A case of systemic mastocytosis associated with severe osteoporosis and pathologic fractures

Pusl, Thomas; Kenngott, Silke; Bartl, R.; Baur, Andrea; Ludolph-Hauser, Dagmar; Juengst, Dieter

doi:10.1016/j.ejim.2004.11.003

Cited by 6 publications

(4 citation statements)

References 6 publications

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“…Both osteosclerotic and osteolytic lesions can be shown on radiographs, especially in severe disease. Although there were no findings of fracture in the radiographies of our patients, pathological fractures may be seen in mastocytosis patients [ 18 , 19 ].…”

Section: Discussionmentioning

confidence: 78%

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Kanıtez¹,

Erer²,

Dogan³

et al. 2015

Tjh

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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. Materials and 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.

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Section: Discussionmentioning

confidence: 78%

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Kanıtez¹,

Erer²,

Dogan³

et al. 2015

Tjh

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“…We found the lowest BMD levels in patients with mildly and moderately elevated mast cell mass, corresponding to BST levels between approximately 30 and 100 ng/ml. In a recent paper, a patient with secondary osteoporosis due to mastocytosis was described who had a BST value of 50.9 ng/ml [20]. The patient excluded from our study because of previous treatment with anti-osteoporotic medication also suffered from secondary osteoporosis due to biopsy proven systemic mastocytosis.…”

Section: Discussionmentioning

confidence: 96%

Bone Mineral Content in Patients with Anaphylactic Reactions, Signs of Mastocytosis and Elevated Basal Serum Tryptase Levels

Bucher¹,

Uebelhart²,

Wüthrich³

et al. 2010

TOALLJ

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Introduction: To examine the relationship between elevated basal serum tryptase levels (BST), a marker of total mast cell mass, and bone mineral density (BMD) in patients with anaphylactic reactions and signs of mastocytosis. Methods: Retrospective evaluation of patient charts at an allergy unit. Patients with BST levels above 20 ng/ml were eligible if clinical and follow-up data and results of dual X-ray absorptiometry (DXA) were available. Patients with previous use of anti-osteoporotic medications and with osteoporosis not caused by mastocytosis were excluded. Spearman's rank correlation, Mann-Whitney test and receiver operating characteristic curve (ROC) was used for analysis. Results: 24 patients were included. The main presenting symptom (17 of 24 patients) was anaphylactic reactions to insect stings. BST levels ranged between 21 and 158 ng/ml (median 48 ng/ml). Study participants with Z-score values below -1.0 had a median BST level of 46 ng/ml, the patients with Z-score values above or equal to -1.0 had a median BST level of 27 ng/ml. ROC analysis of the patient group with BST values between 30 and 100 ng/ml revealed a best cut-off value of BST to detect a low BMD when BST level would be at least 27 ng/ml resulting in a sensitivity of 92% and a specificity of 70%. Conclusion: Patients with moderately elevated BST levels seem to be at increased risk for low BMD.

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“…Recientemente, Brumsen et al han publicado un artículo en que concluyen que el 9% de los casos de osteoporosis en hombres son secundarios a mastocitosis. El diagnóstico definitivo de muchas de las "osteoporosis idiopáticas" en varones podría, por tanto, conseguirse mediante un estudio anatomo-patológico de la médu-la ósea o incluso midiendo los niveles del metabolito N-metilhistamina en orina de 24 horas (21,22).…”

Section: Complementariaunclassified

Mastocitosis sistémica: Revisión sistemática

Molina‐Garrido

Mora

Ponce

et al. 2008

An. Med. Interna (Madrid)

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INTRODUCCIÓNLa mastocitosis sistémica (MS) es una enfermedad clonal de los progenitores mastocíticos de la médula ósea. El cuadro clínico en la MS varía desde una forma asintomática (indolente) a una forma altamente agresiva con una supervivencia muy corta (leucemia de mastocitos) (1).En 1869, Nettleship describió la urticaria pigmentosa (UP) y en 1887, Unna describió un amento en el número de mastocitos en la UP. Fue ya en 1949, cuando Ellis describió una enfermedad sistémica asociada a hiperplasia de mastocitos.La mayoría de los pacientes afectos son adultos, aunque la enfermedad puede ocurrir a cualquier edad. En función del momento de aparición de los síntomas, en especial, de las [0212-7199 (2008) RESUMENLa mastocitosis es una enfermedad definida por un crecimiento anormal y por la acumulación de mastocitos. En la clasificación de consenso de la OMS del 2001, se distinguió entre procesos bien limitados a piel (mastocitosis cutánea) o bien acumulados a nivel de otros tejidos: médula ósea y/u otros órganos extracutáneos (mastocitosis sistémica) como huesos, hígado, bazo o ganglios linfáticos (70% afectación ósea, con patrón osteolítico u osteoblástico, seguida de 50% de hepatoesplenomegalia).La sintomatología más común en estos enfermos es la afectación de la piel por urticaria pigmentosa (más frecuente en la infancia) o telangiectasia macularis pertans (más frecuente en adultos) donde los mastocitos pueden estar recluidos mucho tiempo, con clínica que proviene de sus mediadores, siendo los niveles de triptasa el reflejo de la carga tumoral. El manejo de esta enfermedad se basa en la administración de tratamiento sintomático con antagonistas de histaminas H1 y H2, así como cromoglicato disódico, necesitando terapia citorreductora sólo en las variantes agresivas de mastocitos sistémica (asociadas a mutación del receptor de tirosin kinasa c-kit D816V) o asociadas a SMP (proliferación de mastocitos e hipereosinofilias asociado a la expresión del gen de fusión FIP1L1-PDGFRA).El interferón tiene un efecto beneficioso sobre los síntomas dermatológicos, hematológicos, gastrointestinales y sistémicos, así como en los esqueléticos, debido a su capacidad de aumentar la densidad ósea y reducir los episodios dolorosos, siendo beneficioso el tratamiento inicial con prednisona.

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A case of systemic mastocytosis associated with severe osteoporosis and pathologic fractures

Cited by 6 publications

References 6 publications

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Bone Mineral Content in Patients with Anaphylactic Reactions, Signs of Mastocytosis and Elevated Basal Serum Tryptase Levels

Mastocitosis sistémica: Revisión sistemática

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