Nutritional Status of the Children with Acute Lymphoblastic Leukemia at Diagnosis and after Completion of Induction

Background: Adequate nutrition is an important concern in children with leukemia. Malnutrition and weight lost are common and are due to verity of mechanism involving the tumor, the host response to the tumor such as infection and pharmacokinetics of chemotherapeutic drugs. Objective: To evaluate and compare the nutritional status of children with ALL at diagnosis and after completion of induction therapy. Methodology: This prospective observational study included 60 children newly diagnosed as ALL, aged 2-15 years, over a period from April 2012 to September 2012 in the Department of Pediatric Hematology and Oncology, BSMMU. The anthropometric measurements and serum albumin level were taken. Anthropometric indices are calculated by NCHS (WHO-2000) and classified as Z score. Children <-2 SD are considered as underweight (WFA), stunted (HFA) and wasted (WFH). Serum albumin level below 21g/dl is considered as severely malnourished. The Hb values of the children are compared with normal values by age. The children got induction chemotherapy according to MRC-11 protocol. They were in regular follow up and again anthropometric measurements and serum albumin level were taken after completion of induction. Results: Out of 60 children with ALL, 48 (70%) were underweight, 45 (75%) were stunted 36 (60%) were wasted at diagnosis. Incidence of malnutrition among leukemia children after completion of induction were 24 (40%) underweight, 45 (75%) were stunted and 6 (10%) were wasted. The results showed that children in the newly diagnosed stage had a higher prevalence of malnutrition. However no statistically significant difference in the nutritional status was found among newly diagnosed and after completion of induction in term of underweight and stunting but newly diagnosed patients had statistically significant wasting than patients who had completed induction chemotherapy. No patient showed severe malnutrition based on the cut-off point for serum albumin on both stages. All the children (100%) had less than normal range hemoglobin levels. Conclusion: Malnutrition was higher in children with newly diagnosed leukemia. Children had significant differences in the nutritional status in term of wasting at diagnosis than after completion of induction therapy. So, the nutritional status of children with leukemia should be monitor periodically.

Spectrum of Genetic Mutation in Beta Globin Gene in Various Type of Thalassaemia in Bangladesh

Background: Hb-E/Beta thalassaemia is a congenital haemoglobin disorder which is a compound heterozygous state consists of qualitative disorder like Hb E variant & quantitative Hb disorder caused by genetic mutation of Beta chain. Objective: The aim of the study was to identify the beta gene mutation in Hb E/Beta thalassaemia. Method: A total of 32 diagnosed Hb E/Beta thalassaemia patients were included in this cross-sectional study from May 2019 to July 2020. Genetic analysis was done by sanger sequencing. Results: In this observational study, we found 13 different types of Beta gene mutations. Heterozygous for IVS 1-5(G>C) mutation was most frequent (53.1%). Conclusion: Genetic mutation is the confirmatory diagnosis for thalassaemia as well as one of the main factors for clinical expression. Mutation pattern also varies according to the geographical distribution. So, this study shows the frequently found mutation in Bangladesh and should carry out routinely to point out phenotypic expression.

Hepatitis in Idiopathic Hypereosinophilic Syndrome: Report of an Unusual Case

World Health Organization defines a rare diagnosis Idiopathic hypereosinophilic syndrome (HES) as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. Most of the patients present with nonspecific symptoms, while others will present with symptoms of the affected organs, commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with idiopathic HES related hepatitis and achalasia. Hypereosinophilic syndrome has been reported to be associated with hepatic dysfunction; liver histology is mainly characterized by a diffuse eosinophilic inflammatory infiltrate. A 49-yr-old woman, diagnosed as a case of idiopathic hypereosinophilic syndrome with bone marrow and pulmonary eosinophilic infiltrates associated with peripheral eosinophilia,high IgE level developed features of chronic gatrornteritis, hepatitis, with a significant eosinophil component. She responded well to systemic glucocorticoid and Imatinib therapy with normalization of liver function tests within a few weeks.

Role of Biomarkers in the Stratification of COVID-19 Disease Severity – A Review

Background: There have been a wide variety of clinical publications on coronavirus disease 19 (COVID-19) focused on specific biomarkers. Acute-phase reactants, such as C-reactive protein (CRP), ferritin, serum amyloid A (SAA), and procalcitonin, have been identified as sensitive markers of acute COVID-19 illness, even though they are nonspecific markers. Objective: The purpose of this study is to summarize the role of several biomarkers in the stratification of COVID-19 disease severity. Methods: This study followed systematic literature review method. The systematic review followed the review process as it was well developed and planned to reduce biases and eliminate irrelevant and low-quality studies. The steps for implementing a systematic review include correctly formulating the COVID-19 question to answer, developing a protocol based on inclusion and exclusion criteria, performing a detailed and broad literature search and screening the abstracts of the studies identified in the search and subsequently of the selected complete texts. After selecting the study, the next steps were synthesis of the evidence like extract the necessary data into a form designed in the protocol to summarise the included studies, assess the biases of each study, identifying the quality of the available evidence, and develop tables and text that synthesise the evidence. The secondary sources of data for this study included different published topics from national & international journals. Good number of Journal articles was taken regarding “Role of Biomarkers in the Stratification of COVID-19”. Published articles were collected from renowned indexing data source like PubMed, Medline, and Scopus. Etc. Conclusion: Significantly increased white blood cell count, lymphopenia, decreased CD3, CD4, or CD8 T-lymphocyte counts, high neutrophil count, thrombocytopenia, and dramatically elevated inflammatory biomarkers were all linked to severe disease and the probability of developing sepsis as the disease progressed. Progressive decreases with lymphopenia, thrombocytopenia, elevated CRP, procalcitonin, increased liver enzymes, impaired renal function, and coagulation derangements were more common in critically sick patients and were linked to a higher rate of clinical sequelae. In seriously and critically ill patients, elevated interleukin levels and significantly increased SAA were most frequently reported. The neutrophil to lymphocyte ratio, the systemic immune inflammation index, and the COVID-19 Severity Score are all indicators of systemic inflammation that can be used to predict disease severity, outcome, and death.

Serum ferritin level of transfusion dependent thalassaemia patients- A Single Centre Study

The aim of the study was to determine serum ferritin level in transfusion dependent thalassaemia patients. A total of 64 transfusion dependent thalassaemia (TDT) patients was included in this cross sectional study from April, 2018 to September, 2019 according to selection criteria. Body iron load was estimated by serum ferritin level. The study sample consisted of 43 male and 21 female TDT patients, with a mean age of 25.5 years. Most of the patients (43.8%) patients had serum ferritin level in between 1000-2500 ng/ml. 12 patients (18.7%) had serum ferritin level >5000 ng/ml and 3 patients (4.7%) had serum ferritin in between 2501-5000 ng/ml. The mean serum ferritin was found 2462.6 ± 2792.7ng/ml with range from 207.0 -11891.2ng/m.

Chronic Myeloid Leukaemia Presented with Extreme Isolated Thrombocytosis

CML presenting with isolated extreme thrombocytosis is rare. We reported a 47 years old man who presented with history of right sided lower abdominal pain, vomiting, significant lethargy and chest tightness. Patient was mildly anaemic and abdominal examination revealed no organomegaly. On investigation, he was found to have extreme thrombocytosis (2050x109/L) and mild leucocytosis (31.7 x109/L) with mild anaemia. In view of extreme thrombocytosis, he was investigated for myeloproliferative disease especially essential thrombocythemia. He was found to be positive for BCR-ABL by reverse transcription PCR (RT-PCR) and negative for JAK2, CALR, MPL mutations. Ultimately, he was diagnosed as a case of CML with an atypical presentation. He received imatinib 400 mg/day and achieve complete haematological response at 15 days.

Outcome of Diffuse Large B-Cell Lymphoma with First-line Chemotherapy

Background: Diffuse Large B-Cell Lymphoma (DLBCL), most common Non-Hodgkin Lymphoma (NHL) variety, is an aggressive, fast-growing form comprising up to 40% of all cases globally. Objective: To observe the treatment outcome of different subtypes of Diffuse Large B-Cell Lymphoma (DLBCL) after first-line chemotherapy and also the association with IHC, presenting age, sex, and IPI score with outcome. Methodology: This is a retrospective data analysis included all DLBCL patients registered in the department of Haematology of National Institute of Cancer Research and Hospital (NICRH) between July 2016 to June 2019. Results: Total 188 cases were included in this study and mean age was 48 years with a Standard deviation of 15 years with Male (69.1%) predominance. We divide the cases into three different entities of DLBCL [Germinal Centre B-cell like (GCB), Non-GCB and others (NOS) among them Non-GCB variety was the prevalent (47.3%) one. After first line chemotherapy 52.1% complete remission with 7% death was observed in overall outcome. There was no significant difference in outcome among different types of DLBCL after chemotherapy based on Han’s algorithm. Rituximab with CHOP has significantly better outcome than CHOP alone arm (p: 0.021). Conclusion: This limited database study of NICRH will help to ascertain the outcome of DLBCL after first-line chemotherapy in Bangladesh.

A Cas Report of Diamond-Blackfan Anaemia with RPS19 Mutation

Diamond Blackfan Anaemia (DBA) is a rare disorder which presents with anaemia in early childhood. This heterogenous disorder is mainly autosomal dominantly inherited. Significantproportions of the cases are associated with craniofacial anomalies and some cases may end up developing malignancy. The diagnosis is established by blood investigations, and bone marrow studies in which red cell precursors are reduced or absent. Screening for the mutations including those encoding for ribosomal proteins in the patient and the family members is confirmatory for diagnosis. Human Leukocyte Antigen (HLA) matched hemopoietic stem cell transplantation is the definitive treatment of choice. In other cases, corticosteroids have been tried. The haemoglobin level is maintained with packed red cell transfusion. We are presenting here a male baby who had anaemia soon after birth and was brought to us at the age of 1 year 3 months. The diagnosis of DBA was made since the patient presented with anaemia and supportive biochemical and histological evidence. Genetic screening revealed mutation in ribosomal protein S19 (RPS19) gene in the baby.

Pattern of Haematological Malignant Patients Attending OPD in a Tertiary Level Hospital in A Calendar Year

Background: Hematologic malignancies are of diverse incidence, prognosis, and etiology. Dhaka medical college hospital (DMCH) is a tertiary level hospital and dealing with patients attending from all over the country. We have analyzed data of patients present with hematological malignancy in our Out Patient Department (OPD). As Hematology Dept DMCH deal with patients referred from all over the country, its OPD data represents the picture of whole country. Methods: This is a retro-spective study of OPD patients with hematological malignancy in the department of Hematology of DMCH from January 2018 to December 2018. The diseases were diagnosed on the basis of bone marrow morphology or histopathology (Lymphoma) and or immunophenotyping & molecular genetics. Results: Total 5747 patients were treated in 2018 with the complaints related to hematological malignancy of which 4599 (80%) were old patients and 1148 (20%) were new patients. Among all the treated patients 1420 (24.7%) were female and 4327 (75.3%) were male. Among 1148 new cases 37 were AL(3%), 386 were ALL (34%), 265 were AML(23%), 32 were CLL (3%), 126 were CML (11%), 40 were HL (3%), 18 were MDS (1%), 76 were MM (7%), 168 were NHL (15%). Data shows variation in total number of new patients per month- 83 in January (7.23%), 96 in February (8.36%), 140 in March (12.20%), 98 in April (8.54%), 94 in May (8.19), 89 in June (7.75%), 98 in July (8.54%), 66 in August (5.75%), 99 in September (8.62%), 103 in October (8.97%), 98 in November (8.54%), 84 in December (7.31%) But it does not show any statistical significant frequencies of diseases according to months. Conclusion: We deal a lot of patients with hematological malignancy each year in DMCH. A good portion of them are of ALL because of monthly maintenance of ALL protocols. Analysis of new cases shows ALL, AML, NHL is most common three diseases encounter in OPD. OPD patients’ inflow does not show any significant monthly variation for any of the diseases.