Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Jun 18;4(3):142-149.
doi: 10.36401/JIPO-21-1. eCollection 2021 Aug.

Chronic Myelomonocytic Leukemia: Hematopathology Perspective

Siba El Hussein  1 , Sa A Wang  1 , Naveen Pemmaraju  2 , Joseph D Khoury  1 , Sanam Loghavi  1
Affiliations
Review

Chronic Myelomonocytic Leukemia: Hematopathology Perspective

Siba El Hussein et al. J Immunother Precis Oncol. .

Abstract

Our understanding of chronic myelomonocytic leukemia (CMML) has evolved tremendously over the past decade. Large-scale sequencing studies have led to increased insight into the genomic landscape of CMML and clinical implications of these changes. This in turn has resulted in refined and improved risk stratification models, which to date remain versatile and subject to remodeling, as new and evolving studies continue to refine our understanding of this disease. In this article, we present an up-to-date review of CMML from a hematopathology perspective, while providing a clinically practical summary that sheds light on the constant evolution of our understanding of this disease.

Keywords: ASXL1; CMML; MDS; MPN; NRAS; SRSF2; chronic myelomonocytic leukemia; monocyte; myelodysplastic; myelproliferative.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest: Naveen Pemmaraju: Consulting/honorarium from Pacylex Pharmaceuticals, Incyte, Novartis, LFB Biotechnologies, Stemline, Celgene, AbbVie, MustangBio, Roche Diagnostics, Blueprint Medicines, and DAVA Oncology; research funding/clinical trials support from Affymetrix, SagerStrong Foundation, Novaris, Stemline, Samus, AbbVie, Cellectis, Daiichi Sankyo, and Plexxikon.

Figures

Figure 1
Figure 1
A systematic diagnostic approach to suspected chronic myelomonocytic leukemia (CMML) cases: After documentation of persistent monocytosis, the next step is to exclude nonneoplastic conditions including reactive, infectious, and autoimmune causes. A thorough morphologic-imunophenotypic evaluation of bone marrow specimens, integrating cytogenetic and molecular findings, is recommended to deliver the most accurate diagnosis. *Can also be performed on peripheral blood. **In cases with eosinophilia or abnormal mast cell aggregates. FISH: fluorescence in situ hybridization; NGS: next-generation sequencing; RT-PCR: real-time polymerase chain reaction.
Figure 2
Figure 2
An example of chronic myelomonocytic leukemia demonstrating (a) hypercellular bone marrow with myemolonocytic hyperplasia; (b) granulocytic and erythroid dysplasia; (c) megakaryocytic dysplasia with increased monolobated megakaryocytes; (d) this case showed increased ring sideroblasts and harbored an SF3B1 mutation.
Figure 3
Figure 3
Recurrent mutations and their frequencies and respective functions in chronic myelomonocytic leukemia. *Associated with adverse prognosis in various studies. CMML: chronic myelomonocytic leukemia; mRNA: messenger RNA.
Figure 4
Figure 4
Evolution of prognostic models for chronic myelomonocytic leukemia (CMML) over time. Illustration created with BioRender.com. BM: bone marrow; CMML: chronic myelomonocytic leukemia; CPSS: CMML-specific prognostic scoring system; MD-CMML: myelodysplastic chronic myelomonocytic leukemia; MDAPS: MD Anderson Prognostic Scoring System; MP-CMML: myeloproliferative chronic myelomonocytic leukemia; WCB: white blood cell.
See this image and copyright information in PMC

References

    1. Swerdlow SH, Campo E, Harris NL, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th ed International Agency for Research on Cancer. 2017.
    1. Solal-Celigny P, Desaint B, Herrera A, et al. Chronic myelomonocytic leukemia according to FAB classification: analysis of 35 cases. Blood . 1984;63:634–638. - PubMed
    1. Germing U, Gattermann N, Minning H, et al. Problems in the classification of CMML–dysplastic versus proliferative type. Leuk Res . 1998;22:871–878. - PubMed
    1. Dinmohamed AG, van Norden Y, Visser O, et al. The use of medical claims to assess incidence, diagnostic procedures and initial treatment of myelodysplastic syndromes and chronic myelomonocytic leukemia in the Netherlands. Leuk Res . 2015;39:177–182. - PubMed
    1. McCullough KB, Patnaik MM. Chronic myelomonocytic leukemia: a genetic and clinical update. Curr Hematol Malig Rep . 2015;10:292–302. - PubMed

LinkOut - more resources