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Diagnosis of these disorders requires integration of clinical, laboratory, and morphological features. While their classification mostly remains unchanged compared to the revised 2016 WHO classification and the 2014 International Myeloma Working Group consensus, some changes in criteria and terminology were proposed in the 2022 International Consensus Classification (ICC) of mature lymphoid neoplasms. MGUS of IgM type is now divided into IgM MGUS of plasma cell type, precursor to the rare IgM MM and characterized by MM-type cytogenetics, lack of clonal B-cells and absence of <jats:italic>MYD88<\/jats:italic> mutation, and IgM MGUS, NOS including the remaining cases. Primary cold agglutinin disease is recognized as a new entity. MM is now formally subdivided into cytogenetic groups, recognizing the importance of genetics for clinical features and prognosis. MM with recurrent genetic abnormalities includes MM with <jats:italic>CCND<\/jats:italic> family translocations, MM with <jats:italic>MAF<\/jats:italic> family translocations, MM with <jats:italic>NSD2<\/jats:italic> translocation, and MM with hyperdiploidy, with the remaining cases classified as MM, NOS. For diagnosis of localized plasma cell tumors, solitary plasmacytoma of bone, and primary extraosseous plasmacytoma, the importance of excluding minimal bone marrow infiltration by flow cytometry is emphasized. Primary systemic amyloidosis is renamed immunoglobulin light chain amyloidosis (AL), and a localized AL amyloidosis is recognized as a distinct entity. This review summarizes the updates on plasma cell neoplasms and related entities proposed in the 2022 ICC.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Key points<\/jats:title>\n                <jats:p><jats:italic>\u2022 Lymphoplasmacytic lymphoma can be diagnosed with lymphoplasmacytic aggregates in trephine biopsies\u2009&lt;\u200910% of cellularity and evidence of clonal B-cells and plasma cells<\/jats:italic>.<\/jats:p>\n                <jats:p><jats:italic>\u2022 IgM MGUS is subdivided into a plasma cell type and a not otherwise specified (NOS) type.<\/jats:italic><\/jats:p>\n                <jats:p><jats:italic>\u2022 Primary cold agglutinin disease is recognized as a new entity<\/jats:italic>.<\/jats:p>\n                <jats:p><jats:italic>\u2022 The term \u201cmultiple myeloma\u201d replaces the term \u201cplasma cell myeloma\u201d used in the 2016 WHO classification<\/jats:italic>.<\/jats:p>\n                <jats:p><jats:italic>\u2022 Multiple myeloma is subdivided into 4 mutually exclusive cytogenetic groups and MM NOS<\/jats:italic>.<\/jats:p>\n                <jats:p><jats:italic>\u2022 Minimal bone marrow infiltration detected by flow cytometry is of major prognostic importance for solitary plasmacytoma of bone and to a lesser extent for primary extraosseous plasmacytoma<\/jats:italic>.<\/jats:p>\n                <jats:p><jats:italic>\u2022 Localized IG light chain amyloidosis is recognized as a separate entity, distinct from systemic immunoglobulin light chain (AL) amyloidosis<\/jats:italic>.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00428-022-03431-3","type":"journal-article","created":{"date-parts":[[2022,11,22]],"date-time":"2022-11-22T21:32:45Z","timestamp":1669152765000},"page":"163-177","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":68,"title":["Plasma cell neoplasms and related entities\u2014evolution in diagnosis and classification"],"prefix":"10.1007","volume":"482","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5496-293X","authenticated-orcid":false,"given":"Falko","family":"Fend","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmet","family":"Dogan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James R.","family":"Cook","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,11,21]]},"reference":[{"key":"3431_CR1","doi-asserted-by":"publisher","first-page":"5113","DOI":"10.1182\/bloodadvances.2022007608","volume":"6","author":"C Agbuduwe","year":"2022","unstructured":"Agbuduwe C, Iqbal G, Cairns D, Menzies T, Dunn J, Gregory W, Kaiser M, Owen R, Pawlyn C, Child JA, Davies F, Morgan GJ, Jackson GH, Drayson MT, Basu S (2022) Clinical characteristics and outcomes of IgD myeloma: experience across UK national trials. 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