Evaluation of the International Society on Thrombosis & Haemostasis scoring system & its modifications in diagnosis of disseminated intravascular coagulation: A pilot study from southern India

Background & objectives: Diagnosis of disseminated intravascular coagulation (DIC) rests primarily on the clinical profile along with supportive laboratory tests. The International Society on Thrombosis and Haemostasis (ISTH) had proposed a scoring system for the diagnosis of overt DIC. However, fibrinogen values which are supposed to be low are often found to be elevated due to the associated inflammation seen in some cases. Moreover, peripheral smear is known to show schistocytes, which is also not included in the score. This study was done to evaluate ISTH scoring system and its modifications in suspected DIC. Methods: Fifty-six patients were enrolled for the present study of whom; in four, fibrinogen assay could not be done. Modifications in the ISTH scoring with the exclusion of fibrinogen, i.e. modified ISTH (MI) score and subsequent inclusion of schistocytes, i.e. modified ISTH with schistocytes (MIS) score, were used. The modified scores were analyzed for diagnostic accuracy parameters and agreement with ISTH score. Results: Amongst 56 cases, 9/52 (17.3%), 22 (39.3%) and 17 (30.4%) were diagnosed as positive for overt DIC by ISTH, MI and MIS scores and mortality was 33, 22.7 and 17.6 per cent, respectively. The sensitivity, specificity, positive and negative predictive values for the MI score were 100, 74.4, 45 and 100 per cent and for MIS score were 100, 86, 60 and 100 per cent, respectively. The agreement between MI score and MIS score with ISTH score was moderate [?=0.502, 95% confidence interval (CI): 0.272-0.732, P<0.001] and substantial (?=0.681, 95% CI: 0.45-0.91, P<0.001). Interpretation & conclusions: In the present study, the calculated mortality was highest by ISTH score. Best agreement was between MIS score and ISTH score. In a resource-constrained setup where fibrinogen assay and therefore ISTH score is difficult, it is suggested that MIS score can be considered.

Diagnóstico diferencial de las anemias hemolíticas / Differential diagnosis of hemolytic anemias

Introducción: El término hemólisis hace referencia a la destrucción de los eritrocitos y ocurre en un amplio rango de condiciones clínicas fisiológicas y patológicas. Es empleado para definir situaciones en la que la vida media de los eritrocitos está disminuida por causas mecánicas, tóxicas, autoinmunes o infecciosas. Objetivo: Describir los principales marcadores de hemólisis que se encuentran variablemente alterados en las diferentes formas de anemias hemolíticas. Métodos: Se realizó una revisión de la literatura, en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google Académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la información. Análisis y síntesis de la información: La hemoglobina es el marcador más directo de la gravedad clínica en las enfermedades hemolíticas. Sus valores pueden estar muy próximos a los valores de referencia en las formas ligeras (Hb > 100 g/L) o significativamente reducidos en las moderadas (Hb entre 80-100 g/L), graves (Hb entre 60-80 g/L) y muy graves (Hb < 60 g/L). Sin embargo, existen otros marcadores esenciales para diferenciar las formas de presentación aguda y crónica, la hemólisis extravascular de la intravascular y la presencia de signos extrahematológicos tales como: los reticulocitos y esquistocitos, la deshidrogenasa láctica, la haptoglobina, la bilirrubina, la ferritina y la hemosiderinuria. Conclusiones: Los parámetros hemolíticos pueden estar diferencialmente alterados en varias condiciones lo cual ayuda en la realización del diagnóstico diferencial de las anemias hemolíticas(AU)

Introduction: The term hemolysis refers to the destruction of erythrocytes, a process occurring in a wide range of physiological and pathological clinical conditions. The term is used to define situations in which mean erythrocyte lifespan is reduced due to mechanical, toxic, autoimmune or infectious causes. Objective: Describe the main markers of hemolysis found to be variably altered in the different forms of hemolytic anemias. Methods: A review was conducted of the literature about the topic published in English and Spanish in the website PubMed and the search engine Google Scholar in the last 10 years. Data were analyzed and summarized. Data analysis and synthesis: Hemoglobin is the most direct marker of clinical severity in hemolytic diseases. Its values may be very close to reference levels in mild disease (Hb > 100 g/l), whereas they will be significantly reduced in moderate (Hb 80-100 g/l), severe (Hb 60-80 g/l) and very severe disease (Hb < 60 g/l). However, other markers are also essential to distinguish acute from chronic presentation, extravascular from intravascular hemolysis, and the presence of extrahematological signs such as reticulocytes and schistocytes, lactate dehydrogenase, haptoglobin, bilirubin, ferritin and hemosiderinuria. Conclusions: Differentially altered hemolytic parameters may be found in several conditions, which makes them useful for the differential diagnosis of hemolytic anemias(AU)

Congenital Thrombotic Thrombocytopenic Purpura: An Unusual Case of Microangiopathic Haemolytic Anaemia in a Newborn.

We present an extremely rare case of hyperbilirubinemia with rapid progression leading to bilirubin encephalopathy in term neonate. Despite early recognition and intervention, death occurred as a total serum bilirubin reached 25 mg/dl. It was a case of Coomb’s negative microangiopathic haemolytic anaemia in a newborn period which is autosomal recessive inheritance i.e. Upshaw-Schulman Syndrome. (Congenital thrombotic thrombocytopenic purpura) characterised by numerous schistocytes on peripheral blood smear, thrombocytopenia , increased reticulocyte count, increased bilirubin and LDH level. This rare disease is often misdiagnosed especially in newborn baby. So we present this case not only for its variety but also for to create more awareness among pathologist and paediatrician as treatment protocol entirely differ.

Development of a Poikilocyte Measuring Method Using Image Analysis Software

BACKGROUND: To achieve consistency in poikilocytes grading in peripheral blood cell examinations, we made an image-based differential count (IDC) software to measure the degree of abnormalities in individual red blood cells (RBCs) and relative fractions of poikilocytes. METHODS: Thirty peripheral blood samples were analyzed. Smear slides were examined on a microscope with charge-coupled device (CCD) camera. To verify this program, we compared the IDC results with the results of manual differential counting (MDC). Relative fractions of schistocytes, echinocytes, and elliptocytes were measured by IDC and MDC. The error rate of IDC was measured by confirming the final processed images of IDC. Correlations of IDC and MDC results were compared using linear regression analysis and the time required for each test was measured. For presentation of the mathematical decision criteria of poikilocytes, IDC algorithms for recognizing schistocytes, echinocytes, and elliptocytes were made using simple geometrical or mathematical formulas. RESULTS: The error rate of IDC was 2.8%. For analysis of 1,000 RBCs, MDC took 7.3 minutes and IDC took 2.7 minutes. Linear regression coefficients were 0.776 (P<0.001) for schistocytes, 0.895 (P<0.001) for echinocytes, and 1.001 (P<0.001) for elliptocytes. CONCLUSIONS: It was possible to define poikilocytes with geometrical or mathematical formulas using image analysis programs. The IDC program would be helpful for consistent grading of poikilocytes.