Impact of Mutational Status and Prognostic Factors on Survival in Chronic Myelomonocytic Leukemia With Systemic Inflammation and Autoimmune Disorders

Dussiau, Charles; Dupuy, Henry; Bidet, Audrey; Sauvezie, Mathieu; De-Grande, Anne-Charlotte; Boureau, Lisa; Rivière, Étienne; Forcade, Édouard; Bonnet, Fabrice; Dumas, Pierre‐Yves; Duffau, Pierre; Pigneux, Arnaud; Viallard, Jean‐François; Dimicoli‐Salazar, Sophie; Lazaro, Estibaliz

doi:10.1097/hs9.0000000000000847

HemaSphere

2023

DOI: 10.1097/hs9.0000000000000847

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Impact of Mutational Status and Prognostic Factors on Survival in Chronic Myelomonocytic Leukemia With Systemic Inflammation and Autoimmune Disorders

Charles Dussiau

Henry Dupuy

Audrey Bidet

et al.

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Cited by 4 publications

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“…Quantification of reticulated platelets (i.e., immature platelet fractions) has been attempted in ITP with inconclusive results [ 7 ], but did not provide insight into the mechanism of thrombocytopenia in MDS/CMML [ 8 ]. Notably, CMML was the main disease associated with peripheral destruction of platelets in our study population, as suggested previously [ 2 , 9 ]. Furthermore, we showed that a bone marrow smear is probably not useful for diagnosis of the mechanism of thrombocytopenia under these conditions [ 10 ].…”

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confidence: 81%

Utility of indium-111 platelet scintigraphy for understanding the mechanism of thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Durand

Pottier²,

Mesguich

et al. 2023

Exp Hematol Oncol

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Background Thrombocytopenia occurs in 60% of patients with myelodysplastic syndromes (MDS), increasing the risk of life-threatening haemorrhage in this population of mainly old patients with comorbidities. However, data are scare regarding immune thrombocytopenia (ITP) secondary to MDS. Aim We analyzed the utility of indium-111 platelet scintigraphy (IPS) to better characterize the mechanisms of thrombocytopenia in 21 adult patients with MDS. Methods Adult patients with a definite diagnosis of MDS according to the international criteria who underwent IPS between 2009 and 2018 because of an increased bleeding risk were retrospectively selected. Autologous 111Indium platelet labelling was performed with a technique similar to that described previously using a standardized method. Results Platelet lifespan ≤ 6 days identified patients with peripheral platelet destruction. Taking into account the response to ITP-directed therapies after IPS, the sensitivity, specificity, and positive and negative predictive values of IPS were 100%, 84.6%, 80%, and 100%, respectively. Conclusion We show that IPS can be a useful tool to identify the mechanism and guide treatment of a chronic thrombocytopenia increasing the bleeding risk in patients with MDS.

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supporting

confidence: 81%

Utility of indium-111 platelet scintigraphy for understanding the mechanism of thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Durand

Pottier²,

Mesguich

et al. 2023

Exp Hematol Oncol

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Chronic myelomonocytic leukaemia causing orbital inflammation

McGrath,

Fay,

McAnena

2024

BMJ Case Rep

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We present a case of acute-onset orbital inflammation with rapidly progressive proptosis, episcleral venous stasis with raised intraocular pressure and loss of vision in a patient with a recent diagnosis of chronic myelomonocytic leukaemia (CMML). The patient’s orbital inflammation and ocular hypertension showed no response to topical and systemic pressure-lowering agents and non-steroidal anti-inflammatory agents but resolved rapidly after the commencement of intravenous steroids. The patient was subsequently treated with the hypomethylating agent azacitidine with good systemic control of CMML with no further orbital inflammation. CMML is strongly associated with systemic inflammatory disease, possibly due to the upregulation of inflammatory pathways in the abnormal monocytes. CMML is a rare cause of orbital or ocular inflammation but should be considered in patients with persistent monocytosis.

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Autoinflammation in patients with leukocytic CBL loss of heterozygosity is caused by constitutive ERK-mediated monocyte activation

Bohlen,

Bagarić,

Vatovec

et al. 2024

Journal of Clinical Investigation

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Patients heterozygous for germline CBL loss-of-function (LOF) variants can develop myeloid malignancy, autoinflammation, or both, if some or all of their leukocytes become homozygous for these variants through somatic loss of heterozygosity (LOH) via uniparental isodisomy. We observed an upregulation of the inflammatory gene expression signature in whole blood from these patients, mimicking monogenic inborn errors underlying autoinflammation. Remarkably, these patients had constitutively activated monocytes that secreted 10 to 100 times more inflammatory cytokines than those of healthy individuals and CBL LOF heterozygotes without LOH. CBL -LOH hematopoietic stem and progenitor cells (HSPCs) outgrew the other cells, accounting for the persistence of peripheral monocytes homozygous for the CBL LOF variant. ERK pathway activation was required for the excessive production of cytokines by both resting and stimulated CBL -LOF monocytes, as shown in monocytic cell lines. Finally, we found that about 1 in 10,000 individuals in the UK Biobank were heterozygous for CBL LOF variants and that these carriers were at high risk of hematological and inflammatory conditions.

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Impact of Mutational Status and Prognostic Factors on Survival in Chronic Myelomonocytic Leukemia With Systemic Inflammation and Autoimmune Disorders

Cited by 4 publications

References 14 publications

Utility of indium-111 platelet scintigraphy for understanding the mechanism of thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Utility of indium-111 platelet scintigraphy for understanding the mechanism of thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Chronic myelomonocytic leukaemia causing orbital inflammation

Autoinflammation in patients with leukocytic CBL loss of heterozygosity is caused by constitutive ERK-mediated monocyte activation

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