Ultrasound Classification of Finger Pulp Blood Flow in Patients With Systemic Sclerosis: A Pilot Study.

OBJECTIVE: To define a semiquantitative classification of finger pulp blood flow (FPBF) and to evaluate whether this classification could be used to assess FPBF in healthy controls and in systemic sclerosis (SSc) patients. METHODS: Thirty controls and 86 SSc patients were consecutively included. A classification of FPBF including 5 grades (from grade 0 [no signal] to 4 [signal detected on the entire finger pulp, including the subepidermal vascular network]) was evaluated. This classification was explored in basal conditions and after hand baths in hot and cold water in controls. Its relevance was also assessed at room temperature in SSc patients. RESULTS: In controls, power Doppler ultrasonography (PDUS) of FPBF was improved after hot challenge (P = 0.024), whereas cold challenge decreased FPBF (P = 0.001). FPBF correlated with the vasodilation status assessed by the resistivity index of radial arteries (Spearman's correlation coefficient = -0.50, P = 0.0049). Grade 0 was more frequent in SSc patients than in controls (22.1% versus 3.3%; P < 0.05). In SSc patients, grade 0 was associated with severity markers of the digital vasculopathy such as digital ulcers (DUs) (current or past) (P < 0.05) or ulnar artery occlusion (P < 0.05). On the other hand, DUs were less frequent in patients with grade 4 (P < 0.05). A pathologic threshold of <2 (grade 0 or 1) was significantly associated with DUs (odds ratio 6.67 [95% confidence interval 2.31-19.21], P < 0.0001). CONCLUSION: PDUS allowed a semiquantitative evaluation of FBPF in SSc patients and controls. Further studies are warranted to validate these results in independent SSc populations and to compare PDUS to existing tools assessing digital blood flow.

[Troponin T cardiac analysis: clinical cases of the limits of its cardiospecificity]. / Illustrations cliniques d'un manque de cardiospécificité du dosage de troponine T hypersensible.

INTRODUCTION: Myositis are systemic diseases, in which heart damage is possible. Cardiac troponin T is often found to be defective to detect cardiac involvement. OBSERVATION: We report cases of two patients with a myositis. Diagnosis was retained based on muscle pain, increase in serum creatinine kinase, and inflammatory muscle damage on MRI. Histology confirmed the diagnosis for one of the two patients. Cardiac troponin T was measured in both patients, to detect myocardial involvement. Despite a serum elevation of this marker, cardiological assessment remained negative (electrocardiogram, cardiac ultrasound, cardiac MRI). Cardiac troponin I was normal in serum because of the absence of correlation with peripheral muscle involvement. CONCLUSION: Cardiac troponin T is correlated with muscle involvement in patients with myositis. Cardiac troponin I should be preferred because of a better specificity.

Prognostic value of involved/uninvolved free light chain ratio determined by Freelite and N Latex FLC assays for identification of high-risk smoldering myeloma patients.

Background Smoldering multiple myeloma (SMM) is an asymptomatic plasma cell disorder with a high risk of progression to symptomatic multiple myeloma (MM). The serum free light chain (sFLC) ratio is a powerful prognostic factor for SMM: an sFLC ratio ≥8 has been reported to be associated with a high risk of progression to MM, and an sFLC ratio ≥100 has been described as a criterion for ultra-high-risk SMM, and has been integrated into the definition criteria for MM since 2014. However, all recommendations were based on sFLC measured using the first commercialized assay, Freelite™, while other assays are now available. We aimed to evaluate the safety and accuracy of N-Latex sFLC to identify high-risk and ultra-high-risk SMM. Methods The sFLC ratio was measured at diagnosis with both Freelite and N-Latex assays in a cohort of 176 SMM patients on a BN Prospec nephelometer. Demographic, clinical, therapeutic and laboratory data were collected at the time of diagnosis and at follow-up. Results Sixty-two patients (35.2%) progressed to MM within 2 years. Compared to Freelite™ sFLC, N Latex sFLC ratios ≥8 and ≥100 provided similar performances for the identification of high-risk and ultra-high risk SMM patients. Conclusions Our results evidenced that the N-Latex assay could be used for SMM monitoring, like Freelite. However, an N-Latex sFLC ratio ≥70 appears to provide similar performances to a Freelite sFLC ratio ≥100, with a slightly better positive predictive value. Both assays provided accurate identification of high-risk and ultra-high risk SMM patients. These results should be confirmed in an independent study.

Evaluation of the Impact of Renal Failure on Correlation and Concordance Between 2 Free Light Chain Assays.

BACKGROUND: Free light chain (FLC) assays are essential for diagnosis and follow-up of plasma cell dyscrasia. Two assays are available: Freelite (Binding Site) and N Latex FLC (Siemens). The aim of our study was to evaluate the impact of renal failure on concordance and correlation between the 2 FLC assays. METHODS: FLC measurements using both assays were performed on 1215 fresh serum samples from patients with or without monoclonal gammopathy and renal failure. Concordance and correlation were evaluated using Passing-Bablock regression, Pearson correlation coefficient, and the Cohen kappa coefficient, taking into account the renal failure stage (evaluated with Chronic Kidney Disease-Epidemiology Collaboration formulae) and evaluation of treatment response in patients' follow-up. RESULTS: A good correlation was demonstrated between both assays, irrespective of the renal failure stage (Pearson correlation coefficient > 0.90). For FLC ratio interpretation, there remained 7.6% to 20.8% discordances between the 2 methods throughout the whole range of renal impairment. To evaluate FLC evolution in patient follow-up, 41 patients were selected with at least 6 consecutive serum samples being collected during the study period: we observed a concordant evolution of FLC concentrations between both assays. However, few discrepancies were observed with 4 patients. CONCLUSIONS: Despite adjusted reference ranges for Freelite FLC ratio, there are approximately 12.5% discrepancies in interpretation of FLC ratio between the 2 available assays. They are not linked to renal failure stage and neither of the assays performed better than the other: results must be interpreted taking into account clinical data and the same assay must be used for patient follow-up.

LRP overexpression in monocytic lineage.

The failure to chemotherapy is a multi factorial phenomenon and lung resistance protein (LRP) overexpression has already been discussed as implicated in drug resistance. But its role is still discussed. In 1996, we studied the expression of LRP and P170 (MDR) in a series of leukemias, at the time of diagnosis, by immunocytochemical (ICC) method. The observation of a strong and unusual expression of LRP in acute myeloid leukemia (AML) with monocytic component led us to test (for P170 and LRP) a new series of 47 AML with different FAB subtypes. The expression of LRP was scored from 1 to 5 in blast cells and monocytes separately. We demonstrate that LRP is not correlated with clinical outcome but is statistically related to monoblastic leukemias. Code 5 reaction was found in 10/13 M5 versus the other FAB subtypes (P<10(-3)). The strongest LRP overexpression was also found in chronic myelomonocytic leukemia (four cases), reactive monocytosis (three cases) and in a dendritic cell line. In conclusion, we report that LRP is rather a marker of monocytic lineage than a prognostic index for MDR and we suggest that detection of LRP by ICC could be an argument for the diagnosis of monoblastic and monocytic leukemias.

Localized epidural and bone amyloidosis, rare cause of paraplegia in multiple myeloma.

We report two cases of spinal cord compression by AL amyloid deposits in the setting of multiple myeloma. The first patient, a 57 year old man, had a surgical procedure late in his course, when his neurological status worsened despite medical treatment. The second patient, a 61 year old man, had surgical treatment as soon as vertebral body collapse and epiduritis were diagnosed and spinal amyloidosis revealed a non secretory myeloma. Neurological recovery was significant in both cases and neither developed manifestations of systemic amyloidosis 4 years later.