Flow cytometric osmotic fragility test and eosin-5'-maleimide dye-binding tests are better than conventional osmotic fragility tests for the diagnosis of hereditary spherocytosis.

INTRODUCTION: Hereditary spherocytosis (HS) is the most common inherited hemolytic anemia with heterogeneous clinico-laboratory manifestations. We evaluated the flow-cytometric tests: eosin-5'-maleimide (EMA) and flow-cytometric osmotic fragility test (FOFT) and the conventional osmotic fragility tests (OFT) for the diagnosis of hereditary spherocytosis (HS). METHODS: One hundred two suspected HS patients underwent EMA, FOFT, incubated OFT (IOFT), and room temperature OFT (RT-OFT). In addition, 10 cases of immune hemolytic anemia (IHA) were included, and performance of the above 4 tests was evaluated. For EMA and FOFT, 5 normal controls were assessed together with the patients and cutoffs were calculated using receiver-operator-characteristics curve (ROC) analysis. RESULTS: The best cutoff for %EMA decrease was 12.5%, and for FOFT, %residual red cells (%RRC) was 25.6%. The sensitivity and specificity of RT-OFT was 62.06% and 86.3%, respectively, while that of IOFT was 79.31% and 87.67%, respectively. Both flow cytometric tests performed better. Sensitivity and specificity of EMA was 86.2% and 93.9% respectively, and that of FOFT was 96.6% and 98.63%, respectively. The combination of the FOFT with IOFT or EMA dye-binding test yields a sensitivity of 100%, but with EMA, it had a higher specificity. Hb/MCHC was a predictor of the severity of the disease while %EMA decrease and %RRC did not correlate with severity of the disease. CONCLUSION: Flow-cytometric osmotic fragility test is the best possible single test followed by EMA for diagnosis of HS. A combination of FOFT and EMA can correctly diagnose 100% patients. These tests are likely to replace conventional OFTs in future.

A rare case of adult T cell leukemia/lymphoma: clinicopathological correlation with autopsy confirmation.

Adult T cell leukaemia/lymphoma (ATLL) is a rare T lymphoproliferative disorder which is etiologically linked with human T cell lymphotropic virus type-1 (HTLV-1). HTLV-1 is endemic in Japan, Caribbean and Africa. The highest incidence of ATLL is in Japan although sporadic cases have been reported elsewhere in the world. We describe a case of ATLL with an unusual presentation with clinic-pathological correlation and autopsy confirmation. A 56 year old male was referred to Command Hospital (Southern Command) for an incidental finding of lymphocytosis on a routine Hemogram. Clinical examination did not reveal hepatosplenomegaly, lymphadenopathy, jaundice or skin lesions. Laboratory investigations showed lymphocytosis with predominance of atypical lymphomonocytoid cells. Immunophenotyping of the bone marrow mononuclear cells showed positivity for CD45, CD2, CD3, CD4, CD5 and negative for CD7, CD8, CD13, CD33, CD19, which is characteristic of ATLL phenotype. Clonality was confirmed by PCR for TCR gene rearrangement on post mortem tissue. He succumbed to his illness after 40 days of initial presentation and 16 days of being diagnosed as ATLL. Here, we discuss the pathogenesis and characteristics of ATLL with clinico-pathological correlation and autopsy confirmation.

Comparative Study of Blood Cross Matching Using Conventional Tube and Gel Method.

BACKGROUND: Conventionally tube method is used for compatibility and cross matching in transfusion medicine. METHODS: A comparative study was carried out to evaluate the efficacy of conventional tube and gel technique. RESULT: Compatibility testing was performed on 1000 blood samples by conventional test tube method and DiaMed gel method. The results were analysed. CONCLUSION: The gel method was found to be a rapid and reliable procedure without controls. There was no requirement of wash phase in indirect antiglobulin test and sensitivity and specificity was comparable to spin tube method. We conclude that this technique is a better substitute for spin tube method.

Injection practices in a metropolis of North India: perceptions, determinants and issues of safety.

BACKGROUND: At least 50 percent of the injections administered each year are unsafe, more particularly in developing countries, posing serious health risks. An initial assessment to describe injection practices; their determinants and adverse effects can prevent injection-associated transmission of blood borne pathogens by reducing injection frequency and adoption of safe injection practices. AIMS: To assess the injection practices in a large metropolitan city encompassing varied socio-cultural scenarios. STUDY SETTING AND DESIGN: Field based cross sectional survey covering urban non-slum, slum and peri-urban areas of a large metropolitan city. METHODS AND MATERIAL: Injection prescribers, providers and community members selected by random sampling from the study areas. Pre tested questionnaires assessed knowledge and perceptions of study subjects towards injections and their possible complications. Observation of the process of injection and prescription audit also carried out. STATISTICAL ANALYSIS: MS Access for database and SPSS ver 11 for analysis. Point estimates, 95% confidence intervals, Chi Square, t test, one-way ANOVA. RESULTS: The per capita injection rate was 5.1 per year and ratio of therapeutic to immunization injections was 4.4:1. Only 22.5%of injections were administered with a sterile syringe and needle. The level of knowledge about HIV and HBV transmission by unsafe injections was satisfactory amongst prescribers and community, but inadequate amongst providers. HCV was known to a very few in all the groups. The annual incidence of needle stick injuries among providers was quite high. CONCLUSION: A locally relevant safe injection policy based on multi disciplinary approach is required to reduce number of injections, unsafe injections and their attendant complications.

Cryptococcal Meningitis: A Clinicopathological Account of Seven Cases Encountered in a Military Setting.

The clinicopathological profile of 7 cases of Cryptococcal meningitis encountered over one year period in a military hospital has been described. Severe persistent headache was the main form of presentation followed by features of progressive ill-health. Age range of cases was 34-55 years (mean 40.8 years). All were males and 6 were serving soldiers. Duration of symptoms ranged from 10 days to 6 weeks. Six patients were positive for HIV-1 antibodies. This was the initial presentation of AIDS in 6 patients accounting for 10.34% among all cases of HIV infection during that year. Both clinical and laboratory findings were subtle. Nuchal rigidity was rare, cerebro spinal fluid (CSF) cell count and protein level was normal in 3 out of 7 cases. Although culture for Cryptococcus was positive in all, it took 10 days to grow in 2 cases. India-ink stain showed scanty number of organisms in 3 cases. Low cell counts in CSF and presence of associated hyponatremia appear to be bad prognostic indicators in Cryptococcal infections of central nervous system. Latex agglutination test is a worthwhile screening test.

Pathogenetic factors underlying juvenile deep vein thrombosis (DVT) in Indians.

The role of hereditary antithrombotic protein defects in juvenile deep vein thrombosis (DVT) was evaluated. Fifty six young patients (age <45 yr) with doppler-proven DVT were investigated for the presence of resistance to activated protein C (APC-R), lupus anticoagulant (LA), anticardiolipin antibodies and deficiencies of protein C, protein S, ATIII activities. Fifty nine normal healthy individuals served as controls. APC-R was observed to be the commonest defect underlying the Indian DVT as seen in 39.2% of patients followed by elevated ACA (5.3%), PAI (2.8%), presence of LA (2.8%) and reduced ATIII levels (2.8%). None of the subjects had protein C or S deficiency. APC-R was associated with ATIII deficiency in one case, and elevated ACA in two cases. In two subjects, APC-R was associated with elevated PAI levels. Patients with more than one prothrombotic factor had a higher prevalence of pulmonary thromboembolism, suggesting that the thrombogenic potential of APC-R is enhanced by the presence of coexisting hereditary or acquired prothrombotic defect.

Erythrocyte indices for discriminating thalassaemic and non-thalassaemic microcytosis in Indians.

BACKGROUND: Microcytosis is a common red cell change seen in anaemias of varied aetiology. These include iron deficiency, thalassaemia, chronic disease and sideroblastic anaemias. The microcytosis of heterozygous beta-thalassaemia needs to be distinguished from non-thalassaemic microcytosis for its role in thalassaemia control. Red cell indices derived from automated red cell analysers have been used to discriminate between microcytic patients with a high probability of thalassaemia minor from those with a low probability. There is a controversy on the choice of red cell indices to be used and the cut-off values for this distinction, because the prevalence of iron deficiency as a cause of non-thalassaemic microcytosis is variable. Since no Indian study using receiver operator characteristic (ROC) curves was available to determine the above, we conducted this study. METHODS: Red cell indices (mean corpuscular volume, total red blood cell count, red cell distribution width, linear discriminant function), serum iron, total iron binding capacity and haemoglobin A2 were estimated in 640 adults with microcytosis (mean corpuscular volume 80 fl). The ROC curves were plotted in all. RESULTS: Total red blood cell count was observed to be the most efficient single test followed by linear discriminant function and Bessman index. Mean corpuscular volume had the least efficacy. The cut-off values obtained for the Indian population were mean corpuscular volume < or = 76 fl, total red blood cell count > or = 4.9 x 10(12)/L and red cell distribution width > or = 18% and a positive linear discriminant function. These were different from those observed in the West, possibly because of the high prevalence of iron deficiency in India. CONCLUSION: In countries with a high prevalence of iron deficiency, cut-off values for red cell indices should be recalculated using ROC curves.

A community mental health group designed by clients.

"Support," a group learning project, was conceived and designed by patients who completed a twenty-four hour occupational therapy stress management program offered by an occupational therapy assistant at a community mental health day treatment center in Upstate New York. The patients had become a very "close knit" group because of the nature and intensity of the therapeutic activities. As they moved to ex-patient status and to a self-directed group they identified familial and community stressors they expected would arise and wanted to strengthen their network. They sought resources to provide knowledge and practice for themselves and their families in the management of those stressors. This paper describes the rationale and the process of the "Support" group and the ways in which patients contributed to its formation, ongoing activities and closure.