Annamma Kurien

Correlation of morphologic and cytochemical diagnosis with flowcytometric analysis in acute leukemia

INTRODUCTION The classification of acute leukemias has revolutionized over the years. Immunophenotyping of acute leukemia has gained popularity because of its influence on treatment and prognosis of the disease. The various antigens expressed by the leukemic cells can be assessed by flowcytometry (FCA) and can be used in rendering specific treatment and predicting the outcome of the different types of acute leukemia. AIMS The main aim of this study was to compare the morphologic and cytochemical diagnoses with flowcytometric diagnoses in acute leukemia and to analyze the usefulness of FCA over morphology. RESULTS In this study we analyzed 50 cases of acute leukemia and found concordance rate as high as 86% between morphologic/cytochemical diagnosis and flowcytometric diagnosis. Of these, complete concordance was seen in 58% of the cases and partial concordance was seen in 22% of the cases. Non-concordance was seen in only 4% of our cases. In remaining 16% of our cases FCA helped in sub classifying the acute leukemia where morphology and cytochemistry had failed to do so. CD19 and 20 were found to be consistent B-cell markers and CD3 was a very specific marker for T-cell leukemia. CD13 and 33 were important myeloid markers and were aided by other secondary panel of markers like CD14, CD117 and CD41. CONCLUSION FCA not only helps in confirming morphologic diagnosis in acute leukemia but also helps in assigning specific lineage to the blasts, particularly in acute lymphoid leukemia. Immunophenotyping is of utmost importance in classifying acute leukemia as it greatly influences the treatment and the prognosis.

Monocyte chemoattractant protein-1, transforming growth factor-β1, nerve growth factor, resistin and hyaluronic acid as serum markers: comparison between recurrent acute and chronic pancreatitis

Background Diagnostic parameters that can predict the presence of chronic pancreatitis (CP) in patients with recurrent pain due to pancreatitis would help to direct appropriate therapy. This study aimed to compare the serum levels of monocyte chemoattractant protein-1 (MCP-1), transforming growth factor-β1 (TGF-β1), nerve growth factor (NGF), resistin and hyaluronic acid (HA) in patients with recurrent acute pancreatitis (RAP) and CP to assess their ability to differentiate the two conditions. Methods Levels of serum markers assessed by enzyme-linked immunosorbent assay (ELISA) were prospectively compared in consecutive patients with RAP, CP and in controls, and stepwise discriminant analysis was performed to identify the markers differentiating RAP from CP. Results One hundred and thirteen consecutive patients (RAP=32, CP=81) and 78 healthy controls were prospectively enrolled. The mean (SD) age of the patients was 32.0 (14.0) years; 89 (78.8%) were male. All markers were significantly higher in CP patients than in the controls (P Conclusion Serum resistin is a promising marker to differentiate between RAP and CP and needs validation in future studies, especially in those with early CP.

Chronic Myeloid Leukemia with Hyperdiploidy: A Case Report with Review of Literature

Chronic myeloid leukemia (CML) is a common marrow stem cell neoplasm characterized by the presence of the Philadelphia (Ph) chromosome in more than 90% of patients. Studies have shown that CML can be associated with various other cytogenetic abnormalities. 5-10% of these cases can show complex translocations involving other chromosomes in addition to Ph chromosome. Here, we report a Ph-positive CML patient with a hyperdiploid karyotype who presented clinically in chronic phase but progressed to blast crisis in spite of treatment with Imatinib. This case highlights the significance of cytogenetic abnormalities on the prognosis in CML.

Effectiveness of a training program for bleeding disorders among accredited social health activists in Udupi District, Karnataka, India

Hemophilia and other bleeding disorders are underreported and cause significant morbidity and mortality in resource‐constrained countries. Training and the creation of awareness among accredited social heath activists (ASHAs) will aid in the early detection of bleeding disorders at the community level.

Comparing acid steatocrit and faecal elastase estimations for use in M-ANNHEIM staging for pancreatitis

AIM To compare two tests for exocrine pancreatic function (EPF) for use in M-ANNHEIM staging for pancreatitis. METHODS One hundred and ninety four consecutive patients with acute pancreatitis (AP; n = 13), recurrent acute pancreatitis (RAP; n = 65) and chronic pancreatitis (CP; n = 116) were enrolled. EPF was assessed by faecal elastase-1 (FE-1) estimation and stool fat excretion by the acid steatocrit method. Patients were classified as per M-ANNHEIM stages separately based on the results of the two tests for comparison. Independent Student’s t-test, χ2 test, Kruskal-Wallis test, Mann-Whitney U test and McNemar’s test were used as appropriate. RESULTS Sixty-one (52.5%) patients with CP had steatorrhoea when assessed by the acid steatocrit method; 79 (68.1%) with CP had exocrine insufficiency by the FE-1 test (χ2 test, P < 0.001). The results of acid steatocrit and FE-1 showed a significant negative correlation (Spearman’s rho = -0.376, P < 0.001). A statistically significant difference was seen between the M-ANNHEIM stages as classified separately by acid steatocrit and the FE-1. Thirteen (6.7%), 87 (44.8%), 89 (45.8%) and 5 (2.5%) patients were placed in M-ANNHEIM stages 0, I, II, and III respectively, with the use of acid steatocrit as against 13 (6.7%), 85 (43.8%), 75 (38.6%), and 21 (10.8%) respectively by FE-1 in stages 0, I, II, and III thereby altering the stage in 28 (14.4%) patients (P < 0.001, McNemar’s test). CONCLUSION FE-1 estimation performed better than the acid steatocrit test for use in the staging of pancreatitis by the M-ANNHEIM classification since it diagnosed a higher proportion of patients with exocrine insufficiency.

Continuing episodes of pain in recurrent acute pancreatitis: Prospective follow up on a standardised protocol with drugs and pancreatic endotherapy

AIM To assess the outcomes of drug therapy (DT) followed by pancreatic endotherapy for continuing painful episodes in recurrent acute pancreatitis. METHODS DT comprised of pancreatic enzymes and anti-oxidants failing which, endotherapy (ET; pancreatic sphincterotomy and stent placement) was done. The frequency of pain, its visual analogue score (VAS), quality of life (QoL), serum C peptide and faecal elastase were compared between baseline and after 1 year of follow up in all patients and in the two subgroups on DT and ET. Response was defined as at least 50% reduction in the severity of pain to below a score of 5. RESULTS Of the thirty nine patients analysed, 21 (53.9%) responded to DT and 18 (46.1%) underwent ET. The VAS for pain (7.0 ± 2.0 vs 1.3 ± 2.5, P < 0.001) and the number of days with pain per month decreased [1.0 (1.0, 2.0) vs 1.0 (0.0, 1.0), P < 0.001], and the QoL scores [55.0 (44.0, 66.0) vs 38.0 (32.00, 51.00), P < 0.01] improved significantly during follow up. Similar significant improvements were seen in patients in the subgroups of DT and ET except for QoL in ET. The serum C-peptide (P = 0.001) and FE (P < 0.001) levels improved significantly in the entire group and in the two subgroups of patients except for the C peptide levels in patients on DT. CONCLUSION A standardised protocol of DT, followed by ET decreased the intensity and frequency of pain in recurrent acute pancreatitis, enhanced QoL and improved pancreatic function.

Glanzmann's thrombasthenia: The clinicopathological spectrum and its diversities

Aim: To study the clinical spectrum of patients with Glanzmann′s Thrombasthenia (GT) in a tertiary care hospital in South India and to correlate the clinical & laboratory parameters of GT. Materials and Methods: This study includes 15 patients who were diagnosed as GT by characteristic clinical features, basic lab parameters (prolonged bleeding time, abnormal clot retraction and absence of platelet clumps on direct smear) and platelet aggregometry. Results: 66.6% of our patients were below 10 years of age. The common forms of bleeding in our patients was epistaxis,gum bleeding and prolonged bleeding from trauma site which was seen in >50% of our patients.The significant findings on routine laboratory testing was a prolonged bleeding time (93.33%) and an abnormal clot retraction test (66.6%). Platelet aggregation studies showed decreased aggregation with ADP, Collagen and Epinephrine in 100% of our patients whereas 50% also showed decreased aggregation with Ristocetin.Hence most of the patients in this study presented with the classical symptoms and laboratory findings of GT however some had unusual findings. Conclusion: The prevelance of GT is higher in southern India due to increased incidence of consanguineous marriages in this area.Though basic hemostatic screening tests and platelet aggregometry are sufficient to establish the diagnosis in majority of the patients,however some may require more sophisticated tests like flowcytometry for glycoprotein (GP) IIb-IIIa.

Oral hygiene and dentition status in children and adults with hemophilia: A case-control study

BACKGROUND & OBJECTIVES People with hemophilia constitute a significant proportion of the population and an oral health care professional faces a considerable challenge while treating them. This study aimed to assess the oral health and dentition status as well as fear of dental treatment in patients with hemophilia and compare it with age-matched healthy subjects. PATIENTS & METHODS This single-center, case-control cross-sectional study was performed on 100 subjects with hemophilia and 100 age-matched healthy controls. Oral health and dentition status was recorded for all the subjects and scored using the simplified oral hygiene index (OHI-S), plaque index, and the dmft/DMFT index. RESULTS There were a total of 41 children and 59 adults in the hemophilia group and 36 children and 64 adults in the healthy group. When compared to healthy subjects it was observed that individuals with hemophilia had higher debris and calculus scores which was indicative of poor orodental status. There was no significant difference observed in the DMFT scores among the study groups. INTERPRETATION & CONCLUSIONS The oral hygiene of the hemophilics was poorer when compared to the healthy controls. The findings highlight the need for establishing interdisciplinary care for such individuals.

Knowledge among mothers’ of children and youth with hemophilia-A cross sectional survey at a hemophilia center

Introduction: Hemophilia is a genetic disease caused by deficiency of factor VIII or IX. Awareness about the disease condition among parents and children also is as important to manage the disease effectively. Objectives: To assess the knowledge on hemophilia among mothers’ of children with hemophilia and youth with hemophilia. Methodology: A cross sectional survey was conducted at hemophilia center(Manipal, Karnataka). In total, 23 mothers’ of children and 27 youth with moderate and severe hemophilia were included. The participants were selected based on purposive sampling. Approval was taken from the ethical committee. Demographic proforma and knowledge questionnaire was used to collect the data. The data was analyzed using SPSS 16.0 version. Results: The mean age of the participants were 13.68 years. Out of 50 participants 42 had moderate and 8 had severe hemophilia. Moderate knowledge was found among 43.5% of mothers’ and 66.7% of youth with hemophilia. Conclusion: This study shows that mothers’ knowledge on hemophilia is limited and highlights the importance of continual education of parents and youth about their disease.

Effectiveness of a comprehensive educational programme for Accredited Social Health Activists (ASHAs) to identify individuals in the Udupi district with bleeding disorders: A community-based survey

The awareness and knowledge on bleeding disorders is generally poor among the rural population. Accredited Social Health Activists (ASHAs) serve as the facilitators between the rural community and the health care system. Training of ASHAs in screening of rural population for early identification of bleeding disorders can enable prompt referral, timely detection and management of bleeding disorders.