Comparison of clinical outcomes of several risk stratification tools in newly diagnosed AML patients: A real-world evidence in our current therapeutic era.

BACKGROUND OF THE STUDY: AML classification tools have been developed to stratify the risk at AML diagnosis. There is a need to evaluate these tools in the current therapeutic era. COHORT CHARACTERISTICS: In this retrospective study, we compared five classifiers: ELN 2017, ELN 2022, ALFA classifier, Papaemmanuil et al. classifier, and Lindsley et al. classifier, in a real-life cohort of 281 patients newly diagnosed for AML in Nice University Hospital. In our cohort median age was 68 years old, sex ratio was M/F 56%/44%, performance status was lower than 2 in 73.1% of patients, AML subtype was "De novo" in 71.5%, "secondary" in 22.4%, and "therapy-related" in 6.0% of patients. Intensive chemotherapy was used in 53.0% of patients, and non-intensive chemotherapy in 40.6% of patients. Molecular analysis was available in a large majority of patients and the main mutations found were NPM1 (22.7%), DNMT3A (17.4%), TP53 (13.1%), TET2 (12.4%), and FLT3-ITD (12.4%). RESULTS: In our findings, the comparison of overall survival between the three prognostic groups in the global cohort was statistically significant in all classifiers: ELN 2017 p < 0.0001, ELN 2022 p < 0.0001, ALFA classifier p < 0.0001, Papaemmanuil classifier p < 0.0001, Lindsley classifier p = 0.001. ELN 2017, ELN 2022, ALFA classifier, Papaemmanuil classifier, and Lindsley classifier were calculated respectively in 99%, 99%, 89%, 90%, and 89% of patients. CONCLUSIONS: Using Akaike's information criteria (AIC) to compare all five classifiers, ELN 2022 is the best classifier into younger and older patients and for prognosis.

Allogeneic stem cell transplantation as a curative therapeutic approach for VEXAS syndrome: a case report.

TO THE EDITOR: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described entity linked to somatic mutation of UBA1, encompassing inflammatory disorders and hematological malignancies. Patients experiments symptoms related to inflammatory manifestations on the skin, joints, lungs. Most patients are refractory to usual anti-inflammatory or immunosuppressive treatments. Half of them will develop hematological diseases, mostly myelodysplastic syndromes. VEXAS patients with hematological malignancies have a poor outcome and no curative option has been described so far. Because in the first reported cohort of VEXAS patients the UBA1 mutation was only found in hematopoietic stem cells but not in fibroblasts, we hypothesized that bone marrow transplantation would provide a cure for the disease. Here we report the case of a VEXAS patient who successfully received an allogeneic hematopoietic stem cell transplantation as a curative option.

Perls' Stain Guidelines from the French-Speaking Cellular Hematology Group (GFHC).

In order to standardize cellular hematology practices, the French-speaking Cellular Hematology Group (Groupe Francophone d'Hématologie Cellulaire, GFHC) focused on Perls' stain. A national survey was carried out, leading to the proposal of recommendations on insoluble iron detection and quantification in bone marrow. The criteria presented here met with a "strong professional agreement" and follow the suggestions of the World Health Organization's classification of hematological malignancies.

[Assessment of an immature platelet fraction (IFP%) in the diagnosis of thrombocytopenia]. / Valeur du pourcentage de plaquettes réticulées dans le diagnostic étiologique d'une thrombopénie.

Reticulated platelets are young platelets containing mRNA. They reflect the rate of thrombopoesis. The aim of this study was to evaluate the reliability of the percentage of reticulated platelets (IPF%) as a diagnostic test for thrombocytopenia pathogenesis. IPF% was measured using XE 2100 Sysmex. An IPF% reference range in 52 healthy individuals was established as 1-4.5% with a median 2.2%. In all the 13 patients with idiopathic thrombocytopenic purpura IPF% was increased (median 11.8, range 5.3-54.3%). Only 7 out of 18 patients with disseminated intravascular coagulation had high IPF% (median 5.4%, range 2.9-14.1%). Surprisingly, IPF% was increased in 17 out of 22 patients with acute leukaemia (median 9.7%, range 0.9-41.9%). In CIVD, IPF% values correlated with the severity of the illness. Increased values in acute leukaemia could not be explained by non specific staining but by delayed maturation of reticulated platelets. A high IPF% does not substantiate hyperdestructive thrombocytopenia but a diagnosis of idiopathic thrombocytopenic purpura should be questioned if IPF% is not raised.

[Erythrocytic parameters Sysmex in a case of severe haemolysis]. / Utilisation des paramètres érythrocytaires Sysmex dans un cas d'hémolyse sévère.

We are reporting a case of severe haemolytic anemia with cold agglutinins which combines several spurious determinations. It shows the usefulness of the new erythrocytic parameters of the XE 5000 Sysmex, specially: red blood cells with optical count (RBC-O), GR-He (intra-erythocytic hemoglobin) and R-MFV (most frequent volume). Optical red blood cells act as a substitute for red cells count instead of impedance red cells and R-MFV as a substitute for MCV (mean cell volume). The hematocrit (HCT) is corrected thanks to the following formula: HCT=(RBC-O X R- MFV)/1000. Free plasmatic hemoglobin is included in the measure of hemoglobin by the analyzer but is not available for tissue oxygenation. So, hemoglobin (HGB) has to be corrected by the means of GR- He thanks to the following formula: HGB=(GR He x RBC-O)/10.

Successful re-treatment of a relapsed V600E mutated HCL patient with low-dose vemurafenib.

Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder that accounts for 2% of all leukemia. Recent identification of the recurrent V600E BRAF mutation in a majority of HCL patients has led some teams to evaluate the clinical potential of vemurafenib, a BRAF V600 specific inhibitor in a limited number of refractory HCL patients. Recently, we published the case of an HCL patient successfully treated with a low dose of vemurafenib. Eight months after the ending of treatment this patient relapsed. We present here the successful retreatment of this patient with a second line of vemurafenib. Our data suggest for the first time that vemurafenib at the dose of 240 mg once a day could be sufficient to maintain a complete hematological remission after an initial induction treatment with low-dose vemurafenib (2 × 240 mg) daily without inducing major toxicity.