Does Profile of Hemoglobin Eβ-thalassemia Patients Change After Splenectomy? Experience of a Tertiary Thalassemia Care Centre in Eastern India

Hemoglobin Eβ-thalassemia is by far the commonest form of thalassemia intermedia. Its phenotype ranges from mild anemia to severe transfusion-dependency necessitating splenectomy in many patients. The present study aimed to systematically analyze both clinical as well as laboratory parameters in profile of Eβ-thalassemia patients after splenectomy in terms of transfusion requirement, infections and other complications. Retrospective study conducted over a period of 3 years included 72 cases of splenectomized Eβ-thalassaemia patients, considering decrease in transfusion requirements, new complications, antibiotic, anti-malarial prophylaxis and iron chelation therapy. Out of 1380 registered Eβ-thalassemia patients, 618 (44.78 %) were regularly transfused and 72(5.22 %) underwent splenectomy. Mean age of diagnosis was 10.3 years. Nineteen patients (26.4 %) underwent splenectomy between 5 and 10 years, 38 cases (52.7 %) between 10 and 20 years. The leading cause (51.39 %) for splenectomy was mechanical discomfort. Mean steady state hemoglobin raised from pre-splenectomy level of 5.43–6.8 gm/dl after splenectomy. Mean transfusion requirement reduced from 18.1 to 7.8 units/year. Mean serum ferritin level increased from 907.58 to 1,091.6 ng/ml. Post-splenectomy; 21 (29.17 %) patients developed facial deformities, 17 (23.6 %) delayed pubertal growth, 11 (15.28 %) venous thromboembolism, five (6.94 %) pulmonary hypertension and four (5.5 %) had extramedullary hematopoiesis. Five (6.96 %) patients had documented bacterial infections and two (2.78 %) suffered from malaria. Forty eight patients (66.67 %) started with iron chelation therapy; but majority (52.7 %) stopped. Major advantage of splenectomy is reduced transfusion requirement, though it cannot prevent skeletal abnormalities and delayed pubertal growth. In resource constraint countries like India, routine anti-malarial and antibacterial prophylaxis is not desirable; iron chelation therapy should be encouraged and ensured.     

The Expression Pattern of CD33 Antigen Can Differentiate Leukemic from Normal Progenitor Cells in Acute Myeloid Leukemia

Leukemic stem cells (LSC) in acute myeloid leukemia (AML), defined by CD34 and CD38 antigens also express CD33 similar to normal hematopoietic stem cells. Residual LSC are believed to be responsible for relapse in AML after chemotherapy. Leukemic progenitor cell compartments were defined by CD34 and CD38 expression by flow cytometry in 61 new cases of AML. In each of four compartments thus defined, CD34+CD38−, CD34+CD38+, CD34−CD38− and CD34−CD38+, the pattern and intensity of expression of CD33 were studied in comparison to similar progenitor cell compartments in normal bone marrow and peripheral blood stem cell harvests. Post induction bone marrow samples from 10/61 cases were studied for aberrant CD33 expression. The intensity and pattern of expression of CD33 in AML progenitor cells were significantly different compared to normal progenitor cells. In two cases who were in morphological remission post induction, aberrant CD33 expressing progenitor cells were detectable at a frequency of 1.6 and 0.5 % respectively in the bone marrow. Aberrant CD33 expression in bone marrow LSC identified as CD34+CD38− cells in the CD45 dim/low side scatter region on flow cytometry may be useful as minimal residual disease marker after AML therapy. The method involves the use of a limited number of reagents and can be applied to all cases of AML.     

Apolipoproteins AI/B/E gene polymorphism and their plasma levels in patients with coronary artery disease in a tertiary care-center of Eastern India

AimThe present study was designed to investigate whether the three-apolipoprotein (AI, B, E) gene polymorphisms were related to alter their plasma protein levels and hence associated to coronary artery disease (CAD).MethodsWe determined distribution of MspI apo AI, EcoRI apo B, HhaI apo E gene polymorphisms, plasma apolipoproteins and lipids levels among 150 patients having CAD admitted to the Department of Cardiology, N.R.S. Medical College & Hospital, Kolkata, India during June 2010–June 2012 and 150 age sex matched healthy controls.ResultsWe found that ApoAI concentration of studied population was significantly different in each genotypes of ?75 G/A apo AI (p < 0.0001) gene polymorphism. A significant association was found in multivariate analysis for the genotypes with apo E4 allele [odds ratio (OR): 3.639; 95% confidence interval (CI): 1.019–12.995, p = 0.040] with four conventional risk factors (i.e. smoking, low-density lipoprotein, ApoAI and ApoB) with CAD. In contrast E2 allele has reverse effect, but the genotypes with apo E2 allele was no longer significant in the multivariate model (OR: 1.788; 95% CI: 0.400–8.001, p = 0.447) where as being significant in univariate analysis (OR: 0.219; 95% CI: 0.087–0.552, p = 0.001).ConclusionsOur findings suggest that the polymorphisms apo AI MspI and apo B EcoRI do not seem to affect CAD. But the genotype with E4 allele of apo E gene independent of other risk factors is associated with this disease.     

Mitochondrial 16S ribosomal RNA gene for forensic identification of crocodile species

All crocodilians are under various threats due to over exploitation and these species have been listed in Appendix I or II of CITES. Lack of molecular techniques for the forensic identification of confiscated samples makes it difficult to enforce the law. Therefore, we herein present a molecular method developed on the basis on 16S rRNA gene of mitochondrial DNA for identification of crocodile species. We have developed a set of 16S rRNA primers for PCR based identification of crocodilian species. These novel primers amplify partial 16S rRNA sequences of six crocodile species which can be later combined to obtain a larger region (1290 bp) of 16S rRNA gene. This 16S rRNA gene could be used as an effective tool for forensic authentication of crocodiles. The described primers hold great promise in forensic identification of crocodile species, which can aid in the effective enforcement of law and conservation of these species.     

Diabetic microvasculopathy: the renal-retinal link

Manifestations of diabetic microvasculopathy are protean. Graded increases in the severity are recognised in both nephropathy and retinopathy. This study was undertaken with 100 patients of type 2 diabetes mellitus to evaluate how far these graded increments could be linked at each stage and in each patient. The renal parameters studied were the various accepted levels of albuminuria; the retinopathy parameters ranged from normal retina to severe proliferative stages. Corresponding grades were proposed and altered, if required, to reach the best possible correlation. The correlation was attempted though the common link of mean glomerular filtation rates at each level and the concordance of either parameter grade in an individual patient. The correlations of the mean glomerular filtration rate for all propositions of severity were significant. However, there was significant variability of the parameters in an individual patient. This was more with less severe grades but diminished with increased duration. The predictive value of one lesion for the other was low in cases with shorter duration and less severe grades. Probably, shorter duration patients have an interplay of both genetic factors and the assault of the risk factors while in longer duration patients the cumulative risk exposure play the dominant role.     

Reconstruction of facial deformities in leprosy patients

Almost all the parts of the face may be involved in deformities caused by leprosy. Reconstructive surgery can be undertaken after the disease is arrested by medical treatment. Reconstruction of nose can be done by augmentation with autogenous bone graft, retronasal inlay graft, etc. Loss of eyebrows can be reconstructed with hair bearing skin with hair follicles. Sagging face can be corrected by nasolabial face-lift with correction of glabellar folds. Blepharoplasty is done for correction of some conditions. The temporal muscle sling is a dynamic procedure to reconstruct facial nerve palsy.     

Parasitosis: a study among Nepali children in the district of Darjeeling

A cross-sectional study carried out among 370 school-going children of the Nepali community, aged 5-10 years, in the Siliguri subdivision of Darjeeling district revealed the prevalence of parasitic infestation to be 51.4 per cent of which 28.2% and 23.2% had single and multiple parasitism respectively. In the group of single parasitism, A. lumbricoides was the commonest infestation (31.73%) and in multiple parasitisms, A. lumbricoides and T. Trichiura combination was found to be the most prevalent (36.05%). A significant statistical association was observed in the decrease of worm infestation rate among children with the increasing educational status of their mothers. The study also revealed that 19.73% and 18.91% of the mothers had no knowledge about mode of transmission and prevention of worm infestation respectively.     

Testicular tuberculosis mimicking testicular malignancy

A 22 years old male presented with right sided scrotal swelling of 5 months duration. On examination it was found to be of orange-sized, firm to hard in consistency with mild tenderness. Ultrasonography of testes failed to arrive at a diagnosis. However, fine needle aspiration cytology of the testicular swelling confirmed the diagnosis of testicular tuberculosis. Even conservative approach in the management gave satisfactory result.