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  Citation statistics : Table of Contents
   2015| January-March  | Volume 40 | Issue 1  
    Online since April 24, 2015

 
 
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CASE REPORT
Nasal extranodal T/NK-cell lymphoma presenting as a palatal lesion: an uncommon clinical presentation of a rare disease
M.P.F. Amarante, FD Rocha Filho, A.J.M. Castro, GB Silva Junior, F da Silva Herivaldo
January-March 2015, 40(1):44-48
DOI:10.4103/1110-1067.155798  
A 27-year-old woman presented with a 6-month history of a painful and erosive lesion in the hard palate with a progressive increase in size. Examination indicated two ulcerated lesions in the hard palate. Histopathology showed dense exudation of atypical mononuclear leukocytes composed of small and medium lymphoid cells with round or oval nuclei. Immunohistochemistry showed abnormal T-cell population with strong expression for CD45RO and CD7 with negativity for CD20 and certain T antigenic loss, suggesting T-cell lymphoma. Serology for the Epstein-Barr virus was positive. This is the second case of nasal extranodal T/NK-cell lymphoma presenting as a palatal ulcer reported to date.
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ORIGINAL ARTICLES
Acute retrobulbar haemorrhage in haemophilia B
Jothi Saravanan, Alangudi Achari Rajendraprasad, Begum Zaibunissa, Sundaram Priyadharshni
January-March 2015, 40(1):1-2
DOI:10.4103/1110-1067.155771  
Acute retrobulbar haemorrhage is a very rare complication of haemophilia. We present a 16-year-old boy with haemophilia B with spontaneous retrobulbar haemorrhage of the left orbit. He showed a favourable response to treatment with factor IX replacement with no recurrence until 8 months.
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Platelet aggregation study: valuable aid to diagnose dysfibrinogenemia
Abhishek H. L. Purohit, Mukul Aggrawal, Mrinalini Kotru, Hara P Pati
January-March 2015, 40(1):11-12
DOI:10.4103/1110-1067.155783  
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Analysis of 16 teenage patients with sickle cell anemia and musculoskeletal complications
A Hosseinian Amiri, R Shekarriz, M Yazdanian
January-March 2015, 40(1):13-16
DOI:10.4103/1110-1067.155785  
Background Sickle cell anemia is a genetic hematological disorder characterized by red blood cell abnormality including rigidity and sickling. Once a sufficient number of rigid sickle cells have formed, microvascular occlusion will result, which leads to tissue ischemia and infarction and progressive end-organ damage. Aim The aim of this study was to determine the pattern of musculoskeletal complications in 16 teenage patients with sickle cell disease over a period of 2 years. Patients and methods This is a retrospective study of cases of musculoskeletal complications among sickle cell anemic patients over a period of 2 years, referred to the Rheumatologic Clinic of Mazandaran University of Medical Sciences from January 2010 to December 2012. Data were analyzed using SPSS, version 20. Variables analyzed included age, sex, and type of musculoskeletal complication. Results There were 16 sickle cell patients referred to our Rheumatologic Clinic for musculoskeletal complications. All patients were teenagers aged between 12 and 17 years, with a median age of 14.6 years. There were 12 male and four female patients. The most common musculoskeletal complication was acute painful crisis in 10 (62.5%) patients, osteomyelitis in three (18.5%), dactylitis in four (25%), avascular necrosis of bones in six (37.5%), stress fractures and vertebral collapse in two (12.5%), septic arthritis in one (0.6%), and hyperuricemia/gout arthritis in three (18.5%) patients. Conclusion Serious musculoskeletal complications of sickle cell anemia are very important and must be considered in these patients. Painful crisis and avascular necrosis of bones are common rheumatologic complications of this disorder.
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Estimation of plasma concentrations of hepatocyte growth factor in acute leukemia in Upper Egypt
Ahmed E Ahmed, Sameh S Zytoun, Mohammed A Alsenbesy, Abd-Elrahman A Elsaid
January-March 2015, 40(1):17-23
DOI:10.4103/1110-1067.155789  
Background Angiogenesis is a fundamental element during malignant transformation. The induction of angiogenesis has been proposed to be through angiogenic factors such as hepatocyte growth factor (HGF). Objective The aim of the study was to assess plasma concentrations of HGF in acute leukemia in Upper Egypt. Patients and Methods We performed a cross-sectional study of 90 participants and divided them into three groups: group I included 30 newly diagnosed acute lymphoblastic leukemia patients; group II included 30 patients with newly diagnosed acute myeloid leukemia; and group III included 30 apparently healthy individuals who served as controls. Plasma HGF concentration was measured using the ELISA technique. Results Statistical comparison between the mean values of plasma HGF in the three studied groups on using the F test, followed by the least significant difference, showed a significant difference (F = 77, P = 0.001) between acute lymphoblastic leukemia patients and controls and between acute myeloid leukemia patients and controls. Conclusion The results of the present study suggest that high plasma HGF may play a significant role in leukemia process and contribute to the leukemic cell dissemination. The clinical significance of the increased level of HGF in acute leukemia needs further investigation and may suggest a novel therapeutic approach in this disease.
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Value of thrombopoietin level and platelet size in patients with ischemic stroke
Heba Allah A Sedky, Yasmin N El-Sakhawy, Hany A Hussein, Mohamad A Tork
January-March 2015, 40(1):24-29
DOI:10.4103/1110-1067.155790  
Background Stroke is a disease that affects the blood vessels supplying the brain with interruption of blood flow and thus oxygen delivery to a certain area of the brain, with rapid loss of brain functions. It is the third most common cause of death worldwide, ranking behind heart diseases and all forms of cancers. Aim The aim of this study was to evaluate the mean platelet volume (MPV) and thrombopoietin (TPO) hormone concentration in patients with acute ischemic stroke and compare this with healthy controls. Patients and methods This study was carried out prospectively on 100 patients with acute ischemic stroke who fulfilled the inclusion criteria and compared with 50 control participants. All patients were recruited to the study within the first 24 h after stroke. MPV and TPO concentrations were measured on admission. Results A high TPO concentration was found in two-third of the patients, whereas no participant in the control group had a high TPO concentration. In addition, high MPV was found in about half of the patients. There was a statistically significant difference in the levels of both MPV and TPO between the patient and the control group, which implies increased megakaryopoiesis and increased platelet activity in acute ischemic stroke.
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Clinical, hematological, and cytogenetic profile of aplastic anemia
Subhajit Das, Vijai Tilak, Vineeta Gupta, Anup Singh, Mohan Kumar, Amit Rai
January-March 2015, 40(1):3-10
DOI:10.4103/1110-1067.155779  
Background Aplastic anemia manifests as peripheral blood cytopenias due to inability of the hematopoietic bone marrow to produce blood cells. Aims and objective The aim of this study was to investigate the differences in the clinicohematological profile of pediatric and adult aplastic anemia patients, if any, to establish the necessity of bone marrow biopsy for its diagnosis and to detect any chromosomal abnormality in the patients. Setting and design This study was carried out in the Departments of Pathology, Paediatrics, and General Medicine, and Centre for Genetic Disorders, in a university teaching hospital over 24 months. Materials and methods A total of 42 patients were diagnosed combining the clinical features, peripheral blood counts, and bone marrow examination. Of them, 24 patients were evaluated for cytogenetic abnormality. Statistical analysis All the observations were evaluated using simple and basic statistical tools - for example, range, mean, and median. Results Occurrence of severe anemia and frequency of leukopenia and absolute neutropenia were more common in children. The degree of leukopenia and absolute neutropenia was more severe in children. Because of frequent occurrence of blood tap and dry tap, diagnosis in many cases depended solely on bone marrow biopsy findings. All the core biopsy specimens floated after proper decalcification. Lymphocyte culture failed in nine cases, and the remaining 15 cases showed no cytogenetic abnormality. Conclusion Bone marrow biopsy is mandatory for diagnosing aplastic anemia. The findings of core biopsy floatation and its correlation with marrow cellularity and absence of chromosomal abnormality in aplastic anemia need larger studies to give any statistically significant opinion.
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Basophil progenitor marker histamine and its relation to the treatment response in Egyptian chronic myeloid leukemia patients
Amro M El-Ghammaz, Gehan M Hamed, Mona F Abdel Fattah, Mohamed H Attia
January-March 2015, 40(1):30-36
DOI:10.4103/1110-1067.155793  
Introduction Chronic myeloid leukemia (CML) is characterized by the presence of the BCR-ABL fusion gene with constitutive tyrosine kinase activity that activates many signaling pathways contributing to the abnormal growth and survival of leukemic cells. Basophilia is a strong independent prognostic variable that correlates with the phase of CML. In CML, basophils are difficult to be identified by their morphology due to their immaturity or low frequency by tyrosine kinase inhibitors (TKIs) therapy. It is important to predict responses to therapy and to monitor levels of minimal residual disease in CML patients by cytogenetics and PCR. However, these parameters are expensive and require special technologies. Thus, the aim of the current study was to clarify whether the serum level of the basophil-marker histamine correlates with hematologic, cytogenetic, and molecular responses in CML patients and with the achievement of the optimal response to TKI therapy. Patients and methods The histamine level was measured by enzyme-linked immunosorbent assay in 48 CML patients, together with cytogenetic analysis at diagnosis and after 6 months from the start of imatinib treatment. Molecular study of BCR-ABL was also performed at 6 months. Results Histamine levels were upregulated in CML patients at diagnosis compared with the healthy control group and correlated with the total leukocytic count, the absolute peripheral blood basophil count, and the percent of bone marrow basophils. After 6 months of imatinib treatment, histamine levels were significantly reduced in CML patients compared with the levels at diagnosis and were still significantly higher than in controls. Histamine levels at 6 months correlated significantly with PCR measurements for BCR-ABL. A significant difference in histamine levels was found between patients according to their hematological, cytogenetic, and molecular responses. Also, it differed significantly among TKI response groups (optimal, warning, failure). Conclusion We found that the serum histamine level may serve as a cheap early predictor of cytogenetic and molecular remission as well as the response to imatinib. However, we could not encourage measuring serum histamine as an alternative to PCR for BCR-ABL for the monitoring of minimal residual disease.
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Cryopreservative against noncryopreservative therapy in autologous hematopoietic stem cell transplantation (the Egyptian experience)
Maha T Elzemity, Essam A Elwahed, Amal M Elafifi, Mohamed M Moussa, Mohamed H Attia, Hany M Hegab, Haydi M Elsaid
January-March 2015, 40(1):37-43
DOI:10.4103/1110-1067.155796  
Introduction Autologous peripheral blood stem cell transplantation is a 'rescue' of patients' self hematopoietic stem cells from the myeloablative effects of chemotherapy or irradiation. Cryopreservation of hematopoietic stem cells using 10% dimethyl sulfoxide as a cryoprotectant under liquid nitrogen storage conditions (−196°C) for long-term usage is a well-established procedure, whereas liquid preservation of stem cells is usually performed for storage at 4°C for 36-96 h. The aim of the study was to compare the outcome of autologous stem cell transplantation using cryopreserved or noncryopreserved stem cells. Patients and methods Twenty adult patients younger than 60 years of age were enrolled in this study. They had undergone autologous peripheral stem cell transplantation. They had been stratified into two groups: 10 patients received cryopreserved autologous peripheral stem cell transplantation (PBSCT) and the remaining 10 patients received noncryopreserved autologous PBSCT. Results When we compared the cryopreserved and the noncryopreserved groups for the clinical outcome, there had been a longer overall survival and disease-free survival in favor of the cryopreserved group, but this had not been translated into statistically significant values. In addition, there was no significant difference with regard to the recurrence of disease. However, the noncryopreserved group had a shorter hospital stay and entailed less cost than the cryopreserved group (8149$ against 8900$, respectively). This could be attributed to the fact that liquid nitrogen was not used, and the shorter hospital stay (P = 0.000) lowered the cost for medications, laboratory tests, and procedures. Cryopreserved therapy was found to be cost-effective with regard to keeping the patient alive in those who cannot tolerate proceeding to autologous stem cell transplant immediately after mobilization. Conclusion Noncryopreserved therapy required a lesser number of aphaeresis sessions, was associated with a shorter hospital stay, was less costly than cryopreserved therapy, and it yielded the possibility of transplantation in patients with hepatitis without the need for buying a special liquid-nitrogen tank.
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