1/5. Systemic mastocytosis presenting with severe spinal osteopenia and multiple compression fractures.Systemic mastocytosis is a rare condition in which mast cells infiltrate various organs, including the skeleton. Because the mast cell secretes various bioactive substances that may induce bone resorption, this condition may cause generalized osteoporosis. We describe a case of a 28-year-old woman who presented with a painful thoracolumbar kyphosis due to generalized osteopenia and multiple pathological compression fractures and was found to have mastocytosis. She underwent operative stabilization of her kyphotic deformity with anterior interbody fusion and posterior Cotrel-Dubousset (CD) instrumentation and fusion. We conclude that mastocytosis should be suspected in an atypical case of so-called idiopathic osteoporosis.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/5. Migratory multiple bone involvement in a patient with systemic mastocytosis.A young patient with severe generalized osteopenia and multiple compressed vertebral fractures caused by systemic mastocytosis is presented. Extensive clinical, laboratory, and pathologic examination showed the involvement of the skeleton alone. Follow-up bone scintigraphy with Tc-99m MDP during a period of 6 years demonstrated migratory multiple axial lesions. These results suggest that Tc-99m MDP imaging is an accurate detection of the activity of the disease.- - - - - - - - - - ranking = 0.71428571428571keywords = mastocytosis (Clic here for more details about this article) |
3/5. Pathologic fracture in systemic mastocytosis. Radiographic spectrum and review of the literature.Systemic mastocytosis is a multiorgan disease that most commonly affects the skin and skeletal system. Radiographically, the skeletal changes in the majority of patients consist of either a wide-spread mixture of bone lysis and osteosclerosis or generalized osteoporosis. The osteoporotic form is less well known but may lead to severe generalized demineralization and pathologic fractures. mast cells secrete a number of substances, two of which (heparin and prostaglandins) are believed to have a role in the induction of osteoporosis. Sclerotic lesions are induced by another mast cell by-product, histamine. One hundred seventy-eight cases of bony mastocytosis have been reported in the literature, including the four patients in the present report. Special staining procedures are necessary for identification of mast cells. diagnosis may be delayed in patients who do not have the skin lesions (urticaria pigmentosa) and in the osteoporotic form of the disease.- - - - - - - - - - ranking = 0.85714285714286keywords = mastocytosis (Clic here for more details about this article) |
4/5. Systemic mastocytosis associated with generalized osteopenia. Histopathological characterization of the skeletal lesion using undecalcified bone from two patients.Although mast cell proliferation in the bone marrow frequently occurs in systemic mastocytosis and is often associated with radiographically detectable bone lesions, the pathologic correlates of the skeletal abnormalities are poorly characterized. We therefore examined three nondecalcified transiliac crest biopsy specimens from two patients with systemic mastocytosis and diffuse osteopenia with vertebral crush fractures. Marrow involvement included unusual mast cell aggregates, as revealed by metachromatic staining, that mimicked granulomas. Histomorphometric analysis or trabecular bone revealed accelerated bone remodeling or "turn over" characterized by osteoidosis, peritrabecular fibrosis, increased numbers of osteoblasts and osteoclasts, and an increase in osteoclastic resorbing surfaces. Our observations and review of the literature suggest that with the recent development of techniques for assessing undecalcified bone biopsy specimens, mastocytosis will probably be shown to be a more common etiology in "osteoporosis" than previously recognized. Metachromatic staining of the biopsy specimen should be a routine procedure in the investigation of any patient who undergoes diagnostic bone biopsy.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
5/5. Case report of bone mastocytosis: total hip arthroplasty for osteoarthritis and open reduction for condylar fracture of the knee.A 72-year-old woman was admitted in 1984 for painful protrusive osteoarthritis of the left hip diagnosed as systemic mastocytosis with bone lesions and clinical features of intestinal malabsorption but no clinical skin lesion. The total hip replacement, refused in a first step because of the bone pathology, was carried out two years later. Signs of loosening appeared after one year. In 1990, following a traumatic bicondylar fracture of the left knee, an osteosynthesis was carried out. Ten days later, a shaft pathologic fracture of the femur above the osteosynthesis plate implied another open reduction. Two and a half years later, the patient is able to walk short distances, using walking sticks, and lives at home receiving social assistance.- - - - - - - - - - ranking = 0.71428571428571keywords = mastocytosis (Clic here for more details about this article) |