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Persistent peripheral blood monocytosis of at least 1\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L and a percentage of monocytes \u226510% of the circulating white blood cell count (WBC) are both prerequisite criteria for this diagnosis. CMML represents the prototype of \u201coverlapping\u201d myeloid neoplasms with concurrent myeloproliferative and myelodysplastic features. However, clinical presentation is heterogeneous, with cases showing prevailing \u201cdysplastic\u201d features and others a predominant \u201cproliferative\u201d phenotype. Accounting for this diversity, two variants of CMML are recognized: \u201cdysplastic\u201d CMML defined by WBC\u00a0&lt;\u00a013\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L and \u201cproliferative\u201d CMML with WBC\u00a0\u2265\u00a013\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L often showing features mimicking a myeloproliferative neoplasm. Although not an official WHO category, the \u201coligomonocytic\u201d variant of CMML is defined by relative monocytosis with an absolute monocyte count of 0.5\u20100.9\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L. It can be considered a \u201cpre\u2010phase,\u201d as it frequently anticipates the development of an overt, classic CMML. In an attempt at improving disease prognostication, the blast count based grading system for CMML of the WHO 2008 Classification has been expanded in 2016 to include a new \u201cCMML\u20100\u201d category. Lastly, the large body of knowledge on the molecular events occurring in CMML has been used to assist diagnosis and assess prognosis. Despite the step forwards, diagnosis of CMML still remains one of exclusion as no clinical, pathologic or molecular findings are specific for this disease. The current review brings insight into the spectrum of CMML and provides practical advice to approach suspected cases of CMML.<\/jats:p>","DOI":"10.1111\/ijlh.13145","type":"journal-article","created":{"date-parts":[[2019,12,16]],"date-time":"2019-12-16T13:55:42Z","timestamp":1576504542000},"page":"101-108","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["How I investigate chronic myelomonocytic leukemia"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3894-2365","authenticated-orcid":false,"given":"Valentina Fabiola Ilenia","family":"Sangiorgio","sequence":"first","affiliation":[{"name":"Department of Cellular Pathology The Royal London Hospital London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2160-9689","authenticated-orcid":false,"given":"Daniel A.","family":"Arber","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Chicago Chicago IL USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1660-7013","authenticated-orcid":false,"given":"Attilio","family":"Orazi","sequence":"additional","affiliation":[{"name":"Department of Pathology Paul L. 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