ABSTRACT
Introduction: Beta thalassemia trait (BTT) must be differentiated from iron defi ciency anemia to avoid unnecessary iron therapy and for the prevention of thalassemia major by genetic counseling. In a tertiary care hospital, it is vital that the screening tool is not only sensitive but also specifi c so as to be cost effective and save time. Aim: The aim of this study was to evaluate the new Sehgal index and compare it to existing complete blood count-based indices for the best combination of sensitivity and specifi city to predict BTT. Materials and Methods: Study was done in 2 phases - Phase 1: A retrospective analysis of 1022 consecutive high-performance liquid chromatography (HPLC) cases from July 2008 to June 2011. Phase 2: A prospective analysis of 973 consecutive HPLC cases from July 1, 2011 to June 10, 2013 was done to confi rm the results of Phase 1 and the applicability of the new Sehgal index. Results: Prevalence of BTT was 28.8% (294/1022) and 25.39% (247/973) in Phase 1 and Phase 2, respectively. Receiver operating characteristic-area under the curve and Youden index was highest for new Sehgal index, followed by Mentzers index <14. The prospective study shows results similar to those in Phase 1 confi rming the superiority of the above two indices. Conclusion: Sehgal index and Mentzers index <14 showed the best combination of sensitivity and specifi city in predicting BTT. The best indices or combination can be used as a “validated fl ag rule” in the analyzer middleware program in a hospital for identifying suspected cases of BTT.
ABSTRACT
Large granular lymphocytes (LGL) leukemias are commonly of the T-cell or NKcell type. T-cell LGL leukemia is typically a disorder of mature CD3, CD8 and T-cell receptor TCR (TCR — T cell receptor)-αβ positive cytotoxic T-cells. Rare variants include TCRγδ+ variants and CD4+ TCRαβ+ cases. We report a case of each of these rare variants. An 83-year-old female presented with anemia and lymphocytosis with LGLs on peripheral smear. Six-color multiparametric fl owcytometric analysis showed expression of CD3, heterogeneous CD7, dim CD2 and TCRγδ and lacked expression of CD5, TCRαβ, CD56, CD4 and CD8. A fi nal diagnosis of TCRγδ+ T-cell LGL leukemia was made. Differentiation between TCRγδ+ T-cell LGL leukemia and other γδ+ T-cell malignancies is of utmost importance due to the indolent nature of the former as compared to the highly aggressive behavior of the latter. An 85-year-old male diagnosed with liposarcoma was identifi ed to have lymphocytosis during preoperative evaluation. Peripheral smear showed presence of LGLs. Flowcytometric immunophenotyping showed expression of TCRαβ, CD3, CD2, CD5, CD4, dim CD8, CD56 with aberrant loss of CD7 expression. Vβ repertoire analysis by fl owcytometry showed 97% cells with Vβ14 clonality. A fi nal diagnosis of TCRαβ+ CD4+ T-cell LGL leukemia was made. CD4+ T-cell large granular lymphocytic leukemias have an indolent, less aggressive course when compared to their CD8+ counterparts and are not necessarily associated with cytopenias. However, their association with secondary neoplasia (29% of the cases) warrants a high degree of suspicion in the diagnosis as also noted in the index case. Use of a wide panel of antibodies and newer modalities such as Vβ repertoire analysis helps in accurate subtyping of LGL leukemia
ABSTRACT
Background: There are several methods for counting platelets, of which the international fl ow reference method (IRM) is considered to be the gold standard. We compared the platelet count given by this method to the count given by automated analyzers using other methods, such as optical fl uorescence and impedance. Aims: The aim of this study is to compare the platelet counts obtained by Sysmex XE 2100 by Impedance (Sysmex-I), optical fl orescence (Sysmex-O) and reported (Sysmex-R) based on the switching algorithm and LH-750 by Impedance (LH-750) with the IRM in thrombocytopenic blood samples. To calculate the sensitivity, specifi city, positive predictive value (PPV) and negative predictive value (NPV) of various technologies at the clinically relevant transfusion thresholds of 10 × 109/l and 20 × 109/l. Materials and Methods: A total of 118 blood samples with platelet count of <50 × 109/l were selected for the study. Platelet counts of all samples were analyzed by all methods using the Sysmex analyzer, LH-750 and IRM in parallel within 6 h of collection. Statistical Analysis Used: Pearson correlation, bland Altman analysis, sensitivity and specifi city, PPV and NPV. Results and Conclusions: Sysmex-R had the least Bias and 95% limits of agreement (95%LA) range and thus correlated best with IRM values. LH-750 had a higher Bias compared to Sysmex-O and Sysmex-R, but a strikingly similar 95% LA ensures similar results in all three methods. In fact, in the oncology subset, it had the narrowest 95% LA, which made it the best performer in this subgroup. Of the three Sysmex results, Sysmex-I had the highest bias, widest 95% LA and highest potential risk of over transfusion. Hence, Sysmex-R and LH-750 were found to be reliable tools for estimation of platelet count in thrombocytopenic patients.
ABSTRACT
An elderly woman with a continuously bleeding small wound was investigated for the presence of antibodies to FVIII using activated partial time-based screening and confirmatory tests. A late acting coagulation factor inhibitor was detected. The same was characterised to be a low titre antibody against FVIII (5.2 Bethesda units). Cryoprecipitate infusions, corticosteroids and topical desmopressin were unsuccessful in controlling the bleeding. Addition of cyclophosphamide brought about stoppage of bleeding and disappearance of the autoantibody.