A case report recently published in Hematology in Clinical Practice involving a patient with osteoporosis secondary to systemic mastocytosis (SM) has illustrated the therapeutic options available.
SM is a condition associated with excessive mast cellMast cell A type of white blood cell produced in the bone marrow. They help defend against infections and play a key role in allergic reactions. In SM, mast cells become overactive and build up throughout the body. proliferation and activation that can affect a variety of organs and produce diverse symptoms, such as flushing, palpitations and diarrhea. Loss of bone density (osteoporosis) is a common manifestation of SM; however, the exact mechanisms that lead to osteoporosis in patients with SM are not fully understood.
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Osteoporosis is associated with an increased risk of bone fractures even after minor trauma, and in patients with SM, it appears to affect mostly the spine. Spinal fractures can have debilitating consequences that can significantly affect long-term quality of life.
“In cases of bone infiltration, the primary goal was to protect against the progression of osteolytic lesions with fractures,” the authors wrote.
The authors described a case involving a 50-year-old man with a year-long history of recurrent flushing and palpitations who was admitted to the hospital due to a vertebral fracture in 2015. The attending physicians performed a bone marrow biopsyBone marrow biopsy A procedure to collect a sample of bone marrow using a needle. Often used to examine the characteristics of mast cells and diagnose SM., revealing findings suggestive of SM.
Further workup revealed severe osteoporosis, and the patient was started on calcium, vitamin D and risedronate 35. Three years after the SM diagnosis, treatment for osteoporosis had to be suspended due to adverse effects (nausea and abdominal pain).
Risedronate 35 was then substituted for intravenous zoledronic acid. The patient tolerated the new treatment well and suffered no more fractures.
“In this case, zoledronic acid therapy has shown a significant increase of BMD and reduced risk of fracture. In severe cases or in patients with BP contraindications, IFN-alpha and denosumab are used,” the authors wrote.
They added that additional studies and more data is necessary to better understand the mechanism of bone involvement and determine the effectiveness of each treatment option.
