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Background:Diagnosis of fever of unidentified cause remains challenging despite the availability of modern diagnostic techniques. Pyrexia of Unknown Origin (PUO) may result from various etiologies, among which infectious diseases are largely responsible. Many diagnostic approaches are currently applied. A detailed history with a complete general physical examination followed by baseline investigations with other specific tests like imaging, microbiological tests, and biopsies is employed to diagnose PUO. This study was designed for the evaluation and effectiveness of bone marrow biopsy in the identification of PUO and highlighted the existing spectrum of diseases involved in PUO. Methodology:This Cross-sectional study was conducted at Dow University of Health Sciences, Karachi from 2015 to 2018 evaluated the records of patients who had bone marrow aspiration and biopsy Patients included in this study had a history of fever due to an Unfamiliar cause and met the Peters Dorf and Beeson criteria for PUO, i.e. fever for more than 3 weeks before diagnosis. Informed consent was taken from all patients. Results:We analyzed the medical histories of 67 patients (48 males and 19 females) who were recommended bone marrow biopsy and aspiration for the assessment of PUO. The mean age was 38 years. The most common clinical symptoms found in patients of PUO were fever, hepato-splenomegaly followed by abdominal pain and weight loss. Anemia was the most common hematological parameter found in this study. The most frequent diagnosis in biopsies of PUO patients was Non-Hodgkins Lymphoma in about 25% of patients. Conclusion:Certain laboratory and clinical parameters can detect major hematological diseases like malignancies when bone marrow biopsy is used for the workup of pyrexia of unknown origin. Bone marrow biopsy should be regarded as a constitutive part of the workup and diagnosis of pyrexia of unknown origin in conjunction with clinical findings. Link:http://aeirc-edu.com/ojs14/index.php/IJEHSR/article/view/542/644

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: Transfusion-related acute lung injury (TRALI) is a rare but potentially fatal complication of blood product transfusion. It is felt worldwide that TRALI is an underrecognized and underreported entity because of lack of awareness. AIM: The purpose of this study was to report all cases of TRALI diagnosed in a tertiary care hospital over a 5-year period. MATERIALS AND METHODS: This is a retrospective review of all TRALI cases reported from January 2011 to December 2015. All TRALI cases were identified from a manual review of reported transfusion reaction forms. For detailed information of all TRALI cases, medical record charts of patients were reviewed. The record of donors implicated in TRALI cases was derived from blood bank system. STATISTICAL ANALYSIS USED: The rate of TRALI cases per 1000 blood products transfused was computed by dividing the transfusion reactions by total number of all blood units transfused. RESULTS: Total number of transfusions during the study was 291,041. Six cases of TRALI were reported during this period. Rate of TRALI per 1000 units transfused was 0.02%. The mortality associated with TRALI was 33.3%. TRALI occurred following the transfusion of fresh-frozen plasma in one patient, packed red blood cells in two patients, and a mixture of blood components in three patients. In all cases, the donors were male. CONCLUSION: The rate of TRALI reported to our blood bank was found to be 0.02%, which is very low as compared to international data. This is the first comprehensive study on TRALI from the country and a step forward to create awareness about the importance of diagnosing and reporting TRALI.

Congenital thrombotic thrombocytopenic purpura (TTP) is an autosomal recessive disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and thrombosis. Congenital TTP should also be considered while investigating neonatal hyperbilirubinemia, hemolytic anemia, or isolated thrombocytopenia. This case is of an 8-year-old male child who presented with prolonged and recurrent history of thrombocytopenia and MAHA, first identified when he was seven weeks of age preceding neonatal hyperbilirubinemia. Peripheral blood smear examination showed thrombocytopenia and schistocytes. He then went through a series of laboratory investigations until at the age of seven years, when the ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) antigen level was performed and found to be low: 40 ng/ml (630-850). Subsequently, he received a trial of steroids and rituximab which were found to be ineffective and associated with complications. In this case, a definitive diagnosis was delayed until the age of eight years when a novel homozygous pathogenic frameshift variant ADAMTS13 c.3033delC, p.Cys1012AlafsX109 in exon 23 was identified. After receiving regular plasma infusions, thrombocytopenia and hemolysis improved. Congenital TTP should be considered in every neonatal hyperbilirubinemia, thrombocytopenia or hemolytic anemia to avoid delay in diagnosis. Early diagnosis through analysis of the ADAMTS13 gene is crucial for optimal management as well as for genetic counselling.

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.

Iron overload-associated organ damage in transfusion-dependent anemias is a well-known phenomenon. Here, we discuss a case of 28-year-old, poorly chelated thalassemia major patient, whose blood workup revealed pancytopenia and moderately raised serum ferritin levels. His bone marrow examination was performed which revealed massive iron overload. Aggressive iron chelation led to successful recovery of peripheral blood counts in his patient. This case focuses on the importance of early detection and timely management of reversible iron overload toxicities. Serum ferritin although is convenient marker to asses iron overload, but it should not be relied upon to assess the severity of iron overload. Hence, organ-specific diagnostic modalities must be used along with serum ferritin to assess the severity of iron overload to prevent long-term complications in patients with regular blood transfusions.

The International Society of ThrombosisHemostasis (ISTH) bleeding assessment tool (ISTH-BAT) is used to record bleeding symptoms in patients with possible bleeding disorders.To investigate the utility of the ISTH-BAT in predicting platelet dysfunction in individuals with suspected inherited platelet function disorders.Individuals with clinical evidence of bleeding and suspected inherited platelet function disorder and healthy volunteers were included in the study. The ISTH-BAT questionnaire was applied prior to light transmission aggregometry (LTA).A total of 261 participants were included (100 healthy volunteers, and 161 with suspected inherited platelet function disorders). The ISTH-BAT score in participants with suspected inherited platelet function disorders (median 2; interquartile range [IQR] 5-1) was significantly higher than in healthy volunteers (median 0; IQR 2-0). There was also a significant difference between participants with suspected inherited platelet function disorders with a platelet defect detected by LTA (median 4; IQR 8-3) and those with normal platelet function (median 2; IQR 3-1) (p 0.001). The ISTH-BAT score was associated with a demonstrable platelet defect on platelet function testing (area under the receiver operating characteristic curve = 0.8 [95% confidence interval 0.72-0.87, p = 0.001] and odds ratio 3.25 [95% confidence interval 2.13-4.37, p = 0.001]).The ISTH-BAT is a useful tool for documenting bleeding symptoms and the score obtained is also predictive of the presence of a platelet defect on LTA in patients with suspected inherited platelet dysfunction.

Secondary haemophagocytosis in malignant tumours have been reported in bone marrow. However we present engulfment of malignant apoptotic Ewing’s sarcoma cells by macrophages in the bone marrow. A 24 year old Pakistani male patient presented with complains of backache. After imaging, a laminectomy was performed the biopsy results of which were consistent with Ewing’s sarcoma. Subsequently for staging, bone marrow and trephine was done. The bone marrow aspirate showed markedly increased haemophagocytic activity with histiocytes engulfing malignant apoptotic Ewing’s sarcoma cells. The presence of histiocytes engulfing apoptotic Ewing’s sarcoma cells was of particular interest. Similar findings have rarely been reported.