Hypotension, Syncope, and Fever in Systemic Mastocytosis without Skin Infiltration and Rapid Response to Corticosteroid and Cyclosporin: A Case Report

Case Reports in Medicine - Tập 2010 - Trang 1-4 - 2010
Didem Özdemır1, Selçuk Dağdelen2, Tomris Erbaş2, Kemal Ağbaht1, Songül Şerefhanoğlu3, Salih Aksu3, Sibel Ersoy Evans4
1Department of Internal Medicine, Hacettepe University Medical School, Sıhhiye, 06350 Ankara, Turkey
2Department of Endocrinology and Metabolism, Hacettepe University Medical School, 06350 Ankara, Turkey
3Department of Hematology, Hacettepe University Medical School, 06350 Ankara, Turkey
4Department of Dermatology, Hacettepe University Medical School, 06350 Ankara, Turkey

Tóm tắt

Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a small proportion, there is extracutaneous involvement without skin infiltration. Other manifestations are flushing, tachycardia, dyspepsia, diarrhea, hypotension, syncope, and rarely fever. Various medications have been used but there is not a definite cure for systemic mastocytosis. The principles of treatment include control of symptoms with measures aimed to decrease mast cell activation. We describe a case of systemic mastocytosis presenting with hypotension, syncope attacks, fever, and local flushing. In bone marrow biopsy, increased mast cell infiltration was demonstrated. She had no skin infiltration. A good clinicopathological response was obtained acutely with combination therapy of glucocorticoid and cyclosporine.

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Tài liệu tham khảo

10.1159/000101711

10.1038/sj.leu.2404955

10.1146/annurev.med.55.091902.103822

10.1016/S0140-6736(96)07056-0

2004, Haematologica, 89, ECR35

1979, Mayo Clinic Proceedings, 54, 51

10.1111/j.1365-2362.2007.01807.x

10.1182/blood-2009-02-205237

10.1182/blood-2003-05-1699

10.1080/10428190400010775

1994, Allergy Proceedings, 15, 119, 10.2500/108854194778702946

10.1016/S0889-8588(03)00089-3

10.1016/j.leukres.2004.05.003

10.1046/j.1365-2141.2002.03944.x

10.1002/ajh.20211

10.1111/j.1365-2133.1995.tb08689.x

10.1056/NEJM200101253440414

10.1002/cncr.21996

2004, Current Hematology Reports, 3, 197

10.1016/j.leukres.2008.12.020

1999, Journal of Allergy and Clinical Immunology, 103, S412, 10.1016/S0091-6749(99)70156-9

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Case Reports in Medicine

Tập 2010

1-4

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