Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Alpay Kan�tez, Nil�fer; Erer, Burak; Do�an, �ner; B�y�kbaban�, Nesimi; Baykal, Can; Sindel, Dil�ad; Tanakol, Refik; Yavuz, Akif Selim

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

Nil�fer Alpay Kan�tez¹, Burak Erer¹, �ner Do�an², Nesimi B�y�kbaban�², Can Baykal³, Dil�ad Sindel⁴, Refik Tanakol⁵, Akif Selim Yavuz⁶
¹�stanbul University �stanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, �stanbul, Turkey
²�stanbul University �stanbul Faculty of Medicine, Division of Pathology, �stanbul, Turkey
³�stanbul University �stanbul Faculty of Medicine, Division of Dermatology, �stanbul, Turkey
⁴�stanbul University �stanbul Faculty of Medicine, Division of Physical Treatment and Rehabilitation, �stanbul, Turkey
⁵�stanbul University �stanbul Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology, �stanbul, Turkey
⁶�stanbul University �stanbul Faculty of Medicine, Department of Internal Medicine, Division of Hematology, �stanbul, 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 Belirte�leri

AMA�: Sistemik mastositozlu (SM) hastalarda osteoporoz, osteoskleroz ve litik kemik lezyonlar� g�r�lebilir. Bu �al��mada nadir bir hastal�k olan SM�de, kemik yo�unluk �l��m� (BMD), serumda kemik �turnover� belirte�leri ve triptaz d�zeyi ara�t�r�lm��t�r.
Y�NTEMLER: �al��maya 5�i kad�n, 12�si erkek olmak �zere median ya�lar� 33 (20-64) olan toplam 17 hasta dahil edilmi�tir. Hastalar�n BMD�leri lomber vertebra (L1-L4), femur boynu ve distal radiustan dual enerji x-ray absorbitesi (DXA) y�ntemi, kalkaneustan ise kantitatif ultrasonografi (USG) y�ntemi kullan�larak ayr� ayr� hesaplanm��t�r. Serum triptaz d�zeyleri
ile biyokimyasal kemik �turnover� belirte�lerileri aras�ndaki ili�ki ara�t�r�lm��t�r.
BULGULAR: Hastalar�n 12 tanesinde osteopeni (incelenen kemik alanlar�n�n en az birinde T skoru <-1), 3 tanesinde ise osteoporoz (T skoru <-2,5) saptanm��t�r. Kemik lezyonlar�n�n �iddeti ile DXA sonu�lar� aras�nda korelasyon g�sterilememi�tir. Triptaz ve pridinolin seviyeleri ile hastal�k �iddeti aras�nda pozitif korelasyon bulunmu�tur (p<0,01).
SONU�: Osteosklerotik kemik lezyonlar� sebebiyle SM�li hastalarda kemik tutulumu de�erlendirmesi i�in DXA ve kalkaneus USG uygun teknikler de�ildir. Osteoklastik aktivite belirte�leri olan pridinolin, �iddetli hastal�k aktivitesi ve kemik sklerozlar�n� de�erlendirmede faydal�d�r.

Anahtar Kelimeler: Mastositoz, Kemik yo�unluk �l��m�, Pridinolin, Kemik turnover�, Osteopeni

Nil�fer Alpay Kan�tez, Burak Erer, �ner Do�an, Nesimi B�y�kbaban�, Can Baykal, Dil�ad Sindel, Refik Tanakol, Akif Selim Yavuz. Osteoporosis and Osteopathy Markers in Patients with Mastocytosis. Turk J Hematol. 2015; 32(1): 43-50

Corresponding Author: Nil�fer Alpay Kan�tez, T�rkiye

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