Hematology Commons^™

Frequency Of Von Willebrand Disease And Its Types: Data From A Tertiary Care Hospital Of Karachi, Pakistan, Jyoti Mohan Lal, Anila Rashid, Natasha Ali

Department of Pathology and Laboratory Medicine

Introduction: von Willebrand disease is the most common inherited bleeding disorder. It is classified into 3 main types, type I and III are quantitative defects whereas type II is qualitative defect which is further sub-classified in to II-A, II-B, II-M, II-N. The aim of the present study is to see frequency and types of von Willebrand disease in a tertiary care hospital of Karachi, Pakistan.
Materials and methods: This retrospective cross-sectional study was conducted in the section of Hematology and Transfusion Medicine, Department of Pathology and Laboratory Medicine, the Aga Khan University hospital, Karachi, from May 2020 to April 2021 …

Simultaneous Onset Of Haematological Malignancy And Covid: An Epicovideha Survey, Chiara Cattaneo, Jon Salmanton-García, Francesco Marchesi, Shaimaa El-Ashwah, Federico Itri, Barbora Weinbergerová, Maria Gomes Da Silva, Michelina Dargenio, Julio Dávila-Valls, Natasha Ali

Department of Pathology and Laboratory Medicine

Background: The outcome of patients with simultaneous diagnosis of haematological malignancies (HM) and COVID-19 is unknown and there are no specific treatment guidelines.
Methods: We describe the clinical features and outcome of a cohort of 450 patients with simultaneous diagnosis of HM and COVID-19 registered in the EPICOVIDEHA registry between March 2020 to February 2022.
Results: Acute leukaemia and lymphoma were the most frequent HM (35.8% and 35.1%, respectively). Overall, 343 (76.2%) patients received treatment for HM, which was delayed for longer than one month since diagnosis in 57 (16.6%). An overall response rate was observed in 140 (40.8%) patients …

Lymphoma With Tuberculous Granulomas, Jyoti Mohan Lal, Anila Rashid

Department of Pathology and Laboratory Medicine

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

Role Of Gut Microbiome In Covid-19: An Insight Into Pathogenesis And Therapeutic Potential, Ikram Hussain, Gabriel Liu Yuan Cher, Muhammad Abbas Abid, Muhammad Bilal Abid

Department of Pathology and Laboratory Medicine

Coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), resulted in an unprecedented global crisis. Although primarily a respiratory illness, dysregulated immune responses may lead to multi-organ dysfunction. Prior data showed that the resident microbial communities of gastrointestinal and respiratory tracts act as modulators of local and systemic inflammatory activity (the gut-lung axis). Evolving evidence now signals an alteration in the gut microbiome, brought upon either by cytokines from the infected respiratory tract or from direct infection of the gut, or both. Dysbiosis leads to a "leaky gut". The intestinal permeability then allows access to …

Sickle Haemoglobin: How Critical Are Laboratory Quality Measures For Accurate Identification?, Nazish Sana, Muhammad Shariq Shaikh

Department of Pathology and Laboratory Medicine

No abstract provided.

Karachi Cancer Registry (Kcr): Age-Standardized Incidence Rate By Age-Group And Gender In A Mega City Of Pakistan, Shahid Pervez, Adnan Jabbar, Ghulam Haider, Shamvil Ashraf, Muhammad Asif Qureshi, Fouzia Lateef, Imtiaz Bashir, Manzoor Zaidi, Mohammad Khurshid, Mohammad Saeed Quraishy

Department of Pathology and Laboratory Medicine

Objectives: To estimate the cancer incidence by age group and gender for the population of Karachi Division by analyzing the Karachi Cancer Registry data of 2017-19.
Settings: The population of Karachi division is 16.1 million according to national census 2017. 'Karachi Cancer Registry' which is a part of 'National Cancer Registry' is collecting data from eight major hospitals in Karachi since 2017. For outcome measures, cancer counts and the age standardized incidence rates (ASIR) per 100,000 population were computed for age groups (0-14, 15-19 and ≥20 years), in both genders and all cancer site/type.
Methods: The population denominators were based …

Worldwide Network For Blood And Marrow Transplantation Recommendations For Establishing A Hematopoietic Stem Cell Transplantation Program In Countries With Limited Resources, Part Ii: Clinical, Technical, And Socioeconomic Considerations, Mahmoud Aljurf, Daniel Weisdorf, Shahrukh Hashmi, Amr Nassar, Eliane Gluckman, Mohamad Mohty, Doug Rizzo, Marcelo Pasquini, Mehdi Hamadani, Salman Adil

Department of Pathology and Laboratory Medicine

The development of hematopoietic stem cell transplantation (HSCT) programs can face significant challenges in most developing countries because such endeavors must compete with other government health care priorities, including the delivery of basic services. Although this is may be a limiting factor, these countries should prioritize development of the needed expertise to offer state-of-the-art treatments, including transplantation, by providing financial, technological, legal, ethical, and other needed support. This would prove beneficial in providing successful programs customized to the needs of their population and potentially provide long-term cost savings by circumventing the need for their citizens to seek care abroad. The …

Plasma Cell Leukemia Mimicking Hairy Cell Leukemia: Extended Role Of Immunophenotyping In Correct Diagnosis, Ayesha Majeed Memon, Natasha Ali

Department of Pathology and Laboratory Medicine

No abstract provided.

Infections In Patients With Multiple Myeloma Treated With Conventional Chemotherapy: A Single-Center, 10-Year Experience In Pakistan, Mohammad Faizan Zahid, Natasha Ali, Myra Nasir, Maria Haider Baig, Mustafa Iftikhar, Syed Usman Bin Mahmood, Arhama Malik, Sara Atif, Mohammad Asim Beg

Department of Pathology and Laboratory Medicine

Introduction: Multiple myeloma (MM) is a common hematologic malignancy with variable degrees of immunodeficiency. Disease- and treatment-related compromise of the immune system predisposes patients to infections, which are a major cause of morbidity and mortality.
Objective: We aimed to establish the incidence and main characteristics of infections in MM patients treated at our center over a 10-year period.
Method and results: Of the 412 patients retrospectively analyzed, 154 (37.4%) were documented to have at least one episode of infection and were included in this study. A total of 244 infectious episodes were documented. The most common site of infection was …

Unique Classification Of Parathyroid Dysfunction In Patients With Transfusion Dependent Thalassemia Major Using Nomogram: A Cross Sectional Study, Hafsa Majid, Lena Jafri, Jamsheer Jehangir Talati, Sibtain Ahmed, Bushra Moiz, Aysha Habib Khan

Department of Pathology and Laboratory Medicine

Introduction: Hypoparathyroidism is a rare complication of iron overload in patients with transfusion dependent β thalassemia major (β-TM). We aim to determine the prevalence of parathyroid dysfunction in patients with β-TM.
Methods:Diagnosed cases of transfusion dependent β-TM between 5 and 17 years of age were recruited from outpatient clinics of a non-profit organization in Karachi, Pakistan. Blood and urine samples were collected in fasting to determine Ca, P, Alb, Mg, Cr 25OHD and iPTH. Patients were grouped on the basis of upper and lower levels of Ca, 25OHD and iPTH for assessing parathyroid dysfunction into primary hypoparathyroidism [low calcium …

Blood Film Says It All! Rare Case Of Congenital Ttp Misdiagnosed As Itp, Ayesha Majeed Memon, Natasha Ali

Department of Pathology and Laboratory Medicine

We report a case of a 16-year-old female who presented with bleeding diathesis. Peripheral blood film examination was consistent with microangiopathic hemolytic anemia with 7% fragmented red blood cells. The ADAMTS13 level was 40 ng/ml (reference range: 630-850 ng/ml). She responded to plasma exchange therapy and methylprednisolone and was discharged in a stable condition.

Hematological Profile And Gametocyte Carriage In Malaria Patients From Southern Pakistan, Najia Karim Ghanchi, Mohammad Hassaan Khan, Muhammad Abdullah Arain, Mustafa Bin Ali Zubairi, Ahmed Raheem Buksh, Muhammad A. Khan, Mohammad A. Beg

Department of Pathology and Laboratory Medicine

Background: Malarial infection is a major cause of concern, both worldwide and in Pakistan. Gametocytes are the sexual forms of the parasite that are essential for transmission. They fuse inside the mosquito to develop sporozoites. Gametocytes of the plasmodium parasites, which cause the infection, differentiate into male and female gametocytes. These gametocytes constitute the sexual stage of the malaria parasite and are essential in transmission of the disease from human to vector Anopheles. Gametocytes are affected by factors such as host immunity, drug treatment, reticulocytemia, anemia, low levels of asexual parasitemia and stress to the parasite. The aim of this …

Distribution Of Chromosomal Abnormalities Commonly Observed In Adult Acute Myeloid Leukemia In Pakistan As Predictors Of Prognosis, Muhammad Shariq Shaikh, Zeeshan Ansar Ahmed, Mohammad Usman Shaikh, Salman Naseem Adil, Mohammad Khurshid, Tariq Moatter, Anila Rashid, Farheen Karim, Ahmed Raheem, Natasha Ali

Department of Pathology and Laboratory Medicine

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. The study was conducted to determine the distribution of cytogenetic abnormalities in Pakistani adult patients with AML in order to have insights regarding behavior of the disease.
Methods: A …

Inherited Bleeding Disorders-Experience Of A Not-For-Profit Organization In Pakistan, Shabneez Hussain, Shahida Baloch, Azra Parvin, Akbar Najmuddin, Farhana Musheer, Mubashra Junaid, Rab Nawaz Memon, Fareeda Bhanbhro, Hayat Ullah, Bushra Moiz

Department of Pathology and Laboratory Medicine

Patient registry is a powerful tool for planning health care and setting groundwork for research. This survey reports a detailed registry of inherited bleeding disorders (IBD) and their management at a not-for-profit organization in a developing country to form the basis for planning development and research. We reviewed medical records of patients with IBD from 8 hemophilia treatment centers of Fatimid Foundation located in various cities. Information collected included sociodemographic data, diagnostic tests, severity of hemophilia A and B, number of bleeding episodes per year, site and frequency of hemarthrosis, and seropositivity for viral diseases. We analyzed 1497 patients from …

No Risk Reduction For Plasmodium Vivax Malaria In Sickle Cell Disease, Bushra Moiz, Ayesha Majeed Memon

Department of Pathology and Laboratory Medicine

Hemoglobin S is known to protect against uncomplicated Plasmodium falciparum malaria. However, there is paucity of the literature regarding interaction of HbS and other malaria species. Usually, P. vivax malaria is a relapsing condition, and without radical cure with primaquine, recurrence may be observed even with hemoglobin S.

Adequacy Of Platelet Counting By Automated Haematology Analyzers: An Insight On Current Methodologies, Muhammad Shariq Shaikh, Noor Rahman Khan, Zeeshan Ansar Ahmed

Department of Pathology and Laboratory Medicine

No abstract provided.

Cold Agglutinins In Peripheral Blood With Atypical Cells With An Owl-Eye Appearance In Bone Trephine, Ayesha Majeed Memon, Farheen Karim

Department of Pathology and Laboratory Medicine

Autoimmune hemolytic anemia (AIHA) is a form of hemolytic anemia in which red cells lysis occurs due to presence of an autoantibody. Association of AIHA is well known with lymphoproliferative disorders, especially with non-Hodgkin's lymphoma. However, AIHA in association with Hodgkin's lymphoma is seen occasionally. Of the AIHA associated with Hodgkin's lymphoma, most are of warm type or mixed type. Cold AIHA, as seen in our case, is very rare in Hodgkin's lymphoma.

Ectopic Enterobius Vermicularis Iinfestation; An Extremely Rare Cause Of Mesenteric Lymphadenopathy Mimicking Tuberculous Lymphadenitis, Summaya Zafar, Muhammad Usman, Zubair Ahmad

Department of Pathology and Laboratory Medicine

Enterobius vermicularis (EV) is a pinworm which commonly resides in the lumen of the intestinal tract and lays eggs on the perianal skin. However, rarely the worm can infest various other sites in the body and cases with infestation of such ectopic sites have been reported in literature. Rare cases of mesenteric lymph node involvement have also been reported. We report a case in a young male who presented with signs and symptoms of acute appendicitis. During surgery, enlarged mesenteric lymph nodes were identified. Histological examination revealed adult worm in the appendiceal lumen. Histological examination of mesenteric lymph node revealed …

Hematopoietic Stem Cell Transplantation In Pakistan – Country Report, Parvez Ahmed, Tahir Sultan Shamsi, Salman Adil, Tariq Mahmood Satti, Qamar Un Nisa Chaudhry, Tahir Sultan Shamsi, Syed Kamran Mahmood, Saqib Ansari, Natasha Bahadur Ali, Tariq Ghafoor

Department of Pathology and Laboratory Medicine

No abstract provided.

Cerebral Venous Sinus Thrombosis In A Patient With Undiagnosed Factor Vii Deficiency, Hira Qadir, Anila Rashid, Salman Naseem Adil

Department of Pathology and Laboratory Medicine

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). Oral contraceptive pill was found to be prothrombotic risk factor. The CVSToccurred in spite of the congenital FVII deficiency indicating that no definitive antithrombotic protection is assured by this defect. Low molecular weight heparin and anti-Xa assay were found to be safe choice of anticoagulation and monitoring, respectively, in this patient

A Rare Haemoglobin Variant Identified As K Woolwich In A Pakistani Male, Sidra Asad Ali, Natasha Bahadur Ali

Department of Pathology and Laboratory Medicine

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 …

Hiv Prevalence In Blood Donors And Recipients In Pakistan: A Meta-Analysis And Analysis Of Blood-Bank Data, Bushra Moiz, Barkat Ali, Muhammad Hasnain Chatha, Ahmed Raheem, Hasan Abbas Zaheer

Department of Pathology and Laboratory Medicine

Background:
The first instances of HIV-antibody detection in donated blood in Pakistan were reported in 1988. Since then, documentation of HIV in blood donors and of rates of transmission via transfusion has been limited. Previously assumed to have a low prevalence, HIV is an increasing health concern in Pakistan. Since there is no national, centralized blood-banking system, there are no reliable data on which to base estimated risks of transfusion-associated HIV infection. This study was therefore conducted to estimate the prevalence of HIV in blood donors and recipients in Pakistan between 1988 and 2012.
Methods:
Meta-analyses were undertaken of reported …

Leishmania Donovani Bodies In Bone Marrow, Natasha Ali, Shabneez Hussain

Department of Pathology and Laboratory Medicine

We report a case of a 5-year-old female, resident of Afghanistan, who presented with fever and massive splenomegaly. Bone marrow revealed Leishmania donovani bodies (LD bodies) in macrophages characterized by a kinetoplast and characteristic double dot appearance. She was diagnosed as visceral leishmaniasis which is transmitted by sandflies (Phlebotomus).

Response To Imatinib Mesylate In Patients With Early Chronic Phase Chronic Myeloid Leukemia And Derivative Chromosome 9 Deletion Or Clonal Evolution, Mohammad Usman Shaikh, Tariq Moatter, Naveen Naz Syed, Natasha Bahadur Ali, Salman Adil

Department of Pathology and Laboratory Medicine

Objectives: The significance of clonal evolution and derivative chromosome 9 in Philadelphia-positive CML is not fully characterized and studies have yielded conflicting results. After working on emergence of clonal evolution from our region, we continued to find out the response of Imatinib Mesylate on such cases of CML treated in our center.
Materials and methods: We conducted a cross sectional, prospective analysis on response of Imatinib Mesylate on patients with Philadelphia positive chronic myeloid leukemia with clonal evolution treated from period of September 2007 till 2010. Patients were grouped on basis of cytogenetic analysis performed by conventional cytogenetic and fluorescence …

A Review Of G6pd Deficiency In Pakistani Perspective, Bushra Moiz

Department of Pathology and Laboratory Medicine

No abstract provided.

Frequency And Outcome Of Graft Versus Host Disease After Stem Cell Transplantation: A Six-Year Experience From A Tertiary Care Center In Pakistan, Natasha Ali, Salman Naseem Adil, Mohammad Usman Shaikh, Nehal Masood

Department of Pathology and Laboratory Medicine

Objective: The objective of this study was to evaluate the frequency and outcome of graft versus host disease after stem cell transplantation for various haematological disorders in Pakistan.
Materials and Methods: Pretransplant workup of the patient and donor was performed. Mobilization was done with G-CSF 300 mu g twice daily for five day. Standard GvHD prophylaxis was done with methotrexate 15mg/m(2) on day +1 followed by 10mg/m(2) on days +3 and +6 and cyclosporine. Grading was done according to the Glucksberg classification.
Results: A total of 153 transplants were done from April 2004 to December 2011. Out of these were …

Allogeneic Stem Cell Transplantation In Acute Myeloid Leukemia, Natasha Bahadur Ali, Salman Adil, Mohammad Usman Shaikh, Nehal Masood

Department of Pathology and Laboratory Medicine

We report a case series of 12 patients with acute myeloid leukemia who underwent allogeneic stem cell transplant with a matched related donor. Male to female ratio was 1:1. The main complication post-transplant was graft-versus-host disease (n=7 patients). Transplant-related mortality involved one patient; cause of death was multi-organ failure. After a median follow up of 36.0±11.3 months, overall survival was 16%.

Clinical Significance Of Maternal Anti-Cw Antibodies: A Review Of Three Cases And Literature, Shabneez Malik, Bushra Moiz

Department of Pathology and Laboratory Medicine

Anti Cw antibody is a low frequency immunoglobulin (IgG) against red cell antigen- Cw. It is clinically significant and may cause haemolytic disease of the newborn. Due to its low frequency, it is not included in routine antenatal antibody screening panel. The incidence of Anti Cw antibodies has not been reported in our population. Here, we describe the clinical and laboratory spectra of three pregnant women having low titres anti Cw antibodies. Fortunately, haemolytic disease of the newborn was not encountered in any case.

Hemoglobin E Syndromes In Pakistani Population., Bushra Moiz, Amna Nasir, Anila Rashid, Tariq Moatter, Mashhooda Rasool Hashmi

Department of Pathology and Laboratory Medicine

Background:Hemoglobin E is an important hemoglobin variant with a worldwide distribution. A number of hemoglobinopathies have been reported from Pakistan. However a comprehensive description of hemoglobin E syndromes for the country was never made. This study aimed to describe various hemoglobin E disorders based on hematological parameters and chromatography. The sub-aim was to characterize hemoglobin E at molecular level.

Methods:

This was a hospital based study conducted prospectively for a period of one year extending from January 1 to December 31, 2008. EDTA blood samples were analyzed for completed blood counts and hemoglobin variants through automated hematology analyzer and …

Successful Use Of Rituximab In Evans Syndrome And Refractory Immune Thrombocytopenic Purpura, Muhammad Kashif, Adnan Qureshi, Salman Naseem Adil, Mohammad Khurshid

Department of Pathology and Laboratory Medicine

Immune cytopenias are mediated by auto-antibodies produced by B-lymphocytes. Conventional treatment of immune-mediated haematological disorders includes immunosuppression with steroids and other immune modulating therapies and in some refractory cases, splenectomy. Response rates to conventional and second-line agents are variable and a proportion of patients require lifelong immunosuppression to maintain the disease in remission. Rituximab, an anti- CD 20 monoclonal antibody has gained widespread acceptance in the management of B-cell malignancies. Additionally, it has been used to treat the disorders associated with autoantibody production. We report herein the successful use of Rituximab in the treatment of two patients with autoimmune cytopenias …