| dc.description.abstracteng | Introduction<br>
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.</br><br>
Materials and Methods<br>
This retrospective, monocentric observational study was conducted in an
osteological center during
the period between 2005 - 2015. 1,374 patient
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.<br/><br>
Outcome and Results<br>
In the period 2005-2015,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).</br><br>
Discussion<br>
Systemic mastocytosis is a rare cause of osteoporosis. 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. | de |