Spectrum of autoimmune diseases and systemic inflammatory syndromes in patients with chronic myelomonocytic leukemia

Zahid, Mohammad Faizan; Barraco, Daniela; Lasho, Terra L.; Finke, Christy; Ketterling, Rhett P.; Gangat, Naseema; Hanson, Curtis A.; Tefferi, Ayalew; Patnaik, Mrinal M.

doi:10.1080/10428194.2016.1243681

Cited by 51 publications

(45 citation statements)

References 15 publications

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“…The three false negatives showed a specific abnormal profile, drawing a “bulbous aspect” on the CD14/CD16 dot plot, related to an increase of the iMo subset combined to the disappearance of the ncMo population, which has been recently described in CMML patients with an inflammatory state 11 . Concurrent cases of autoimmune diseases and/or systemic inflammatory syndromes have been reported in 20% of CMML patients 27 . In such cases, an increase of the iMo subset can lead to an underestimation of the relative cMo percentage, which drops below the 94% threshold 11 .…”

Section: Discussionmentioning

confidence: 99%

Multicenter validation of the flow measurement of classical monocyte fraction for chronic myelomonocytic leukemia diagnosis

Tarfi

Harrivel

Dumézy

et al. 2018

Blood Cancer Journal

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Peripheral blood monocytes include three subsets defined by CD14 and CD16 surface markers. An increase in the CD14++CD16− classical monocyte fraction ≥ 94% of the total monocytes was proposed to rapidly and efficiently distinguish chronic myelomonocytic leukemia from reactive monocytosis. The robustness of this assay required a multicenter validation. The flow cytometry assay designed to quantify peripheral blood monocyte subsets was implemented by multiple diagnosis laboratories in France. A nationwide survey was performed to evaluate its performance. All the 48 French laboratories answered the questionnaire, revealing that 63% use this assay routinely. Central blind reanalysis of 329 cytometry files collected from five laboratories demonstrated an excellent correlation in classical monocyte fraction measurement (r = 0.93; p < 0.0001). The cutoff value of 94% classical monocytes being the critical readout for diagnosis, we then compared 115 patients with classical monocytes ≥ 94% and 214 patients with a fraction < 94% between initial analysis and reanalysis. An agreement was obtained in 311 files. Finally, an overt diagnosis, available for 86 files, confirmed a good sensitivity (93.6%) and specificity (89.7%). This survey demonstrates the robustness of the flow assay with limited variability of classical monocyte percentage between centers, validates the 94% cutoff value, and confirms its sensitivity and specificity.

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Section: Discussionmentioning

confidence: 99%

Multicenter validation of the flow measurement of classical monocyte fraction for chronic myelomonocytic leukemia diagnosis

Tarfi

Harrivel

Dumézy

et al. 2018

Blood Cancer Journal

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“…Approximately 30% of CMML patients can present with antecedent or concomitant autoimmune diseases (rheumatoid arthritis, psoriasis, etc.) and poorly defined systemic inflammatory syndromes . Rarely, CMML can present with leukemia cutis as an initial manifestation, or directly present with blast transformation (CMML‐BT) …”

Section: Disease Overviewmentioning

confidence: 99%

Chronic Myelomonocytic leukemia: 2020 update on diagnosis, risk stratification and management

2019

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Disease overview: Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder with overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms, with an inherent risk for leukemic transformation (~15% over 3-5 years). Diagnosis: Diagnosis is based on the presence of sustained (>3 months) peripheral blood monocytosis (≥1 × 10 9 /L; monocytes ≥10%), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in~30% of patients, while >90% have gene mutations. Mutations involving TET2 (~60%), SRSF2 (~50%), ASXL1 (~40%) and the oncogenic RAS pathway (~30%) are frequent; while the presence of ASXL1 and DNMT3A mutations and the absence of TET2 mutations negatively impact over-all survival. Risk stratification: Molecularly integrated prognostic models include; the Groupe Français des Myélodysplasies (GFM), Mayo Molecular Model (MMM) and the CMML specific prognostic model (CPSS-Mol). Risk factors incorporated into the MMM include presence of nonsense or frameshift ASXL1 mutations, absolute monocyte count>10 × 10 9 /L, hemoglobin <10 g/dL, platelet count <100 × 10 9 /L and the presence of circulating immature myeloid cells. The MMM stratifies CMML patients into four groups; high (≥3 risk factors), intermediate-2 (2 risk factors), intermediate-1 (1 risk factor) and low (no risk factors), with median survivals of 16, 31, 59 and 97 months, respectively.Risk-adapted therapy: Hypomethylating agents such as 5-azacitidine and decitabine are commonly used, with overall response rates of~40%-50% and complete remission rates of~7%-17%; with no impact on mutational allele burdens. Allogeneic stem cell transplant is the only potentially curative option, but is associated with significant morbidity and mortality.

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“…The clinical spectrum of these AIDs, notably including immune thrombocytopenia or seronegative arthritis, is somewhat distinct from that associated to MDS, with a higher frequency of systemic vasculitis [30,31]. These AIDs can be treated conventionally with steroids, and hypomethylating agents (HMA) can also prove useful [32].…”

Section: Clinical Presentationmentioning

confidence: 99%

CMML: Clinical and molecular aspects

et al. 2017

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Spectrum of autoimmune diseases and systemic inflammatory syndromes in patients with chronic myelomonocytic leukemia

Cited by 51 publications

References 15 publications

Multicenter validation of the flow measurement of classical monocyte fraction for chronic myelomonocytic leukemia diagnosis

Multicenter validation of the flow measurement of classical monocyte fraction for chronic myelomonocytic leukemia diagnosis

Chronic Myelomonocytic leukemia: 2020 update on diagnosis, risk stratification and management

CMML: Clinical and molecular aspects

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