Sequential immunophenotypic analysis of mast cells in a case of systemic mast cell disease evolving to a mast cell leukemia

Escribano, Luís; Órfão, Alberto; Villarrubia, Jesús; Martin, F. S.; Madruga, J. I.; Cuevas, M.; Velasco, J. L.; Ríos, A; Miguel, Jesús F. San

doi:10.1002/(sici)1097-0320(19970415)30:2<98::aid-cyto4>3.0.co;2-9

Cited by 65 publications

(55 citation statements)

References 28 publications

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“…In MCL, MCs usually exhibit a similar phenotype compared to MCs in indolent SM [34][35][36]. However, there are number of subtle phenotypic differences to consider: first, MCs in MCL usually express only low amounts or lack CD2 [34,36].…”

Section: Discussionmentioning

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: Discussionmentioning

confidence: 99%

“…However, there are number of subtle phenotypic differences to consider: first, MCs in MCL usually express only low amounts or lack CD2 [34,36]. Second, MCs in MCL usually express substantial amounts of CD52 and CD123, whereas CD52 and CD123 are expressed less abundantly on MCs in indolent SM [36,37].…”

Section: Discussionmentioning

confidence: 99%

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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“…The cell surface phenotype of MCs in MCL includes various myeloid determinants and is quite similar when compared to the cell surface membrane phenotype of MCs in other variants of SM [9,10,[24][25][26][27]. Notably, leukemic MCs in MCL usually express CD9, CD25, CD33, CD44 and CD117 (KIT) [9,10,[25][26][27].…”

Section: Cell Surface Phenotype Of Mcs In Chronic MCLmentioning

confidence: 94%

“…Notably, leukemic MCs in MCL usually express CD9, CD25, CD33, CD44 and CD117 (KIT) [9,10,[25][26][27]. In addition, MCs in MCL may express CD123 and HLA-DR.…”

Section: Cell Surface Phenotype Of Mcs In Chronic MCLmentioning

confidence: 99%

“…The CD2 antigen (LFA-2) is also detectable on MCs in a subset of patients with MCL [9,27]. However, compared to patients with indolent SM, the number of ´CD2-positive´ cases among MCL patients is low, and if detected on the surface of MCs, the levels of CD2 are usually lower compared to that found on MCs in indolent SM [25][26][27]. These observations suggest that CD2 expression may decrease during progression of SM into MCL which has been confirmed in individual patients with SM [27] as well as by an in vitro model of human MCL [28].…”

Section: Cell Surface Phenotype Of Mcs In Chronic MCLmentioning

confidence: 99%

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Chronic mast cell leukemia: A novel leukemia-variant with distinct morphological and clinical features

Valent¹,

Sotlar²,

Sperr³

et al. 2015

Leukemia Research

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SummaryMast cell leukemia (MCL) is a rare form of systemic mastocytosis characterized by leukemic expansion of mostly immature mast cells, organ damage, drug-resistance, and a poor prognosis. Even when treated with chemotherapy, most patients have a life-expectancy of less than one year. However, there are rare patients with MCL in whom the condition is less aggressive and does not cause organ damage within a short time. In these patients, mast cells exhibit a more mature morphology when compared to acute MCL. A recently proposed classification suggests that these cases are referred to as chronic MCL. In the present article, we discuss clinical, histopathological and morphological aspects of acute and chronic MCL.

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Expression of Bcl-2 by human bone marrow mast cells and its overexpression in mast cell leukemia

et al. 1999

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Bcl-2 protein plays a major role in the prevention of programmed cell death of differentiating cells. In the present study, the expression of cytoplasmic bcl-2 by human Bone Marrow Mast Cells (BMMC) from both normal and pathological bone marrow samples was examined. A total of 35 subjects corresponding to 9 healthy volunteers, 8 cases of adult indolent systemic mast cell disease (SMCD), 4 cases of pediatric mastocytosis (PM), 11 cases of hematological malignancies (HM), 2 cases of reactive bone marrow, and 1 case of mast cell leukemia (MCL) were analyzed. The expression of bcl-2 was studied using quantitative three-color flow cytometry. We also studied the molecular configuration of the bcl-2 gene and other relatives by Southern blot and polymerase chain reaction (PCR) in the MCL case. Bcl-2 expression was detected in BMMC from all samples analyzed. No significant differences on the expression of bcl-2 were detected between BMMC from healthy subjects and patients with SMCD, PM, HM, and reactive bone marrow. By contrast , bcl-2 protein was overexpressed in BMMC from MCL patient without gene rearrangement. Our results show that bcl-2 protein was constitutively expressed by BMMC. BMMC from MCL display overexpression of bcl-2, which could not be related to molecular rearrangements involving the bcl-2 gene. The expression of this protein by mature MC may play a role in the prevention of MC apoptosis and thus help to explain the long survival of these cells. The overexpression of bcl-2 by BMMC in MCL may help to explain their resistance to chemotherapy-induced apoptosis. Am.

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Sequential immunophenotypic analysis of mast cells in a case of systemic mast cell disease evolving to a mast cell leukemia

Cited by 65 publications

References 28 publications

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

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

Chronic mast cell leukemia: A novel leukemia-variant with distinct morphological and clinical features

Expression of Bcl-2 by human bone marrow mast cells and its overexpression in mast cell leukemia

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