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Table of Contents
July-September 2015
Volume 6 | Issue 3
Page Nos. 93-139
Online since Friday, September 18, 2015
Accessed 60,747 times.
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REVIEW ARTICLE
Neural cell adhesion molecule (cluster of differentiation 56) in health and disease
p. 93
Syed Z. A Zaidi, Ibraheem H Motabi, Ali Al-Shanqeeti
DOI
:10.4103/1658-5127.165655
Cluster of differentiation (CD) 56, a member of the immunoglobulin superfamily, and an isoform of neural cell adhesion molecule (NCAM), was the first cell adhesion molecule to be identified. NCAM (CD56) plays an important role both in human health and in disease. Human NCAM gene is located on chromosome 11q23. CD56 antigen is a 175–185-kD cell surface glycoprotein expressed on all subsets of human natural killer (NK) cells except a small minority of CD56
−
NK-cell, on subsets of CD4
+
/CD8
+
T-cells, interleukin-2-activated thymocytes, bone marrow macrophages, osteoclasts, and on adrenal gland and neural tissues. NCAM is important in calcium independent cell-cell interactions that mediate homotypic and heterotypic cell-cell and cell-matrix adhesions. At least 27 alternatively spliced NCAM mRNAs are produced giving a wide diversity to NCAM isoforms sharing a similar structural organization. NCAM in the cerebellum and cerebral cortex mediates homophilic adhesion of neural cells, and plays an important role in brain development, emotions, and memory functions. While CD56
+
NK-cells play an important role in defense against infections, tumor remission, normal pregnancy and graft rejection. Malignancies expressing CD56 are usually aggressive, with more potential for metastasis and extramedullary/central nervous system involvement, and may respond to new CD56-linked targeted therapies.
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ORIGINAL ARTICLES
Sensitive allele specific oligonucleotide-polymerase chain reaction in detection of preexisting mutations in imatinib-resistant chronic myelogenous leukemia patients: A retrospective analysis
p. 106
Mukul Arvind Gharote, Harsha Panchal, Apurva Patel, Sonia Parikh, Rakesh Rawal, Krupa Shah, Asha S Anand
DOI
:10.4103/1658-5127.165648
Introduction:
Pre-existing BCR-ABL kinase domain mutation leads to Imatinib resistance.
Methods:
Retrospective analysis of 50 patients of Imatinib resistance was done in GCRI, from January 2014 till May 2014. Allelle Specific Oligonucleotide–Polymerase Chain Reaction (ASO-PCR) was performed on Genomic DNA, of peripheral blood mononuclear cells (PBMCs).
Results:
47 (94%) were in Chronic phase, 2 (4%) in accelerated phase, 1 (2%) in blastic crisis. Median duration of Imatinib was 48 months. 43/50 had one or more than 1 mutation, T315I mutation in 5 (10%) patients, M351T in 32% (16/50) and F311L in 8.
Conclusion:
We report low cytogenetic response (25%) and durability of response to 600 mg of Imatinib, even in M351T mutation.
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Implication of deferral pattern on the donor pool: Study at a Tertiary Care Hospital
p. 111
Deepika Chenna, Shamee Shastry, Mohandoss Murugesan, Poornima B Baliga
DOI
:10.4103/1658-5127.165649
Background and Objectives:
Donor screening process is one of the most important steps in protecting the safety of blood supply. Donors who do not meet specified criteria are deferred either temporarily or permanently. These criteria are designed to protect both donors and patient safety. Due to the varied rates and reasons for deferrals in the existing literature, we aimed to evaluate the patterns and prevalence of deferrals in our institution.
Materials and Methods:
This retrospective study was conducted at a Tertiary Care Hospital, Karnataka, Southern India, to evaluate the various reasons for blood donor deferral from January 2011 to January 2014. Demographic data of blood donors was obtained through the blood bank database and secondary measures such as the type of deferral (permanent/temporary, pathogenic/nonpathogenic, and harmful to donor/recipient) were assessed.
Results:
A total of 54,653 subjects presented to our blood bank during this period out of which 2935 (5.6%) were deferred. The deferral to donor percentage was higher in females (36.54%) than males (3.64%). Low hemoglobin was the major deferral criterion seen in our participants (48.1%) followed by hypertension (16.4%), underweight (8.9%). Low pulse rate and fasting donor were the least prevalent reasons. A total of 36.8% of reasons for deferral were harmful to donors, 88.2% were nonpathogenic, and 98.1% were temporary causes.
Conclusion:
Variations in donor deferral may be attributed to different donor selection criteria in different regions and centers. Hence, it is important to know the common causes of donor deferral in a region so that measures may be taken to improve the donor pool.
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Elevated levels of pro-coagulant microvesicles in children in-steady state sickle cell disease
p. 115
Hassan A Hamali, Orwa G Elhussein, Abdulmoneim Jamil, Sadaqat Hussain, Mubarak Alshraim, Abdulrahman Alshehri
DOI
:10.4103/1658-5127.165650
Introduction:
Sickle cell disease (SCD) is an inherited genetic disorder characterized by various complications, including thrombosis. Increased levels of circulating microvesicles (MVs) and tissue factor (TF)- bearing MVs have been reported in SCD.
Objectives:
The present study compares the levels of circulating MVs and TF- bearing MVs in steady state SCD children with age- and gender- matched healthy controls using an indirect ELISA. Citrated whole blood was collected from 54 SCD patients homozygous for sickle haemoglobin (HbSS) (aged from 2 to 12 years-old) and 34 healthy controls.
Results:
SCD patients showed significantly higher levels of MVs in their plasma as compared to the controls (
P
= 0.0095). Although the TF activity on MVs was low in both groups, there was a significant difference between them
P
<0.05). A strong correlation between the level of MVs and TF-MVs in the patient group was also noted.
Conclusion:
This suggests their involvement in the hypercoagulable state in the study group of patients. Further studies are recommended to elucidate the functional activity of MVs and TF-MVs, as well as the size and origin of MVs in the plasma.
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Comparative analysis of four methods for enumeration of platelet counts in thrombocytopenic patients
p. 119
Boulassel Mohamed-Rachid, AL-Farsi Raya, Al-Hashmi Sulaiman, Al-Kindi Salam
DOI
:10.4103/1658-5127.165654
Background:
Even with the most reliable automated blood cell analyzers, obtaining an accurate platelet count in patients with thrombocytopenia is still challenging especially when making decisions for platelet transfusions. The Cell-Dyn Sapphire offers three methods for platelet counting including the optical, impedance, and immunological techniques.
Aim:
A comparative analysis evaluating the performance of these three methods, along with the manual technique, was performed in thrombocytopenic patients.
Materials and Methods:
A total of 32 blood samples from patients without a history of chemotherapy and a platelet count <50 ×- 10
9
/l were tested in parallel by four different methods.
Results:
Compared to other techniques, impedance method provided an overestimation of platelet count (
P
= 0.0008) and failed to show a result in 15% of cases with low platelet counts (<15 ×- 10
9
/l). Good to excellent correlations and reliability values were evidenced among study methods, but a poor reliability was noticed between the impedance and immunological methods with an intraclass correlation coefficient of 0.49 (confidence interval: 0.15–0.73;
P
= 0.003). In the bias analysis, the impedance method showed the highest levels of disagreement with other techniques.
Summary/Conclusion:
Collectively, these results provide evidence that the optical or immunological technique appear to be superior to the impedance method in estimating low platelet counts with the automated analyzer Cell-Dyn Sapphire. As such, the healthcare staff and the physicians must be aware of this limitation, especially in the presence of severe thrombocytopenia, when a decision of platelet transfusion has to be made.
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CASE REPORTS
Chronic eosinophilic leukemia-not otherwise specified transformation into acute myeloid leukemia
p. 125
Kshitija Kasturi, Sana Vanajakshi
DOI
:10.4103/1658-5127.162580
Chronic eosinophilic leukemia-not otherwise specified (CEL-NOS) is a rare disorder with hypereosinophilia and an increase number of blood or marrow blasts (<20%) or an evidence of eosinophilic clonality. It is an aggressive disorder with a high rate of acute transformation and resistant to conventional treatment. Its clinical and pathological picture vaguely overlap and adds to diagnostic confusion with idiopathic hypereosinophilic syndrome (IHES) which is characterized by sustained overproduction of eosinophils over a period of 6 months with multiple organ damage. Due to the difficulty in distinguishing CEL from IHES, the true incidence of this disease is rare. We report a case of CEL-NOS, which was preceded by IHES and terminated in acute myeloid leukemia with maturation (M2) in a 38-year-old male.
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Bilateral sixth cranial nerve palsy as a presenting feature of multiple myeloma: A rare presentation
p. 130
Lulup Kumar Sahoo, Nihar Ranjan Biswal, Ashok Kumar Mallik, Geeta Mohanty, Kali Prasanna Swain
DOI
:10.4103/1658-5127.165652
Sixth cranial nerve palsy is the most common cause of acquired extraocular muscle paralysis. Neoplasm, trauma, raised intracranial pressure, and vascular disease are the most common etiologies of sixth nerve dysfunction. Multiple myeloma is a chronic, progressive, and fatal neoplastic proliferation of plasma cells and can produce tumors or plasmacytomas, and bone marrow lesions. We here present a case of a 53-year-old man who was presented with a holocranial headache and double vision. On examination, patient was found to have bilateral lateral rectus palsy. The patient was diagnosed as having IgA kappa type multiple myeloma based on immunological and pathological investigation. Contrast-enhanced computed tomography brain showed an enhancing lesion in clivus and sphenoid bone. Under chemotherapy, sixth cranial nerve palsy was resolved completely. We speculated that sixth nerve palsy might be caused by compression and stretching along the path of the sixth nerve at clivus.
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Primary Burkitt lymphoma of kidney: A rare presentation in a child
p. 133
Shilpi Agarwal, Gunjan Mahajan, Pallavi Prakhar Rana, Riti Yadav
DOI
:10.4103/1658-5127.165651
Primary renal Burkitt lymphoma (BL) is an unusual occurrence in children. Although the prognosis of this tumor is poor, early diagnosis on biopsy confirmed by immunohistochemistry (IHC) and followed by chemotherapy improves survival of such patients. We present a case of 4-year-old male child with bilateral renal lump suggestive of lymphoma on computerized tomography. Renal biopsy and subsequent IHC was suggestive of bilateral BL in the kidney; following which the child was started on FAB LMB 96 based treatment protocol.
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BRIEF REPORT
Distribution of ABO and Rhesus blood group systems among blood donors in Sokoto North-western Nigeria
p. 136
Abubakar Umar Musa, Muhammad Alhaji Ndakotsu, Hassan Abdul-Aziz, Ayatollah Kilishi, Ibrahim Aliyu
DOI
:10.4103/1658-5127.165653
The ABO and Rhesus (Rh) are considered the most vital blood group systems in transfusion practice and their distribution is determined by genetics and environmental factors. We determined the distribution of ABO and Rh blood group systems among blood donors in Sokoto, North-Western Nigeria by conducting a retrospective study over a two-year period (1
st
Jan 2012 to 31
st
Dec 2013) at Usmanu Danfodiyo University Teaching Hospital (UDUTH) Sokoto, North-Western Nigeria. Blood donor records were studied for the distribution of ABO and Rh blood group systems while the Ag-Ab agglutination method using the tile technique was employed for blood group determination. Microsoft Excel 2010 was used to analyze the generated data into simple proportions and percentages. A total of 8,975 blood donors were attended to and the ABO group distribution was; O 4,659 (51.91%), B 2,109 (23.50%), A 1,865 (20.78%) and AB 342 (3.18%). While the Rh group distribution was 8,657 (96.46%) and 318 (3.54%) for Rh D positive and Rh D negative respectively. The blood group O was the commonest among the ABO group while majority of the donors were Rh positive. Our data can serve as a guide in formulating blood banking and transfusion services for hospitals in Sokoto, North-western Nigeria.
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IMAGE IN HEMATOLOGY
Systemic mastocytosis with associated clonal hematological nonmast cell lineage disease
p. 139
M Indira, KK Prabhalakshmi, Cyril J Kurian
DOI
:10.4103/1658-5127.165647
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