ISSN: 1300-7777 E-ISSN: 1308-5263
Osteoporosis and Osteopathy Markers in Patients with Mastocytosis [Turk J Hematol]
Turk J Hematol. 2015; 32(1): 43-50 | DOI: 10.4274/tjh.2013.0170  

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Nilüfer Alpay Kanıtez1, Burak Erer1, Öner Doğan2, Nesimi Büyükbabanı2, Can Baykal3, Dilşad Sindel4, Refik Tanakol5, Akif Selim Yavuz6
1İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İstanbul, Turkey
2İstanbul University İstanbul Faculty of Medicine, Division of Pathology, İstanbul, Turkey
3İstanbul University İstanbul Faculty of Medicine, Division of Dermatology, İstanbul, Turkey
4İstanbul University İstanbul Faculty of Medicine, Division of Physical Treatment and Rehabilitation, İstanbul, Turkey
5İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology, İstanbul, Turkey
6İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Turkey

OBJECTIVE: Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease.
METHODS: Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were included in this study. We investigated the value of quantitative ultrasound (QUS) of the calcaneus in the assessment of BMD in SM patients, as well as BMD of the lumbar spine (L1-L4), femoral neck, and distal radius using dual energy x-ray absorptiometry (DXA) and plasma tryptase levels, biochemical markers of bone turnover.
RESULTS: At lumbar spine L1-L4, the femoral neck, and the distal radius or as calcaneus stiffness, 12 of 17 patients had T-scores of less than -1 at least at 1 site, reflecting osteopenia. Three of 17 patients had T-scores showing osteoporosis (T-score <-2.5).
There was no relationship between DXA and bone lesion severity. We also found a significant positive correlation between tryptase levels and disease severity, as well as between disease severity and pyridinoline (p<0.01 by Spearman’s test).
CONCLUSION: DXA and calcaneal QUS may not be appropriate techniques to assess bone involvement in SM patients because of the effects of osteosclerosis. This study further shows that the osteoclastic marker pyridinoline is helpful in patients with severe disease activity and sclerotic bone lesions to show bone demineralization.

Keywords: Mastocytosis, Bone mineral density, Pyridinoline, Bone turnover, Osteopenia


Sistemik Mastositozda Osteoporoz ve Osteopati Belirteçleri

Nilüfer Alpay Kanıtez1, Burak Erer1, Öner Doğan2, Nesimi Büyükbabanı2, Can Baykal3, Dilşad Sindel4, Refik Tanakol5, Akif Selim Yavuz6
1İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İstanbul, Turkey
2İstanbul University İstanbul Faculty of Medicine, Division of Pathology, İstanbul, Turkey
3İstanbul University İstanbul Faculty of Medicine, Division of Dermatology, İstanbul, Turkey
4İstanbul University İstanbul Faculty of Medicine, Division of Physical Treatment and Rehabilitation, İstanbul, Turkey
5İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology, İstanbul, Turkey
6İstanbul University İstanbul Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Turkey

AMAÇ: Sistemik mastositozlu (SM) hastalarda osteoporoz, osteoskleroz ve litik kemik lezyonları görülebilir. Bu çalışmada nadir bir hastalık olan SM’de, kemik yoğunluk ölçümü (BMD), serumda kemik ‘turnover’ belirteçleri ve triptaz düzeyi araştırılmıştır.
YÖNTEMLER: Çalışmaya 5’i kadın, 12’si erkek olmak üzere median yaşları 33 (20-64) olan toplam 17 hasta dahil edilmiştir. Hastaların BMD’leri lomber vertebra (L1-L4), femur boynu ve distal radiustan dual enerji x-ray absorbitesi (DXA) yöntemi, kalkaneustan ise kantitatif ultrasonografi (USG) yöntemi kullanılarak ayrı ayrı hesaplanmıştır. Serum triptaz düzeyleri
ile biyokimyasal kemik ‘turnover’ belirteçlerileri arasındaki ilişki araştırılmıştır.
BULGULAR: Hastaların 12 tanesinde osteopeni (incelenen kemik alanlarının en az birinde T skoru <-1), 3 tanesinde ise osteoporoz (T skoru <-2,5) saptanmıştır. Kemik lezyonlarının şiddeti ile DXA sonuçları arasında korelasyon gösterilememiştir. Triptaz ve pridinolin seviyeleri ile hastalık şiddeti arasında pozitif korelasyon bulunmuştur (p<0,01).
SONUÇ: Osteosklerotik kemik lezyonları sebebiyle SM’li hastalarda kemik tutulumu değerlendirmesi için DXA ve kalkaneus USG uygun teknikler değildir. Osteoklastik aktivite belirteçleri olan pridinolin, şiddetli hastalık aktivitesi ve kemik sklerozlarını değerlendirmede faydalıdır.

Anahtar Kelimeler: Mastositoz, Kemik yoğunluk ölçümü, Pridinolin, Kemik turnoverı, Osteopeni


Nilüfer Alpay Kanıtez, Burak Erer, Öner Doğan, Nesimi Büyükbabanı, Can Baykal, Dilşad Sindel, Refik Tanakol, Akif Selim Yavuz. Osteoporosis and Osteopathy Markers in Patients with Mastocytosis. Turk J Hematol. 2015; 32(1): 43-50

Corresponding Author: Nilüfer Alpay Kanıtez, Türkiye


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