1/12. serum interleukin-6 reflects disease severity and osteoporosis in mastocytosis patients.BACKGROUND: Systemic mastocytosis (SM) is a condition typically characterized by an increased number of mast cells in the bone marrow or in skin areas known as urticaria pigmentosa. patients may present with flushing, itching, gastrointestinal symptoms, arrhythmias, headaches and osteoporosis. Some patients experience systemic symptoms indicative of SM in the absence of a positive bone marrow or skin biopsy, and are known as 'clinical mastocytosis', but are herein referred to as suspected of having systemic mastocytosis. serum tryptase has been increasingly used as a biochemical marker of mastocytosis, but is not always elevated. OBJECTIVE: To investigate the association of serum levels of two key mast cell mediators, interleukin-6 (IL-6) and tryptase, to each other and with disease severity in patients with mastocytosis. methods: patients responded to an announcement from the Systemic mastocytosis Society (USA) and submitted frozen serum samples, but the precise diagnosis made by their own health providers was not known until after the assays were completed. There were 9 suspected systemic mastocytosis (SuSM), 3 cutaneous mastocytosis (CM), 11 indolent systemic mastocytosis (ISM), and 3 aggressive systemic mastocytosis (ASM). Five normal volunteers (3 females/2 males) also submitted samples, as did 33 cardiac patients without coronary artery disease. For 2 days prior to and during the collection period, mastocytosis patients were asked to abstain from any over-the-counter or food products containing biogenic amines, as well as drugs prescribed for this condition. serum levels of IL-6 and tryptase were measured using established assays. RESULTS: Twenty-six patients (14 females/12 males) submitted serum samples. There were 9 cases of SuSM (6 females/3 males) in whom tryptase values were borderline normal; IL-6 values were slightly elevated with one being high. In 3 cases of CM (2 females/1 male), both tryptase and IL-6 were slightly elevated. In patients with ISM (5 females/6 males), only 6/11 had any tryptase elevated significantly as compared to 9/11 with elevated serum IL-6. Three patients with ASM had significant elevations of both IL-6 and tryptase. The most consistent finding was that of IL-6 elevations in 7/7 patients (3 females/4 males) who reported symptoms of osteoporosis and/or bone pain (1 SuSM, 3 ISM, 3 ASM) in the absence of any coexisting condition involving bone pathology. CONCLUSION: serum IL-6 is elevated in mastocytosis patients and correlates with severity of symptoms and the presence of osteoporosis. High serum IL-6 may not only signify disease progression, but may also participate in the pathophysiology of mastocytosis.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/12. Acute spinal epidural hematoma and systemic mastocytosis.BACKGROUND: Systemic mastocytosis is a mast cell proliferative disorder affecting many organs that is rarely associated with internal bleeding. OBJECTIVE: To describe a case of spinal epidural hematoma in a patient with past medical history of urticaria pigmentosa and osteoporosis diagnosed with systemic mastocytosis. CASE REPORT: A 63-year-old woman with urticaria pigmentosa was admitted to hospital for severe back pain after minor trauma. physical examination showed pain on pressing T12 and L1 spinous processes, bilateral Lasegue sign, absent ankle jerk, and extensor plantar response. Computed tomography disclosed L3 fracture, and magnetic resonance imaging revealed spinal epidural hematoma and T2 hyperintensive scattered vertebral foci that suggested malignancy. The 24-hour urine histamine was very high. Mast cell infiltration was found in bone marrow biopsy. Because power was normal and there was no clinical sphincter disorder, the patient was successfully treated with conservative care. CONCLUSIONS: To our knowledge, acute intraspinal epidural hematoma has never been associated with mastocytosis. The hematoma was likely related to the vertebral fracture as well as a hemorrhagic diathesis due to anticoagulants released by local mast cells.- - - - - - - - - - ranking = 0.4375keywords = mastocytosis (Clic here for more details about this article) |
3/12. Bone mastocytosis. A report of nine cases with a bone histomorphometric study.Bone mastocytosis is characterized radiographically in some patients by diffuse osteosclerosis and in others by demineralization. The reason for these apparently conflicting bone features is unknown. bone remodeling and marrow mastocytosis infiltration were studied in nine cases of mastocytosis with bone marrow involvement. Six men, ranging from 42 to 78 years of age, and three women, 43, 55, and 73 years old, comprised the series. Two patients had severe and diffuse osteosclerosis. Seven had diffuse demineralization, with crushed vertebrae in four, suggesting common osteoporosis. In three of the seven, cutaneous mastocytosis was absent. Bone biopsies were undecalcified and stained with toluidine blue. In the seven patients with demineralization, the number of marrow mastocytes was increased (154 /- 24 versus 2 /- 0.5/mm2 in normal postmenopausal osteoporosis). Mastocyte nodules covering 1-9% of the marrow area were present in all seven patients. These patients showed a significant increase in remodeling; bone formation rate was increased, coupled with a decrease in mean wall thickness. Concomitantly, osteoclast surfaces were increased, with an increased amount of bone resorbed. The two patients with diffuse osteosclerosis had a markedly different histology; mast cell infiltration was dramatically increased (mastocyte count greater than 1000/mm2) with diffuse marrow fibrosis. Bone volume was increased as well, and most of the bone was woven with an intratrabecular mineralization defect. High bone remodeling and decreased osteoblast activity can explain bone loss in mastocytosis with demineralization. mastocytosis with osteosclerosis is characterized by a more extensive marrow mast-cell infiltration and fibrosis.- - - - - - - - - - ranking = 0.5625keywords = mastocytosis (Clic here for more details about this article) |
4/12. Systemic mastocytosis mimicking inflammatory bowel disease: A case report and discussion of gastrointestinal pathology in systemic mastocytosis.Gastrointestinal (GI) symptoms are present in up to 80% of patients with systemic mastocytosis (SM). GI symptoms include mainly abdominal pain, diarrhea, nausea, and vomiting. It is believed that most of the GI symptoms are due to the secondary effect of mast cell mediators on the GI tract. Direct involvement of the GI tract by neoplastic mast cell infiltration has not been well documented. We report a case of SM that initially mimicked inflammatory bowel disease based on clinical, radiographic, endoscopic, and histopathologic findings. On routine histologic sections of small bowel and colonic mucosal biopsies, there was expansion of the lamina propria by mononuclear inflammatory cells, foci of erosions with associated acute inflammation, and evidence of chronic mucosal injury with architectural distortion and gland foreshortening. Only on repeat biopsies and with ancillary tests for mast cells was a diagnosis of SM made, with extensive involvement of the GI tract. This is the first reported case of SM presenting as and mimicking inflammatory bowel disease. It is critical that clinicians and pathologists are aware that neoplastic mast cells in patients with SM can infiltrate the mucosa throughout the GI tract and that this infiltration can lead to symptoms and findings that can mimic inflammatory bowel disease.- - - - - - - - - - ranking = 0.5625keywords = mastocytosis (Clic here for more details about this article) |
5/12. 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 = 0.3125keywords = mastocytosis (Clic here for more details about this article) |
6/12. osteoporosis associated with mastocytosis confined to bone: report of two cases.Two women, ages 53 and 46, presented with multiple spontaneous vertebral fractures. serum calcium, 25OHD, 1,25-(OH)2D, PTH, and 24-h urinary calcium were all normal. Bone mineral analysis (BMA) was performed at the junction of the distal third of the radius by single photon absorptiometry. BMA on Patient 1 was 0.68 g/cm2 and on Patient 2 was 0.58 g/cm2, with the normal being 0.70 g/cm2 and 0.77 g/cm2, respectively. In addition to vertebral fractures, x rays of Patient 2 revealed lytic lesions of the tibia and ulna. Bone biopsies revealed osteopenia associated with generalized eosinophilic fibromastocytosis and increased resorption without evidence of increased numbers of osteoclasts. There was no evidence of extraskeletal mast cell involvement. Our observations contribute to the increasing evidence that mast cells play a direct role in the etiology of osteoporosis in certain patients. Possible pathophysiologic mechanisms include mast cell release of heparin, histamine, prostaglandin d2 or other osteolytic substances.- - - - - - - - - - ranking = 0.3125keywords = mastocytosis (Clic here for more details about this article) |
7/12. Systemic mastocytosis, an unusual cause of osteoporosis.A 45-year-old patient is described presenting with severe axial osteoporosis. The presence of maculopapular skin lesions and the combination of osteolytic and osteoblastic bone lesions led to the tentative diagnosis of systemic mastocytosis. This diagnosis was confirmed by measurement of urinary histamine metabolites and by bone biopsy showing increased numbers of mast cells and fibroblast-like cells. The importance of these diagnostic procedures in this rare cause of osteoporosis is discussed.- - - - - - - - - - ranking = 0.3125keywords = mastocytosis (Clic here for more details about this article) |
8/12. 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 = 0.375keywords = mastocytosis (Clic here for more details about this article) |
9/12. adult osteosclerosis.Quantitative bone histology was carried out in five osteosclerotic adults. The bone was extremely hard in all patients, and open biopsy was usually required. One patient, aged 18 years, presented with hypoplastic anemia, and the most probable explanation for the osteosclerosis is a marrow stem cell defect leading to defective osteoclasts. Another had the dominant form of osteopetrosis. Her bone contained cartilage remnants, and there were many large, morphologically abnormal osteoclasts, which lacked normal cytoplasmic acid phosphatase activity. The third patient had chronic renal failure and osteomalacia; here the increased bone mass might have resulted from an inability of normal osteoclasts to resorb bone, due to the surface coating of osteoid, though an earlier increase of bone formation cannot be excluded. The fourth patient, who suffered from systemic mastocytosis, had high turnover bone, with greatly increased bone formation. The fifth patient, with fluorosis of bone, also had increased bone formation and resorption, the process being much more pronounced in the head of her pathologically fractured femur than it was in the iliac crest. In this patient some osteoclasts had reduced acid phosphatase activity and long cytoplasmic extensions, both changes similar to those observed in diphosphonate-treated animals. Very diverse processes can result in the increased cancellous bone mass producing the radiographic appearance of diffuse osteosclerosis.- - - - - - - - - - ranking = 0.0625keywords = mastocytosis (Clic here for more details about this article) |
10/12. Long-term treatment of systemic mastocytosis with histamine H2 receptor antagonists.A patient with systemic mastocytosis, accompanied by gastric hypersecretion and duodenal ulcer, was treated with metiamide followed by daily cimetidine for 44 months. Treatment with cimetidine resulted in healing of the ulcer, without recurrence and marked amelioration of the cutaneous symptoms of mastocytosis. Complete suppression of basal gastric hypersecretion was documented after 33 months of treatment and cimetidine and Vitamin B12 absorption remained normal. cimetidine reduced the patient's cutaneous response to intradermal histamine without affecting leucocyte histamine release. No cimetidine toxicity was observed. These results indicate that effective long-term control of histamine-induced gastric hypersecretion can be achieved with cimetidine. They suggest that some of the cutaneous symptoms of mastocytosis are mediated via histamine H2 receptors in the skin.- - - - - - - - - - ranking = 0.4375keywords = mastocytosis (Clic here for more details about this article) |
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