Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings - PubMed (original) (raw)
Review
Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings
H P Horny et al. Leuk Res. 2001 Jul.
Abstract
An increase in mast cell (MC) numbers in hemopoietic tissues may be associated with (a) primary neoplastic MC disease (mastocytosis); (b) non-mast cell lineage myelogenous disorders (myelodysplastic or myeloproliferative syndromes and myeloid leukemias); or (c) reactive, i.e. non-clonal states (MC hyperplasia and reactive mastocytosis). However, the histologic discrimination between hyperplastic states and neoplastic MC proliferative disorders is sometimes very difficult. MC hyperplasia is characterized by a diffuse increase in mature, round or spindle-shaped, metachromatic MC that are loosely scattered throughout the tissue and do not form dense focal infiltrates, even in states of marked hyperplasia. However, loosely scattered MC are also a prominent feature of many cases of myelodysplastic syndromes and acute leukemia involving the MC lineage. In contrast, the demonstration of dense, focal and/or diffuse MC infiltrates can be regarded as indicative of primary MC disease/mastocytosis. In addition to the highly diagnostic focal MC infiltrates, mastocytosis may also present with a predominantly diffuse or a mixed (diffuse and focal) infiltration pattern. The relatively rare diffuse pattern is usually dominated by atypical, often hypogranulated or even non-metachromatic MC and is associated with the aggressive or frankly malignant subtypes of systemic mastocytosis and MC leukemia. Although the demonstration of MC infiltrates in Giemsa-stained tissue sections is still very important for the diagnosis of mastocytosis, immunohistochemical techniques using antibodies against MC-associated antigens such as tryptase or c-kit (CD117) are essential for the identification of highly atypical, hypogranulated MC, especially in MC leukemia, and for the detection of small and even minute MC infiltrates.
Similar articles
- Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis.
Valent P, Sperr WR, Samorapoompichit P, Geissler K, Lechner K, Horny HP, Bennett JM. Valent P, et al. Leuk Res. 2001 Jul;25(7):595-602. doi: 10.1016/s0145-2126(01)00040-6. Leuk Res. 2001. PMID: 11377685 Review. - Diagnostic criteria and classification of mastocytosis: a consensus proposal.
Valent P, Horny HP, Escribano L, Longley BJ, Li CY, Schwartz LB, Marone G, Nuñez R, Akin C, Sotlar K, Sperr WR, Wolff K, Brunning RD, Parwaresch RM, Austen KF, Lennert K, Metcalfe DD, Vardiman JW, Bennett JM. Valent P, et al. Leuk Res. 2001 Jul;25(7):603-25. doi: 10.1016/s0145-2126(01)00038-8. Leuk Res. 2001. PMID: 11377686 Review. - Mastocytosis: state of the art.
Horny HP, Sotlar K, Valent P. Horny HP, et al. Pathobiology. 2007;74(2):121-32. doi: 10.1159/000101711. Pathobiology. 2007. PMID: 17587883 Review. - Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-x(L).
Jordan JH, Walchshofer S, Jurecka W, Mosberger I, Sperr WR, Wolff K, Chott A, Bühring HJ, Lechner K, Horny HP, Valent P. Jordan JH, et al. Hum Pathol. 2001 May;32(5):545-52. doi: 10.1053/hupa.2001.24319. Hum Pathol. 2001. PMID: 11381374 - Utility of flow cytometric analysis of mast cells in the diagnosis and classification of adult mastocytosis.
Escribano L, Díaz-Agustín B, Bellas C, Navalón R, Nuñez R, Sperr WR, Schernthaner GH, Valent P, Orfao A. Escribano L, et al. Leuk Res. 2001 Jul;25(7):563-70. doi: 10.1016/s0145-2126(01)00050-9. Leuk Res. 2001. PMID: 11377681 Review.
Cited by
- Beyond Midostaurin: Role of Avapritinib in Managing Systemic Mastocytosis.
Pokima N, Khattar G, Keesari PR, Khan S, Asogwa N, Niazi M, Zheng R, Dai Q. Pokima N, et al. Cureus. 2024 May 12;16(5):e60161. doi: 10.7759/cureus.60161. eCollection 2024 May. Cureus. 2024. PMID: 38868249 Free PMC article. - Aggressive systemic mastocytosis with multiple organ involvement: a case report.
Iranijam E, Salimi M, Negaresh M, Javanshir N. Iranijam E, et al. Oxf Med Case Reports. 2023 Nov 28;2023(11):omad095. doi: 10.1093/omcr/omad095. eCollection 2023 Nov. Oxf Med Case Reports. 2023. PMID: 38033410 Free PMC article. - Drug delivery targets and strategies to address mast cell diseases.
Rische CH, Thames AN, Krier-Burris RA, O'Sullivan JA, Bochner BS, Scott EA. Rische CH, et al. Expert Opin Drug Deliv. 2023 Feb;20(2):205-222. doi: 10.1080/17425247.2023.2166926. Epub 2023 Jan 29. Expert Opin Drug Deliv. 2023. PMID: 36629456 Free PMC article. Review. - Histopathology and Molecular Genetics in Systemic Mastocytosis: Implications for Clinical Management.
Crupi F, Sordi B, Vanderwert F, Gesullo F, Amorosi A, Mannelli F, Santi R. Crupi F, et al. Int J Mol Sci. 2022 Aug 7;23(15):8772. doi: 10.3390/ijms23158772. Int J Mol Sci. 2022. PMID: 35955907 Free PMC article. - CDK4/CDK6 Inhibitors Synergize with Midostaurin, Avapritinib, and Nintedanib in Inducing Growth Inhibition in KIT D816V+ Neoplastic Mast Cells.
Schneeweiss-Gleixner M, Filik Y, Stefanzl G, Berger D, Sadovnik I, Bauer K, Smiljkovic D, Eisenwort G, Witzeneder N, Greiner G, Hoermann G, Schiefer AI, Schwaab J, Jawhar M, Reiter A, Sperr WR, Arock M, Valent P, Gleixner KV. Schneeweiss-Gleixner M, et al. Cancers (Basel). 2022 Jun 23;14(13):3070. doi: 10.3390/cancers14133070. Cancers (Basel). 2022. PMID: 35804842 Free PMC article.