Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reported cases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 %), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 %).
ObjectivesTo determine the prevalence of hepatitis B, hepatitis C, and human immunodeficiency virus (HIV) in chronically transfused β-thalassemia major (TM) patients, and to assess their quality of life (QoL).MethodsThis cross-sectional study was conducted in three different thalassemia centers located in Peshawar, Khyber Pakhtunkhwa from January to July 2019. These centers provide screened blood and essential medical care for thalassemia patients. These centers include the Fatimid Foundation, Hamza Foundation, and Rehman Medical Institute, Peshawar, Khyber Pakhtunkhwa. A total of 431 blood transfusion-dependent β-thalassemia patients registered at these centers were selected. QoL in β-TM patients was assessed by a newly developed instrument, the TranQoL questionnaire. For the data analysis procedure, Microsoft Excel and Statistical Package for the Social Sciences; version 22 (SPSS Inc., Chicago, IL) was used.ResultsA total of 431 patients were included in our study. The ages ranged from five years to 23 years with a mean age of 11.54 ± 3.6 years; 58.93% were male and the rest were female with a male to female ratio of 1.43:1. A total of 129 (29.93%) patients were infected by transfusion-transmitted infections (TTIs). Hepatitis C virus (HCV) was found prevalent in 23.66%, hepatitis B virus (HBV) was found in 4.87%, and HIV was found prevalent in 1.39% cases. The results showed a high proportion of HCV in males 27.95% as compared to females 17.51% (p value = 0.31). Patients were divided into high (good) QoL score of >50 and low (poor) score of <50. In patients with hepatitis C, the QoL was poor in 90 (88.23%) patients and was good in only 12 (11.76%) patients (p value=0.01); in the hepatitis B group, it was good in only eight (38.09%) and poor in 13 (61.90%) patients (p-value 0.04), and for patients with HIV, it was poor in all six patients (p=0.001).ConclusionOur study concludes that transfusion-transmitted disease is very high and that HCV is the leading TTI followed by HBV and HIV. QoL in patients with TTIs was poor. The use of advanced technology in blood screening, voluntary donations, donor selection, and asepsis during blood transfusion is imperative to curtail the transmission.
The aim of our study was to correlate liver function tests with serum ferritin levels in multitransfused thalassemia patients. Methods This was a descriptive cross-sectional study conducted in the department of hematology, Khyber Medical University, from January 2018 to December 2018. Thalassemia patients of either sex dependent on transfusion ≥ 1 year and having a confirmatory report of the disease were included in our study. The nonprobability convenience sampling technique was used. The Pearson correlation coefficient was applied to observe the correlation between serum ferritin level and liver function tests. A p-value of ≤0.05 was considered statistically significant. SPSS version 23 (SPSS Inc., Chicago, Illinois) was used for data analysis. Results A total of 138 subjects of age range 2-23 years, with a mean age of 12.08 ± 6.02 years, were included in our study. The mean serum ferritin of patients in our study was 3278.64 ng/ml with the lowest of 285.2 ng/mL and the highest of 10940.2 ng/ml. With the increase in serum ferritin levels, a rapid increase in alanine aminotransferase (ALT), aspartate aminotransferase (AST), and alkaline phosphatase (ALP) levels was seen. When serum ferritin levels were correlated with total bilirubin level, the bilirubin level remains static with a further increase in serum ferritin levels. Conclusion It was deduced that iron deposition is the ultimate reason for increased liver enzymes. There was a positive correlation between serum ferritin and ALT, AST, and ALP while a weak connection was found between serum ferritin and bilirubin levels.
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