Paediatric Thrombosis: A Five-Year Experience From A Tertiary Care Center Of Pakistan.

BACKGROUND: Advances in imaging techniques and longer survival of chronic medical conditions contribute to the increase in paediatric thrombosis. We aim to determine the incidence, underlying risk factors, management and clinical outcome of paediatric thrombosis at a multidisciplinary facility of Pakistan. METHODS: A retrospectively analysis of the medical records of patients in the paediatric age group admitted at the Aga Khan University hospital from January 2013-September 2018 was performed. Site of thrombosis, associated risks factors, management options and outcome of thrombotic event were evaluated. RESULTS: Of the 22,320 paediatric hospitalization, 35 paediatric patients were diagnosed with thrombosis (15 cases per 10,000 admissions). The median age of the study group was 15 years and twenty patients (57%) were male. The commonest site of thrombosis was in lower limb venous 11 (31%), followed by upper limb venous thrombosis 6 (17%), abdominal vein thrombosis 7 (20%), cerebral venous thrombosis 5 (14%), pulmonary embolism and arterial thrombosis 3(9% each). Eighty three percent had underlying clinical condition including central venous catheter [CVC] (26%), malignancy and infection (14% each), antiphospholipid antibody syndrome (9%), inherited thrombophilia (9%), congenital heart disease (6%), while thrombotic thrombocytopenic purpura and autoimmune disorder (3% each). Twelve (34%) patients were treated with heparin only, 8 (23%) received heparin followed by warfarin while warfarin as a single agent was given in 2 (5.7%) patients. One patient died of pulmonary embolism while 9 (25%) had persistence or recurrence of thrombosis. CONCLUSIONS: Incidence of paediatric thrombosis was 0.15%. CVC placement was the most common associated risk factor. Warfarin and heparin both were found to be safe anticoagulation option. Recurrence rate was found to be high.

Different Shapes Of Megakaryocytes In Essential Thrombocythemia.

Essential thrombocytopenia is the myeloproliferative neoplasm associated with the JAK2/CALR/MPL mutation. It is characterized by an increase in thrombocytes and abnormal megakaryocytes. WHO established the diagnostic criteria for diagnosing the myeloproliferative disorder, which is the combination of molecular, clinical, and histological findings. The appearance of megakaryocytes on bone marrow biopsy is the distinguishing feature to identify myeloproliferative neoplasm, and this short review would like to emphasize the presentation of megakaryocytes in bone marrow biopsy.

Association of Factor V Leiden G1691A and Prothrombin gene G20210A mutations with adverse pregnancy outcomes.

OBJECTIVE: To determine the association of Factor V Leiden / prothrombin gene mutation in Pakistani women with adverse pregnancy outcomes. METHOD: The prospective study was conducted at the Aga Khan University Hospital, Karachi, from January 1 to December 31, 2016, and comprised females > 40 years having history of two or more foetal losses with no apparent aetiology. Restriction fragment length polymorphism- Polymerase chain reaction was performed using MnlI and HindIII restriction enzymes for factor V Leiden G1691A and prothrombin gene mutation G20210A. Females with two or more consecutive normal pregnancies were enrolled as the control group. Data was analysed using SPSS 19. RESULTS: Of the 172 participants with a mean age of 29.3±5.9 years (range: 19-38 years). 86(50%) each were healthy controls and those with recurrent pregnancy loss. There were 238 livebirths among the controls compared to 13 in the other group. Factor V Leiden G1691A was identified in 2(2.3%) women, and prothrombin gene mutation G20210A in 1(1.2%) woman in the patient group, while no mutation was identified in the control group. CONCLUSIONS: The prevalence of Factor V Leiden / prothrombin gene mutation in women with recurrent pregnancy loss was found to be very low.

Extensive iron overload in bone marrow: A cause of pancytopenia in a thalassemia major patient - A case report.

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.

Better get moving on laboratory quality assurance.

PURPOSE: With recent advances in laboratory hematology automation, emphasis is now on quality assurance processes as they are indispensable for generating reliable and accurate test results. It is therefore imperative to acquire efficient measures for recognizing laboratory malfunctions and errors to improve patient safety. The paper aims to discuss these issues. DESIGN/METHODOLOGY/APPROACH: Moving algorithm is a quality control process that monitors analyzer performance from historical records through a continuous process, which does not require additional expenditure, and can serve as an additional support to the laboratory quality control program. FINDINGS: The authors describe an important quality assurance tool, which can be easily applied in any laboratory setting, especially in cost-constrained areas where running commercial controls throughout every shift may not be a feasible option. ORIGINALITY/VALUE: The authors focus on clinical laboratory quality control measures for providing reliable test results. The moving average appears to be a reasonable and applicable choice for vigilantly monitoring each result.

"A" for Life.

This is a story of a medical intern whose cascade of baffling thoughts about pursuing a career finally led to a gratifying conclusion. This young intern is easily swayed by the opinions of others, but circumstances proved that the field he chose was the one he was made for. This is a story of determination and belief in one's ability, which gave the power to fight against the odds.

Performance evaluation of a coagulation laboratory using Sigma metrics.

Purpose Two-thirds of medical decisions are based on laboratory test results. Therefore, laboratories should practice strict quality control (QC) measures. Traditional QC processes may not accurately reflect the magnitude of errors in clinical laboratories. Six Sigma is a statistical tool which provides opportunity to assess performance at the highest level of excellence. The purpose of this paper is to evaluate performance of the coagulation laboratory utilizing Sigma metrics as the highest level of quality. Design/methodology/approach Quality indicators of the coagulation laboratory from January 1, 2009, to December 31, 2015, were evaluated. These QIs were categorized into pre-analytical, analytical and post-analytical. Relative frequencies of errors were calculated and converted to Sigma scale to determine the extent of control over each process. The Sigma level of 4 was considered optimal performance. Findings During the study period, a total of 474,655 specimens were received and 890,535 analyses were performed. These include 831,760 (93.4 percent) routine and 58,775 (6.6 percent) special tests. Stat reporting was requested for 166,921 (18.7 percent). Of 7,535,146 total opportunities (sum of the total opportunities for all indicators), a total of 4,005 errors were detected. There were 2,350 (58.7 percent) pre-analytical, 11 (0.3 percent) analytical and 1,644 (41 percent) post-analytical errors. Average Sigma value obtained was 4.8 with 12 (80 percent) indicators achieving a Sigma value of 4. Three (20 percent) low-performance indicators were: unacceptable proficiency testing (3.8), failure to inform critical results (3.6) and delays in stat reporting (3.9). Practical implications This study shows that a small number of errors can decrease Sigma value to below acceptability limits. If clinical laboratories start using Sigma metrics for monitoring their performance, they can identify gaps in their performance more readily and hence can improve their performance and patient safety. Social implications This study provides an opportunity for the laboratorians to choose and set world-class goals while assessing their performance. Originality/value To the best of the authors' knowledge and belief, this study is the first of its kind that has utilized Sigma metrics as a QC tool for monitoring performance of a coagulation laboratory.

Fulminant hemolysis in glucose-6-phosphate dehydrogenase deficiency.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked disorder affecting some 400 million people worldwide. Though clinically silent, it may result in hemolysis on oxidative stress induced by drugs or infections. Viral hepatitis A with coexisting G6PD deficiency can be devastating associated with severe hemolysis, anemia, renal failure, and hepatic encephalopathy.

A Rare Haemoglobin Variant Identified as K Woolwich in a Pakistani Male.

Haemoglobin (Hb) K Woolwich is a rare Hb variant which was first described in a family from West Indies and its occurrence was later reported mainly in Black families from many African countries. We report a case of a young male who came for evaluation of anemia. His complete blood count showed hypochromic, microcytic anemia and his serum ferritin was low. Hb electrophoresis done as part of initial workup showed an abnormal band which moved faster than HbA. For further evaluation, Hb analysis by high performance liquid chromatography was subsequently performed and revealed Hb K Woolwich. This is a rare Hb variant recognized in Pakistani population for the first time through careful interpretation of the chromatographic behavior of the Hb.