Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in peripheral blood and bone marrow. In 95% of cases, it is always due to the presence of Philadelphia chromosome characterized by the presence of reciprocal translocation between chromosome 9 and 22. However, in 7%-17% of individuals, extramedullary proliferation also occurs, either in skin, lymph nodes, bone or central nervous system (CNS), which could be either myeloid, lymphoid or mixed progenitor in origin. The present case is of a 23-year-old male who presented with lower limb weakness, bowel and urinary incontinence. His complete blood count (CBC) findings showed a raised white blood count (WBC) of 408 X 10E9/L. Peripheral film, bone marrow biopsy and immunohistochemistry showed findings consistent with CML in chronic phase. Bone marrow cytogenetic revealed the presence of Philadelphia chromosome. Simultaneously, magnetic resonance imaging (MRI) was done which revealed extradural mass at L1-L3 level; histopathological and immunohistochemistry findings showed features compatible with precursor B cell lymphoblastic lymphoma. His cerebrospinal fluid (CSF) cytology revealed similar blast cells. This extramedullary presence of lymphoid blast cells in the CNS put the patient in the rare entity of CML in blast crisis. He was started on tablet nilotinib and also received multiple cycles of intrathecal chemotherapy with cytosar, methotrexate and hydrocortisone. He also underwent radiotherapy of extradural mass. His lower limb weakness improved dramatically. However, after receiving the fourth cycle of intrathecal therapy, the patient died consequent to neutropenic sepsis. Extramedullary blast crisis in CML has a poor prognosis. Any patient with CML, presenting with CNS symptoms or lymph node enlargement should be thoroughly investigated for extramedullary blast crisis, as there is a considerable change in management and prognosis from the prototype CML in chronic phase.
Abstract Objective:To ascertain the frequency of markers of transfusion-transmitted infections. among blood donors in a blood bank at a tertiary care hospital Material and Methods:The study was a retrospective cross-sectional descriptive study, covering from 1stJanuary 2013- October 2018 and was conducted in the blood bank section, in the Department of Pathology at Dow University of HealthSciences, Hospital. All blood donors were screened for hepatitis B, hepatitis C, HIV (I & II), syphilis through electrochemiluminescence and malaria (immunochromatography).Data was entered and subsequently analyzed by statistical package for social sciences (SPSS) version 21. The frequency of infectious disease markers (HbsAg, Anti HCV, HIV, syphilis, and malaria) was calculated among blood donors. Results:The total number of donors in our study was 29732, out of which 2587 donors were positive for an infectious disease.Out of the total donors, 29712 were male and 20 were female. There were 12 volunteer donors and 29720 exchange donors. The mean prevalence of donors with positive infectious markers was as follows; Anti HCV was 3 %, HbsAg was 2.9%, Syphilis was 2.0%, HIV was 0.5% and Malaria was 0.02 %. Conclusion:HbsAg and Anti HCV were the most frequent infections (3%) found in our blood donors, followed by syphilis with a frequency of 2%. Keywords: Blood transfusion, transfusion-transmitted infections, blood donors.
BACKGROUND: Ideal blood inventory management involves guaranteeing maximal availability of blood while minimizing wastage. Benchmark for the guidance of O (Rh) D-negative red blood cells (ONEG RBCs) is not widely available. In this study, we aimed to identify the areas of improvement in blood center inventory of ONEG RBCs through a clinical audit. MATERIALS AND METHODS: During April 2017 to March 2018, patients who received ONEG RBCs units were studied for their demographics, primary reason for admission, location, and clinical condition. Data were collected from computerized blood center information system, online integrated laboratory data (Integrated Laboratory Management System), and patients’ medical record charts. Children at ≤18 years were included in the pediatric population as per our institutional criterion while a female between 15 and 49 years was considered as having childbearing potential according to previously published data. RESULTS: Overall, 807 units (2.8%) of ONEG RBCs were transfused during 577 transfusion events with a median (inter quartile range) of 2 (1–3) units per patient in each transfusion event. Recipients of ONEG RBCs were 221 unique patients including 91 females (41%) and 130 males (59%) and only 44 (20%) females had child-bearing potential. Overall, 72 of 807 red cell units (8.9%) were transfused to young females of O/non-O negative/unknown group and were classified as “obligatory.” Neonates, pediatric patients, chronically transfused, and bone marrow transplant recipients received 337 of 807 (42%) units and were marked as “acceptable.” Transfusion of 398/807 units (49%) to females of nonchildbearing potential and adult males could have been saved for those with a mandatory transfusion requirement of ONEG RBCs. CONCLUSIONS: This clinical audit showed that 409 of 807 of ONEG RBCs (51%) were transfused according to the guidelines while 398 of 807 of these (49%) could have been saved for other mandatory requirements. Appropriate policies, planning, education of physicians, and regular clinical audits are needed to bring the desired change in transfusion practices.
Dengue virus belongs to flavivirus family which gains entry into the host organism through skin following an infected mosquito bite. Humoral, cellular, and innate host immune responses are involved in the progression of the disease. Dengue fever is getting common in Pakistan, and at times shows high mortality, but there is limited literature available. Therefore, this study was designed to evaluate hematological parameters in patients with dengue fever. This was a retrospective cross-sectional study conducted in the Department of Microbiology, Dow Diagnostic Reference and Research Laboratory, Karachi, Pakistan from 1 January 2021 till December 2021. A total of 6140 were collected, out of which 1746 were found positive. Dengue infection was confirmed by rapid screening NS1 antigen by ICT method. IgM antibodies were detected by Enzyme linked immunosorbent assay (ELISA). Hematological analysis was performed on Sysmex analyzer. Among the 1746 positive samples, 1036 (60%) were males and 710 (40%) were females. More than 10 hemoglobin and 45 hematocrits were found in greater than 40% of cases. Leucopenia less than 4000 was observed mostly in age group 0-20 years. Eosinophilia, basophilia, lymphocytosis and atypical lymphocytosis were shown equally in all age groups. Our study found greater incidence of Dengue fever among 21-40 years of age group with male predominance. Hematological spectrum revealed thrombocytopenia, lymphocytosis, high hematocrit, eosinophilia, basophilia and monocytosis at the time of diagnosis.
Niemann-Pick disease has an autosomal recessive inheritance pattern and occurs due to a deficiency of a lysosomal enzyme, sphingomyelinase. It causes variable clinical signs and symptoms such as hepatosplenomegaly, delayed milestones, and peripheral cytopenia due to bone marrow involvement. Here, we report a case of a child who presented with hepatosplenomegaly and pancytopenia, who was later found to have Niemann-Pick disease on bone marrow examination. This case highlights the case presentations of this rare disease and the importance of bone marrow trephine in prompt diagnosis and management of a patient.
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