion in urine (NW 8.4-19.7) 23.4 ± 15.6 µg/g (m) and 42.7 ± 36.9 µg/g (w) were found. The 13 patients (m: n=8; w: n=5) who were observed in the long-term course developed the number of follow-up fractures shown under therapy (number of patients): Men: month 22: 0.5 ± 0.9 (n=8), month 33: 0 (n=6), month 51: 0.5 ± 0.8 (n=6), month 69: 0 (n=5) Women: Month 28: 0.2 ± 0.45 (n=5), month 43: 0 (n=3), month 67: 0 (n=3), Month 81: 0.33 ± 0.58 (n=3). Discussion Systemic mastocytosis is a rare cause of osteoporosis. Relative to the total collective of 7,722 patients with osteoporosis it was very rare in the collective described above (0.5% of cases). In the patients who fulfilled the criteria for a bone punch (therapy refractory or rapidly progressive course, premenopausal women, men < 60 years, inadequate response to drug therapy, particularly painful course, urticaria pigmentosa), mastocytosis was present in 3.1% (m:5.8%). It is possible that the determination of tryptase in serum may facilitate the indication for bone biopsy in the future. With a therapy with 5 mg zoledronate, the fracture progression could be stopped in the long-term course. Thus, therapy with 5 mg zoledronic acid seems to be a suitable treatment of severe osteoporosis in mastocytosis.
Keywords: osteoporosis; mastocytosis
Schlagwörter: systemische Mastozytose