Systemic mastocytosis revisited with an emphasis on skeletal manifestations
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COMPUTED TOMOGRAPHY
Systemic mastocytosis revisited with an emphasis on skeletal manifestations Antonio Leone1 · Marianna Criscuolo1 · Consolato Gullì1 · Antonella Petrosino1 · Nicola Carlo Bianco1 · Cesare Colosimo1 Received: 26 July 2020 / Accepted: 15 November 2020 © Italian Society of Medical Radiology 2020
Abstract Systemic mastocytosis (SM) is a rare form of mastocytosis that can affect various organ systems. Bone involvement is the most common and prominent imaging feature in patients with SM regardless of the subtype. Furthermore, bone involvement is a prognostic factor as it may entail an aggressive course of the disease. Diagnosis is established by bone marrow biopsy complemented by imaging modalities such as radiography, CT, and magnetic resonance (MR) imaging. The radiographic and CT appearances are that of sclerotic, lytic, or mixed patterns with focal or diffuse distribution, involving primarily the axial skeleton and the ends of the long bones. Bone marrow infiltration is best recognized on MR imaging. Osteoporosis is common in SM; thus, a bone mineral density measurement at lumbar spine and proximal femur by dual-energy X-ray absorptiometry should be obtained. Imaging plays a huge part in the diagnostic process; when skeletal imaging findings are carefully interpreted and correlated with clinical features, they can lead to the suspicion of SM. The primary aims of this review article were to focus on the role of imaging in detection and characterization of skeletal patterns of SM and to discuss relevant clinical features that could facilitate prompt and correct diagnosis. Keywords Systemic mastocytosis · Bone marrow · Radiography · CT · Magnetic resonance imaging
Introduction Mast cells derive from myeloid stem cells of the bone marrow [1]. Upon activation, mast cells release a wide spectrum of chemical mediators stored in mast cell cytoplasmic * Antonio Leone [email protected] Marianna Criscuolo [email protected] Consolato Gullì [email protected] Antonella Petrosino [email protected] Nicola Carlo Bianco [email protected] Cesare Colosimo [email protected] 1
Department of Radiological and Hematological Sciences Fondazione, Policlinico Universitario A. Gemelli, IRCCS Università Cattolica del Sacro Cuore, Largo A. Gemelli, 100168 Rome, Italy
granules, including histamine, heparin, serotonin, proteases, tumor necrosis factor, and prostaglandins. Thus, mast cells contribute in modulating several physiological processes such as allergic reactions, vascular homeostasis, angiogenesis, innate, and acquired immunity [2–4]. Mastocytosis is a rare hematological disease group marked by abnormal proliferation and accumulation of neoplastic mast cells in bone marrow and various organ systems, e.g., cardiovascular, respiratory, lymphoreticular, gastrointestinal systems, and mostly, skin and skeletal system [5]. In the 2019 update on diagnosis, risk stratification, and management of SM in adults, Pardanani [6] reported that the most accepted pathogenic
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