RBC-Y/MCV as a discriminant function for differentiating carriers of thalassaemia and HbE from iron deficiency

P>Individuals with alpha-thalassaemia (ATT), beta-thalassaemia (BTT) and HbE trait (HET) are often initially identified based on haematological parameters. However, the values of these parameters usually overlap with iron deficiency anaemia (IDA) and anaemia of chronic disease (ACD). We evaluated the use of RBC-Y in 156 normal individuals and 332 patients; ATT (n = 37), BTT (n = 61), HET (n = 25), HbH disease (n = 5), ACD (n = 67), IDA (n = 83) and ACD with IDA (n = 54). Diagnostic efficiency was analysed by receiver operating characteristics (ROC). MCH was better compared with RBC-Y in discriminating normal from abnormal with sensitivity and specificity of 94% at a cut-off of 26 pg. The Green and King (G&K) index performed the best in discriminating carriers from IDA and ACD with area under the ROC curve (AUCROC) of 0.81. However, if ACD was excluded, RBC-Y/MCV was a good discriminator for carriers from IDA with AUCROC = 0.845. In general screening of populations with ATT, BTT and HET, we propose that hypochromic individuals be first identified by MCH < 26 pg and carriers distinguished within these hypochromic individuals from IDA by using RBC-Y/MCV. However, if the prevalence of ACD were high within the screening population, G&K index would be a more suitable discriminator

Serum free light chains: diagnostic and prognostic value in multiple myeloma

Background: Measurement of serum free light chains (FLCs) has recently become available for the diagnosis and monitoring of patients with plasma cell dyscrasias. The aim of this study was to investigate the performance of the serum FLC assay as a tumour marker by comparing FLC concentrations with serum protein electrophoresis (PE) results in the diagnosis of multiple myeloma (MM). In addition, we also evaluated the prognostic value of the baseline serum FLC ratio in patients with MM. Methods: We measured FLC concentrations and calculated the kappa/lambda (κ/λ) FLC ratios for three groups (control, polyclonal gammopathy and MM). Results: The FLC ratio at a cut-off threshold of 2.0 showed higher sensitivity and specificity compared with serum electrophoresis for the diagnosis of MM. We used the median FLC ratio of &#62;57.5 and &#60;0.04 for κ and λ secretors, respectively, for assessing survival. Survival was 30 months in patients with the κ/λ ratio of &#62;57.5 and &#60;0.04 compared to 47 months in patients with the ratio &#60;57.5 and &#62;0.04, indicating that more abnormal serum FLC ratios are associated with poorer survival (p&#60;0.011). Conclusions: Despite the limitations of the assay, the results of our study indicate that the FLC assay in combination with serum PE has an increased sensitivity in the diagnosis of MM. Also, baseline measurement of the κ/λ ratio provides prognostic information in these same patients. Clin Chem Lab Med 2009;47:1101–7.Peer Reviewe

Acquired Factor VIII Inhibitors: Three Cases

Acquired hemophilia A is a rare, but devastating bleeding disorder caused by spontaneous development of autoantibodies directed against coagulation factor VIII. In 40%-50% of patients it is associated with such conditions as the postpartum period, malignancy, use of medications, and autoimmune diseases; however, its cause is unknown in most cases. Acquired hemophilia A should be suspected in patients that present with a coagulation abnormality, and a negative personal and family history of bleeding. Herein we report 3 patients with acquired hemophilia A that had different underlying pathologies, clinical presentations, and therapeutic responses. Factor VIII inhibitor formation in case 1 occurred 6 months after giving birth; underlying disorders were not identified in cases 2 or 3. The bleeding phenotype in these patients’ ranged from no bleeding tendency with isolated prolongation of APTT (activated partial thromboplastin time) to severe intramuscular hematoma and hemarthrosis necessitating recombinant activated factor VII infusion and blood components transfusion. Variable responses to immunosuppressive treatment were also observed