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| Diagnostic criteria and classification of mastocytosis: a consensus proposal |
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| Author(s): Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nunez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM |
| Source: LEUKEMIA RESEARCH Volume: 25 Issue: 7 Special Issue: Sp. Iss. SI Pages: 603-625 Published: JUL 2001 |
| Times Cited: 289 References: 163 |
| Abstract: The term 'mastocytosis' denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Over the last 20 years, there has been an evolution in accepted classification systems for this disease. In light of such developments and novel useful markers, it seems appropriate now to re-evaluate and update the classification of mastocytosis. Here, we propose criteria to delineate categories of mastocytosis together with an updated consensus classification system. In this proposal, the diagnosis cutaneous mastocytosis (CM) is based on typical clinical and histological skin lesions and absence of definitive signs (criteria) of systemic involvement. Most patients with CM are children and present with maculopapular cutaneous mastocytosis (= urticaria pigmentosa, UP). Other less frequent forms of CM are diffuse cutaneous mastocytosis (DCM) and mastocytoma of skin. Systemic mastocytosis (SM) is commonly seen in adults and defined by multifocal histological lesions in the bone marrow (affected almost invariably) or other extracutaneous organs (major criteria) together with cytological and biochemical signs (minor criteria) of systemic disease (SM-criteria). SM is further divided into the following categories: indolent systemic mastocytosis (ISM), SM with an associated clonal hematologic non-mast cell lineage disease (AKNMD), aggressive systemic mastocytosis (ASM), and mast cell leukemia (MCL). Patients with ISM usually have maculopapular skin lesions and a good prognosis. In the group with associated hematologic disease, the AHNMD should be classified according to FAB/WHO criteria. ASM is characterized by impaired organ-function due to infiltration of the bone marrow, liver, spleen, GI-tract, or skeletal system, by pathologic MC. MCL is a 'high-grade' leukemic disease defined by increased numbers of MC in bone marrow smears (greater than or equal to 20%) and peripheral blood, absence of skin lesions, multiorgan failure, and a short survival. In typical cases, circulating MC amount to greater than or equal to 10% of leukocytes (classical form of MCL). Mast cell sarcoma is a unifocal tumor that consists of atypical MC and shows a destructive growth without (primary) systemic involvement. This high-grade malignant MC disease has to be distinguished from a localized benign mastocytoma in either extracutaneous organs (= extracutaneous mastocytoma) or skin. Depending on the clinical course of mastocytosis and development of an AHNMD, patients can shift from one category of MC disease into another. In all categories, mediator-related symptoms may occur and may represent a serious clinical problem. All categories of mastocytosis should be distinctively separated from reactive MC hyperplasia, MC activation syndromes, and a more or less pronounced increase in MC in myelogenous malignancies other than mastocytosis. Criteria proposed in this article should be helpful in this regard. (C) 2001 Elsevier Science Ltd. All rights reserved. |
| Document Type: Proceedings Paper |
| Language: English |
| Reprint Address: Valent, P (reprint author), Univ Vienna, Div Hematol, Dept Internal Med 1, Waehringer Guertel 18-20, A-1090 Vienna, Austria |
Addresses:
1. Univ Vienna, Div Hematol, Dept Internal Med 1, A-1090 Vienna, Austria
2. Med Univ Lubeck, Inst Pathol, D-23538 Lubeck, Germany
3. Hosp Ramon y Cajal, Unidad Mastocitosis, Serv Hematol, E-28034 Madrid, Spain
4. Columbia Univ, Dept Dermatol, New York, NY 10027 USA
5. Columbia Univ, Dept Pathol, New York, NY 10027 USA
6. Mayo Clin & Mayo Fdn, Div Hematopathol, Rochester, MN 55905 USA
7. Virginia Commonwealth Univ, Med Coll Virginia, Dept Internal Med, Div Rheumatol Allergy & Immunol, Richmond, VA 23298 USA
8. Univ Naples Federico II, Cattedra Immunol Clin & Allergol, Fac Med & Chirurg, Naples, Italy
9. NIAID, Lab Allerg Dis, NIH, Bethesda, MD 20892 USA
10. Univ Tubingen, Inst Pathol, D-7400 Tubingen, Germany
11. Univ Vienna, Dept Dermatol, A-1090 Vienna, Austria
12. Univ Minnesota, Dept Lab Med & Pathol, Minneapolis, MN 55455 USA
13. Univ Kiel, Inst Hematopathol & Lymph Node Registry, Kiel, Germany
14. Harvard Univ, Brigham & Womens Hosp, Sch Med, Div Rheumatol Allergy & Immunol, Boston, MA 02115 USA
15. Univ Chicago, Dept Pathol, Hematopathol Sect, Chicago, IL 60637 USA
16. Univ Rochester, Med Ctr, Ctr Canc, Med Oncol Unit, Rochester, NY 14642 USA |
| Publisher: PERGAMON-ELSEVIER SCIENCE LTD, THE BOULEVARD, LANGFORD LANE, KIDLINGTON, OXFORD OX5 1GB, ENGLAND |
| Subject Category: Oncology; Hematology |
| IDS Number: 442AM |
| ISSN: 0145-2126 |
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