Clinico-Haematological Profile Of Chronic Lymphoproliferative Disorders In Patients Presenting With Lymphocytosis.

Objectives: To assess the spectrum and clinico-haematological profile of chronic lymphoproliferative disorders in patients presenting with lymphocytosis.

Methods: The cross-sectional, retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data related to cases of bone marrow aspirate and trephine from January to November 2020. Patients for whom the bone marrow was done for lymphocytosis were studied for the presence of lymphoproliferative disorders, sub-types and patients'characteristics.

Non-severe allergic transfusion reaction: A hidden cause of wastage of blood product and laboratory resources.

Background And Objectives: Blood transfusions are often needlessly aborted following a non-severe allergic reaction despite responding well to medication resulting into partial transfusion of the implicated blood product. This results in the wastage of untransfused blood component and resources spent on unnecessary laboratory work-up of these reactions.

Materials And Methods: We aimed to review the amount of blood product and laboratory resource wastage associated with non-severe allergic transfusion reaction (ATR) in a tertiary care hospital.

Bone marrow biopsy, an effective diagnostic modality for pancytopenia among paediatric and adult population.

Objective: To determine the aetiologies of pancytopenia based on bone trephine biopsy among paediatric and adult patients.

Method: The retrospective cross-sectional study was conducted at the Haematology Department of Aga Khan University Hospital, Karachi, and comprised data from June 1, 2016, to October 31, 2019 related to pancytopenia patients who underwent bone marrow biopsy. Data included age, gender, presenting symptoms, physical examination, complete blood count, peripheral smear, bone marrow aspirate and trephine biopsy findings and final diagnosis.

Indispensable role of immunophenotyping in diagnosing leukemic phase of blastic plasmacytoid dendritic cell neoplasm without cutaneous manifestation.

Blastic plasmacytoid dendritic cell neoplasm usually presents as skin lesions. Diagnostic error occurs when it primarily presents in leukemic phase without skin involvement. Triad of CD4, CD56 and CD123 immunophenotype expression is essential to avoid misdiagnosis of this rare hematological malignancy.

Lymphoma with tuberculous granulomas.

Chronic granulomatous inflammation is a common finding in lymphoproliferative disorders (LPDs), but it is important to exclude coexisting mycobacterium tuberculosis (MTB) especially in patients from areas of high endemicity. This case emphasizes the relevance of performing MTB culture on bone marrow exhibiting LPD and concomitant granulomas.

Reporting transfusion-related acute lung injury cases.

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.

Congenital Thrombotic Thrombocytopenic Purpura With a Novel ADAMTS13 Gene Mutation.

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.

Multivessel Percutaneous Coronary Intervention in a Patient With Bernard-Soulier Syndrome.

Bernard-Soulier syndrome, a congenital bleeding disorder, can rarely present with atherosclerosis and thrombosis. Acute coronary syndrome in such patients present a unique challenge as no standard set of guidelines exist for successful treatment. ().

Development and Pilot Testing of a Novel Tool for Evaluating Practical Skills in Hematopathology Residents in Pakistan.

Background: The mini-Clinical Evaluation Exercise (mini-CEX) and direct observation of procedural skills (DOPS) are reliable tools for work-based assessment of medical trainees. Tools of this type do not yet exist for evaluation of practical laboratory skills of pathology residents.

Objective: We developed and piloted a 9-item instrument for direct observation of laboratory skills (DOLS).

Distribution of Chromosomal Abnormalities Commonly Observed in Adult Acute Myeloid Leukemia in Pakistan as Predictors of Prognosis.

Objectives: The heterogenous response to treatment in acute myeloid leukemia (AML) can be attributed largely to the difference in cytogenetic features identified in between cases. Cytogenetic analysis in acute leukemia is now routinely used to assist patient management, particularly in terms of diagnosis, disease monitoring, prognosis and risk stratification. Knowing about cytogenetic profile at the time of diagnosis is important in order to take critical decisions in management of these patients.

Prospective Comparison of Point-of-Care Device and Standard Analyzer for Monitoring of International Normalized Ratio in Outpatient Oral Anticoagulant Clinic.

Point-of-care testing (POCT) coagulometers are increasingly being used in the hospital setting and patients' self-testing. We determined the agreement of prothrombin time international normalized ratio (INR) results by POCT coagulometer and laboratory instrument through a comparative analysis and investigated whether the results of POCT coagulometer can reliably be used without being confirmed by standard laboratory analyzer. A total of 200 INR measurements by POCT coagulometer (CoaguChek XS Pro) and laboratory analyzer (Sysmex CS2000i) were compared using Passing-Bablok regression analysis and Bland-Altman plot.

Clinico-hematological Profile of 184 Patients with Non-Hodgkin's Lymphoma: An Experience from Southern Pakistan.

Background: Non-Hodgkin lymphoma (NHL) is a diverse group of lymphoma comprises of divergent tumors with paradoxical clinical behaviors and potential difference in response to therapy. We conducted a data-base analysis on NHL patients to evaluate the clinico-epidemiological features and WHO spectrum of NHL in Pakistani patients.

Materials And Methods: This descriptive study was conducted over a period of 5 years from January 2011 to December 2015 at Hematology department of Liaquat National Hospital.

Cerebral Venous Sinus Thrombosis in a Patient with Undiagnosed Factor VII Deficiency.

Factor VII (FVII) deficiency is one of the rare inherited bleeding disorders. Thrombosis has been occasionally described in inherited FVII deficiency. Here, we report a young female with undiagnosed FVII deficiency who presented with cerebral venous sinus thrombosis (CVST).

Use of ISTH bleeding assessment tool to predict inherited platelet dysfunction in resource constrained settings.

Background: The International Society of Thrombosis & Hemostasis (ISTH) bleeding assessment tool (ISTH-BAT) is used to record bleeding symptoms in patients with possible bleeding disorders.

Aim: To investigate the utility of the ISTH-BAT in predicting platelet dysfunction in individuals with suspected inherited platelet function disorders.

Method: Individuals with clinical evidence of bleeding and suspected inherited platelet function disorder and healthy volunteers were included in the study.