CASE REPORTS
Pediatric Dermatology Vol. 29 No. 5 605–609, 2012
Death from Mast Cell Leukemia: A Young Patient with Longstanding Cutaneous Mastocytosis Evolving into Fatal Mast Cell Leukemia
Rattanavalai Chantorn, M.D.,*, and Tor Shwayder, M.D.
*Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand, Department of Pediatric Dermatology, Henry Ford Hospital, Detroit, Michigan
Abstract: Mastocytosis is a broad term used for a group of disorders characterized by accumulation of mast cells in the skin with or without extracutaneous involvement. The clinical spectrum of the disease varies from only cutaneous lesions to highly aggressive systemic involvement such as mast cell leukemia. Mastocytosis can present from birth to adult- hood. In children, mastocytosis is usually benign, and there is a good chance of spontaneous regression at puberty, unlike adult-onset disease, which is generally systemic and more severe. Moreover, individuals with systemic mastocytosis may be at risk of developing hematologic malignancies. We describe a girl who presented to us with a solitary mastocytoma at age 5 and later developed maculopapular cutaneous mastocytosis. At age 23, after an episode of anaphylactic shock, a bone marrow examination revealed mast cell leukemia. She ultimately died despite aggressive chemotherapy and bone marrow transplantation.
Mastocytosis is characterized by the abnormal common forms of CM in childhood. The excoriation growth and infiltration of mast cells (MC) in various of lesions causes hives and perilesional erythema, tissues and is classified into two broad categories: which characterizes Darier’s sign (Fig. 3). SM is cutaneous mastocytosis (CM) and systemic mastocy- characterized by multifocal MC infiltrates with or tosis (SM) (1). The course of mastocytosis is variable without skin involvement and is markedly more and is dependent on the subtype and age of onset common in adults than in children. Mast cell (2–4). Mast cell disease in children is usually only leukemia is a rare variant of SM, with few cutaneous and not associatedwithsystemicor cases reported in the literature and usually associated malignant disorders. In adults, MC disease can with grave prognosis (6–12). The purpose of this be systemic and progressive (4). Mastocytosis affects article is to report a case of longstanding childhood infants and children twice as frequently as adults (5). CM evolving to fatal MC leukemia during young Mastocytoma and urticaria pigmentosa (UP) are adulthood.
Address correspondence to Tor Shwayder, M.D., Director, Pediatric Dermatology, Henry Ford Hospital, Detroit, MI 48202, or e-mail: [email protected].
DOI: 10.1111/j.1525-1470.2011.01650.x