Systemic mastocytosis: a potential neurologic emergency
| Autor: | Giorgio B. Boncoraglio, E. Maccagnano, D. Soligo, L. O. Scappatura, E. A. Parati, M. R. Carriero, L. Robbiolo, Antonio Brucato |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
Brain Infarction
medicine.medical_specialty Abdominal pain Pathology Nausea Hepatosplenomegaly Histamine Antagonists Unconsciousness Quadriplegia Mastocytosis Systemic Urticaria Pigmentosa medicine Aphasia Humans Treatment Failure Systemic mastocytosis Coma Bone pain business.industry Cutaneous Mastocytosis Brain Middle Aged medicine.disease Dermatology Magnetic Resonance Imaging Acute Disease Hypoxia-Ischemia Brain Disease Progression Urticaria pigmentosa Female Neurology (clinical) medicine.symptom Atrophy Hypotension business Infiltration (medical) |
| Zdroj: | Neurology. 65(2) |
| ISSN: | 1526-632X |
| Popis: | Mastocytosis refers to an uncommon and heterogeneous group of clonal hematologic disorders characterized by pathologic accumulation of mast cells (MCs) in various tissues. In 80 to 90% of cases, abnormal MC infiltration is restricted to the skin (cutaneous mastocytosis) and appears as small discrete red-brown maculae (urticaria pigmentosa); prognosis is favorable. By contrast, multifocal MC accumulation in extracutaneous organs (systemic mastocytosis [SM]) is potentially life threatening; the skin may or not be involved.1 The presenting signs and symptoms of SM are due to pathologic infiltration of MCs and release of their chemical mediators, primarily histamine. Typical clinical features include urticaria pigmentosa, hepatosplenomegaly, bone pain, headache, flushing, pruritus, nausea, diarrhea, abdominal pain, and peptic ulcer. Sudden transient loss of consciousness and hypotensive or anaphylactic shock may also occur.2 Patients with undiagnosed SM can also present with neurologic symptoms.3 We describe a 64-year-old woman with unexplained recurrent episodes of loss of consciousness and neuroradiologic signs of chronic brain ischemia, who developed severe anoxic encephalopathy after … |
| Databáze: | OpenAIRE |
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