B-cell lymphoma research in Malaysia - A narrative review.

Lymphomas are a diverse group of malignant proliferations that arise as discrete tissue masses. The most widely accepted taxonomy for lymphoma is the World Health Organization classification of tumours of haematopoietic and lymphoid tissues, the 5th edition of which was released in June 2022. Most (85% to 90%) lymphoid neoplasms are of B cell origin. Mature B-cell neoplasms are a heterogeneous group of malignancies with similar disease courses and treatment paradigms. This review focuses on the various mature B-cell lymphomas in Malaysia, including Hodgkin lymphoma. A literature search was performed in various bibliographic databases. A total of 64 papers were included in this review. We found 15 papers on Hodgkin lymphoma, 14 on follicular lymphoma, 12 on Burkitt lymphoma, 5 on mucosa-associated lymphoid tissue (MALT) lymphoma, 4 on plasmablastic lymphoma, 3 on mantle cell lymphoma, 1 each on primary mediastinal large B-cell lymphoma, B-lymphoblastic lymphoma, and 3 on other unspecified B-cell lymphomas. The site, age, distribution, prognostic markers, and the various subclassification of B cell lymphomas were studied from these papers. Prognostic genetic markers in B-cell lymphomas include C-MYC, BCL2 and BCL6 as they are the most prevalent mutations in this condition. Anecdotal outcomes range from rapid fatality to unexplained spontaneous remission. This review adds to the existing literature on lymphoma in Malaysia by compiling the evidence that may lead to further research on the diagnosis and treatment of lymphoma in Malaysia and worldwide.

Diffuse large B-cell lymphoma research in Malaysia: A review.

Lymphomas are prevalent worldwide and a common malignancy reported in Malaysia. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of B-cell lymphomas accounting for 54% to 65% of all B-cell lymphomas and 39% to 57% of all malignant lymphomas. However, DLBCL comprises a heterogeneous group of diseases with different clinical presentations, biology and response to treatment. Recent advances in understanding the genetic landscape and molecular features of DLBCL have identified high-risk subsets with poor outcomes to chemo-immunotherapy that are actively being studied in various clinical trials. C-MYC is a proto-oncogene located in chromosome 8q24. 10 to 15 % of patients with newly diagnosed DLBCL have an underlying rearrangement of the MYC oncogene, resulting in dysregulated cellular survival and proliferation. Approximately half of these cases also carry a rearrangement of the anti-apoptotic proto-oncogene BCL2 and/or its transcription repressor BCL6. Over 20 case reports of DLBCL cases with notable features in Malaysia have found in the literature, in addition to a few extensive case series and included in this review. R-CHOP remains the mainstay of therapy and can help achieve control of long-term disease in nearly 90% of patients presenting with limited-stage and in up to 60% of those presenting with advanced stages. This review captures all 52 studies that reported DLBCL in Malaysia and summarises the essential aspects, including prevalence, subtype, prognostic markers clinical features in presentation and limited outcomes of cases when available.

Achalasia cardia: A five-year review in Hospital Tuanku Ja'afar, Seremban.

INTRODUCTION: Achalasia cardia is an oesophageal motility disorder that affects various age groups. This study focused on the epidemiological features of achalasia, its risk factors, treatment modalities offered and the clinical outcomes in a tertiary hospital. MATERIALS AND METHODS: A retrospective search was carried out on all patients with a diagnosis of achalasia cardia in Hospital Tuanku Ja'afar (HTJ), Seremban, Malaysia between 2014 and 2018. Demographic data, patient symptomatology, and definitive management options were determined from the records. Telephone interviews were conducted to evaluate patient satisfaction with the outcome of treatment. RESULTS: There were 30 patients with a newly diagnosed achalasia cardia in that 5-year period, with an equal incidence among men and women. The mean age of presentation was 44.63 ± 18.21 years. Malays formed the largest group. The mean weight and body mass index were 46.8 ± 10.4 kg and 18.0 ± 4.4 kg/m2 respectively. There was a wide range of duration of symptoms at presentation with a mean of 30.11 ± 35.29 months. Almost all patients presented with dysphagia (96.7%) while 70% also noted loss of weight. All patients underwent oesophagogastroduodenoscopy (OGDS) and 26 patients (86.7%) had barium swallow as part of diagnostic workup. A total of 18 patients underwent a laparoscopic Heller myotomy with or without Dor Fundoplication and/or cruroplasty while two patients (6.7%) underwent pneumatic dilatation as first treatment. Iatrogenic mucosal perforations were detected in 8 patients who underwent myotomy and fundoplication and were repaired intraoperatively. Of the patients who underwent myotomy and fundoplication, the mean weight increase was 15.6kg, increasing from 43.0 ± 8.4 kg to 58.6 ± 13.7 kg. All the patients who underwent treatment were satisfied with their treatment outcomes. CONCLUSION: Most patients with achalasia cardia deemed suitable for surgery and counselled accordingly accept surgery resulting in high levels of satisfaction and weight gain in almost all these patients. A small minority who opt for pneumatic dilatation may also achieve satisfactory outcomes comparable to surgery in the short term. Although rare, clinicians should be able to recognise this disease early as early intervention often leads to satisfactory longterm outcomes.