Diagnostic criteria and classification of mastocytosis: a consensus proposal

Valent, Peter; Horny, Hans Peter; Escribano, Luís; Longley, B. Jack; Li, Chin Y.; Schwartz, Lawrence B.; Marone, Gianni; Núñez, Rosa; Akin, Cem; Sotlar, Karl; Sperr, Wolfgang R.; Wolff, Klaus; Brunning, Richard D.; Parwaresch, Reza; Austen, K. Frank; Lennert, K.; Metcalfe, Dean D.; Vardiman, James W.; Bennett, John M.

doi:10.1016/s0145-2126(01)00038-8

Cited by 1,016 publications

(1,477 citation statements)

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“…Despite recurrent mutations in codon 816 of the KIT protooncogene, SM is an extremely heterogeneous disease, ranging from a completely indolent course to lifethreatening disease-variants with short survival-times [1][2][3][4]. According to the proposed classification of the World Health Organization (WHO) and the EU-US consensus group, patients with advanced SM can be divided into aggressive SM (ASM), SM with an associated clonal hematologic non-MC-lineage disease (SM-AHNMD) and MC leukemia (MCL) [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

“…MCL is defined by leukemic expansion of immature MCs in various organs, including the BM, liver, and spleen [1][2][3][4][5][6][7][8][9][10]. As per definition, the BM smear in MCL contains an excess of immature neoplastic MCs representing at least 20% of all nucleated BM cells [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

“…As per definition, the BM smear in MCL contains an excess of immature neoplastic MCs representing at least 20% of all nucleated BM cells [5][6][7]. In most cases, these MCs are resistant against various targeted drugs and conventional antineoplastic drugs [8][9][10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

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Chronic mast cell leukemia (MCL) with KIT S476I: a rare entity defined by leukemic expansion of mature mast cells and absence of organ damage

Valent

Berger²,

Cerny-Reiterer

et al. 2014

Ann Hematol

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Mast cell leukemia (MCL) is a rare, life-threatening malignancy defined by a substantial increase in neoplastic mast cells (MCs) in bone marrow (BM) smears, drug-resistance and a poor prognosis. In most patients, the survival-time is less than 1 year. However, exceptional cases may present with a less malignant course. We report on a 49-year-old female patient with MCL Europe PMC Funders Group Europe PMC Funders Author ManuscriptsEurope PMC Funders Author Manuscripts diagnosed in 2013. In February 2013, first symptoms, including flushing, headache and diarrhoea, were recorded. In addition, mild anemia was detected. The disease was characterized by a massive increase in well-granulated, mature and often spindle-shaped MCs (80%) in BM smears. The serum tryptase level amounted to 332 ng/mL. Like in most other MCL patients, no skin lesions were detected. However, unlike in other patients, tryptase levels remained stable and no other signs or symptoms of MCL-induced organ damage were found. Sequencing studies revealed an isolated S476I point-mutation in KIT but no mutation in codon 816. The patient received histamine receptor blockers, but refused cytoreductive therapy. After 9 months, still no progression or organ damage was detected. However, progression with transformation to acute MCL occurred after 12 months. We propose that the chronic type of MCL with stable conditions, absence of organ damage and a mature MC morphology, is recognized as a distinct entity that should be distinguished from the acute variant of MCL.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Chronic mast cell leukemia (MCL) with KIT S476I: a rare entity defined by leukemic expansion of mature mast cells and absence of organ damage

Valent

Berger²,

Cerny-Reiterer

et al. 2014

Ann Hematol

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“…[1][2][3] Indolent and aggressive variants of systemic mastocytosis occur. 4,5 So-called B and C findings have been defined to sub-classify systemic mastocytosis. 4 The latter include organopathies especially of bone marrow, liver, and bone because of mast cell infiltration and is indicative of aggressive systemic mastocytosis.…”

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confidence: 99%

“…4,5 So-called B and C findings have been defined to sub-classify systemic mastocytosis. 4 The latter include organopathies especially of bone marrow, liver, and bone because of mast cell infiltration and is indicative of aggressive systemic mastocytosis. The former include a high mast cell burden (420% mast cells in the bone marrow; serum tryptase 4200 ng/ml; organomegaly) and signs of a spread of the disease into myeloid lineages (dysmyelopoiesis; KIT D816V in other myeloid lineages).…”

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confidence: 99%