Assessment of Hepatic Profile in Acquired Aplastic Anemia: An Experience From Pakistan.

INTRODUCTION:  Aplastic anemia (AA) is characterized by pancytopenia and hypocellular marrow in the absence of an abnormal infiltrate or increase in reticulin fibrosis. The diagnosis of AA is challenging at times due to decreased cellularity and overlapping morphological features with other bone marrow failure syndromes. Hepatitis-associated aplastic anemia (HAAA) is a rare variant in which patients typically present with jaundice and hepatitis followed by pancytopenia almost within 6 months. Post-hepatitis AA accounts for approximately 1-5% of cases, and invariably such cases are negative for the known hepatitis virus as well. There is limited literature available to understand the correlation of AA with hepatitis with none reported at the national level in our region. As AA is relatively more prevalent in Southeast Asia as compared to the western world and hepatitis is a prevalent disease in our population, the main purpose of this study was to assess the hepatic profile and determine the association of hepatitis in AA at the time of diagnosis. MATERIALS AND METHODS:  A cross-sectional study was carried out at the National Institute of Blood Disease and Bone Marrow Transplantation, Karachi, from November 2019 to December 2020 after the informed consent from patients. The study included all treatment-naïve patients of acquired AA with no prior history of taking steroids, immunosuppressive treatment, or chemoradiation therapy. Liver function tests, complete blood count, prothrombin time (PT), and activated partial thromboplastin time were performed, along with viral profiles (HAV, Hep B, Hep C, and HIV). SPSS version 23 (IBM Corp., Armonk, NY) was used for statistical analysis. Mean and standard deviations were computed for quantitative variables while percentages and frequencies were reported for qualitative variables. T-test was used to observe the main difference between groups and a p-value <0.05 was considered to be significant. RESULTS:  Out of a total of 351 patients, 29 (8.2%) patients with AA tested positive for viral hepatitis. Hepatitis A was the most prevalent hepatitis (4.0%), followed by hepatitis C (3.7%). The comparison of platelet counts in patients with and without hepatitis was reported to be of statistical significance (p-value < 0.05). A significant statistical difference (p-value < 0.0001) was found in platelet count and PT in patients of AA with and without hepatitis. CONCLUSION:  Overall, this study revealed that <10% of patients of AA had a positive screening for hepatitis A, B, and C and low platelet count, and PT was statistically significant when compared between the patients with and without hepatitis. Hepatitis being prevalent in our part of the world might have an important causal association with AA. Patients with AA should be screened for liver functions and viral hepatitis at the time of diagnosis. In addition to hepatitis A, B, and C and HIV, other causes of hepatitis should also be screened such as parvovirus B19, human herpes virus 16, and adenovirus which are not included in routine diagnostic viral testing panel.

The Outcome of Hodgkin Lymphoma With Reference to Prognostic Markers.

OBJECTIVES: This study aimed to determine the impact of prognostic markers on the outcomes of Hodgkin lymphoma. METHODS: It is a cross-sectional, single-center study. A total of 60 patients diagnosed with Hodgkin lymphoma were recruited for the study over five years between 2016 to 2020. The study setting was the National Institute of Blood and Bone Marrow Transplant in Pakistan. The Statistical Package for Social Sciences (SPSS) version 23 (IBM Corp., Armonk, NY, USA) was used for statistical analysis. RESULTS: In the study population, 63.3% of the patients were male (38/60), and 36.7% were female (22/60). Hodgkin lymphoma was divided into four stages: stage I (18.3%), stage II (18.3%), stage III (46.7%), and stage IV (16.7%). Patients in stage III had a higher value of hemoglobin (Hb) than in other stages of the disease. The erythrocyte sedimentation rate was high in 56.7% of stage III patients than in patients of the other stages. The lactate dehydrogenase (LDH) levels were not under the normal range in 51.6% of patients. Only 20% of patients in stage III had LDH values within the normal range, whereas 26.6% did not. CONCLUSION: There was a significant impact of prognostic factors on the survival of patients with Hodgkin lymphoma.

Frequency of Extended Red Cell Antigen Phenotype Among Patients of Hematological Diseases: A Single Center Study.

Background Alloimmunization of erythrocytes is a major problem in patients with hematological diseases that require frequent blood transfusions. Matching of extended red cell antigens of Kell, MNS, Kidd, and Duffy can decrease the risk of alloimmunization. Hence, in this study, the frequencies of the extended red cell phenotypes were explored. Objective To find out the frequency of extended red blood cell antigen phenotypes among patients with hematological diseases. Methods This cross-sectional research study was performed on 488 patients diagnosed with hematological diseases who required blood transfusion at the National Institute of Blood Disease and Bone Marrow Transplantation Karachi for a period of 1.42 years from November 2019 to March 2021. The blood of patients was analyzed for antigen phenotypes of different blood group systems including Kell, MNS, Kidd, and Duffy. The data obtained were interpreted. Results Among the 488 patients, 284 (58.20%) patients were male, and 204 (41.80%) patients were female with a mean age of 8.1 years. Beta thalassemia was the most common hematological disease reported in 354 (72.5%) of the patients. The most common blood group was O positive reported in 182 (37.3%) of the patients followed by B positive blood group in 124 (25.4%). The frequencies of extended red cell antigen phenotypes in the patients were K antigen 14 (2.9%), Kpa antigen 26 (5.3%), Kpb antigen 424 (86.9%), Fya antigen 360 (73.8%), Fyb antigen 260 (53.3%), Jka antigen 294 (60.2%), Jkb antigen 326 (66.8%), M antigen 410 (84.0%) and N antigen 306 (62.7%). Conclusion Beta thalassemia was the most common hematological disease followed by iron deficiency anemia, aplastic anemia, and acute leukemia. Patients with hematological diseases had a higher prevalence of Kpb antigen followed by M, Fya, Jkb, N, Jka, Fyb, Kpa, and K antigen. O positive was the most frequent blood group followed by B positive, A positive and AB positive blood group.

Frequency of Specific and Non-specific Inhibitors in Haemophilia A Patients.

OBJECTIVE: To determine the frequency of specific and non-specific inhibitors in haemophilia A patients. STUDY DESIGN: This is a cross-sectional study. PATIENTS AND METHODS: A total of 150 male haemophilia A patients were included in this cross-sectional study at the National Institute of Blood Diseases and Bone Marrow Transplant (NIBD), Karachi, Pakistan, from September 2019 to January 2022. RESULTS: Among 150 patients included in this study, 23 (15.3%) had an inhibitor and 127 (84.6%) did not. All patients had specific inhibitors against Factor VIII (FVIII). Non-specific inhibitors were not identified in our population. Among the patients in the inhibitor group, there were 13 (56.5%) in the severe (<1%) category. There were 10 (43.5%) patients in the moderate (1-5%) category. There were no patients in the mild category. The median inhibitor level was 15.4 Bethesda unit (BU). CONCLUSION: The development of inhibitors has not been identified as a major problem in our population. However, it is noteworthy that only 15.3% of patients with haemophilia A developed inhibitors in this data set. They were essentially treated with plasma and its products.

Coagulation Abnormalities and Risk Assessment in Acute Promyelocytic Leukemia: An Experience From a Resource-Constraint Country.

Introduction The objective of the study was to assess the impact of coagulopathy in risk-stratified acute promyelocytic leukemia (APML) patients irrespective of bleeding manifestation. Patients and methods This was a cross-sectional study design conducted at the National Institute of Blood Diseases and Bone Marrow Transplantation (NIBD & BMT) from November 2019 to December 2021. A total of 62 patients between three years to 74 years of age of either gender and treatment-naive cases of APML were included in the study. Morphological diagnosis was made on bone marrow samples, and confirmation was done by karyotyping/fluorescence in situ hybridization (FISH) and/or polymerase chain reaction (PCR). Complete blood count (CBC), prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimer, and fibrinogen levels were done for bleeding risk assessment. Cases other than APML and cases on treatment were excluded from the study. Results A total of 85 APML patients were registered at our institute. Among them, 62 (73%) were included in the analysis as per the inclusion criteria of the study. The median age was 32 (3-74) years, with a male predominance of 34 (55%). According to the Sanz score, 18 (29%) patients were noted to have low risk; however, 22 (35.4%) patients were found to have an intermediate-risk disease and 22 (35.4%) patients had high-risk disease. There was positive bleeding history among 44 (71%) patients, followed by fever in 28 (45%) patients. Raised PT, APTT, and D-dimer were found in 46 (74%), 38 (61%), and 52(83.8%) patients, respectively. Low fibrinogen levels were observed among 16 (26%) patients. The association of risk stratification and bleeding history with CBC and coagulation parameters was observed. Platelet count and total leucocyte count were noted to be significantly associated with risk stratification. However, there was no association observed between the rest of the parameters with risk stratification and bleeding. Conclusion The results of our study suggest that regardless of bleeding symptoms, coagulation parameters must be investigated at the time of diagnosis in patients with suspected APML, and in addition to all-trans-retinoic acid (ATRA), transfusion of fresh frozen plasma should be done. It has clinical value, and adding it to the algorithm of treatment would be beneficial to the patients in the developing world, where resources are already meager.