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Cell Adhesion Molecules in Mast Cell Adhesion and Migration
Published in Bruce S. Bochner, Adhesion Molecules in Allergic Disease, 2020
Harissios Vliagoftis, Dean D. Metcalfe
Many observations concerning the human mast cell expression of integrins use mast cells dispersed from normal tissues using proteases or examine mast cells that are grown in culture. Other investigators have examined HMC-1 cells, the only human mast cell culture available and which was established from a patient with mast cell leukemia (35). Unfortunately, this cell does not express high-affinity IgE receptors and is not typical of human mast cells isolated from tissues. Table 1 summarizes published data on adhesion molecule expression on human mast cells (36–41).
Chronic Leukemias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Scott J. Graham, James D. Cotelingam
The least common manifestation of systemic mast cell disease is mast cell leukemia (MCL), which is a rare disorder. Patients frequently present with constitutional symptoms and peptic ulcer disease. Total WBC counts are variable (ranging from 8.5 to 66.0 × 109/L), with absolute mast cell counts ranging from 0.65 to 47.0 × 109/L. Mast cells should constitute a minimum of 10% of the WBC differential to establish the diagnosis of MCL. The number of circulating mast cells does not seem to correlate with either tumor burden or survival.
Other Myeloproliferative Neoplasms
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Skin findings are often accompanied by symptoms secondary to mast cell release of mediators. These symptoms can be both localized to the skin lesion and systemic because of the release of mediators into the bloodstream. The majority of pediatric cases of cutaneous mastocytosis show a good prognosis with gradual resolution of both symptoms and skin lesions. Findings in patients with SM include anemia, leukocytosis, eosinophilia, thrombocytopenia, and splenomegaly. SM variants can be further subclassified by the presence of clinicopathological criteria referred to as “B” findings (>30% mast cells in the BM and/or serum tryptase levels >200 ng/mL; increased marrow cellularity/dysplasia without meeting the criteria for another myeloid neoplasm; or enlargement of the liver, spleen, or lymph nodes without evidence of organ damage) and “C” criteria (evidence of organ damage such as abnormal liver function and/or ascites, hypersplenism, cytopenias, large osteolytic lesions/fractures, and malabsorption with weight loss) [1,34,36,37]. Mast cell leukemia is an extremely rare subtype of mastocytosis with a leukemic spread of mast cells and a rapid progression of disease. The criteria for SM are presented in Table 31.3.
Drug delivery targets and strategies to address mast cell diseases
Published in Expert Opinion on Drug Delivery, 2023
Clayton H. Rische, Ariel H. Thames, Rebecca A. Krier-Burris, Jeremy A. O’Sullivan, Bruce S. Bochner, Evan A. Scott
Mastocytosis and mast cell leukemia are relatively rare conditions that are notoriously difficult to treat [177]. Only recently have therapies with improved specificity for the treatment of these diseases become available, delivering improved patient outcomes [10,178,179]. It is important to mention that mastocytosis and its subvariants present a great challenge when it comes to developing novel therapies: the pool of patients available for clinical trials is relatively small [180]. This is made even more difficult by additional constraints tied to disease subtypes or requirements for no preexisting therapies [7–9,177]. Table 2 details selectivity for mast cells and clinical development for each drug. Primary strategies by which malignant mast cells rely on either small molecule therapeutics or antibodies and antibody-drug conjugates have additionally been provided (Figure 3).
Systemic mastocytosis with myeloid sarcoma and B-CLL: molecular and clonal heterogeneity in a rare case of SM-AHN with review of literature
Published in Acta Clinica Belgica, 2023
Philippe Decruyenaere, Dominiek Mazure, Ine Moors, Jo Van Dorpe, Malaïka Van der Linden, Barbara Denys, Mattias Hofmans, Fritz Offner
AML: acute myeloid leukemia AML-MRC: acute myeloid leukemia with myelodysplasia-related changes ASM: aggressive systemic mastocytosis B-CLL: B-cell chronic lymphatic leukemia BM: bone marrow CML: chronic myeloid leukemia CMML: chronic myelomonocytic leukemia ELN: European LeukemiaNet classification FCR: fludarabine, cyclophosphamide and rituximab FFPE: formalin-fixed paraffin-embedded FISH: fluorescence in situ hybridization IPSS-R: Revised International Prognostic Scoring System ISM: indolent systemic mastocytosis MAPS: Mayo Alliance Prognostic System MARS: German registry derived mutation-adjusted risk score MC: mast cell MCL: mast cell leukemia MDS: myelodysplastic syndrome MPN: myeloproliferative neoplasms RB1: RB transcriptional corepressor 1 gene SM-AHN: systemic mastocytosis with an associated hematological neoplasm SSM: smoldering systemic mastocytosis VAF: variant allele frequency WHO: World Health Organization
Pharmacotherapy in Mast Cell Leukemia
Published in Expert Opinion on Pharmacotherapy, 2020
Mariarita Laforgia, Concetta Calabrò, Anna Scattone, Carmelo Laface, Mariangela Porcelli, Cosmo Damiano Gadaleta, Patrizia Nardulli, Girolamo Ranieri
As a rare disease, mast cell leukemia (MCL) hits less than 1% of the systemic mastocytosis (SM) patients [1]. SM is a class of different forms of hematologic disease that WHO detailed and classified in 2008 for the first time and then revised in 2016 in: Indolent SM (ISM),Smoldering Systemic Mastocytosis (SSM);SM with associated clonal hematologic non-mast cell lineage disease (SM-AHN);Aggressive SM (ASM)Mast cell sarcoma (MCS)Mast cell leukemia (MCL)