Clinicopathologic features and therapy outcome in childhood Hodgkin's lymphoma: a report from tertiary care center in Saudi Arabia.

BACKGROUND: Hodgkin lymphoma (HL) is lymphoid neoplasm usually affecting lymphatic system; it accounts 3.6% of cancers in Saudi Arabia. Modern treatment protocols had shown particular success rates in overall-survival (OS) and event-free-survival (EFS). In our study, we reviewed the medical records of 80 pediatric and young adolescent patients diagnosed HL from January 2006 to July 2020, treated at tertiary care hospital in Riyadh, Saudi Arabia. Demographic, clinical, and pathological data were explored. First line therapy was ABVD, COG, COPP, R-CHOP, or radiotherapy alone in 53/80 (66.4%), 24/80 (30%), 1/80 (1.2%), 1/80 (1.2%), or 1/80 (1.2%) patients; respectively. Response assessment was done by CT + / - PET scan after first 2 cycles then every 2 cycle and end of therapy. Another assessment was done if any clinical suspicion of recurrence. RESULTS: Median age 11 (range 3-16) years. Males to females 1.3:1. Seventy-two out of eighty (90%) patients showed first complete remission (CR1) and maintained remission for median 40 (range 7-136) months. Eight out of eighty (10%) patients showed refractory disease. Nineteen patients received salvage therapy (ICE or ESHAP/brentuximab vedotin or gemcitabine/brentuximab vedotin), 14/19 (73.7%) had 2nd complete remission (CR2) for median time 24 (ranged 9-78) months, while 5/19 (26.3%) did not show any response. Five-year OS and EFS were 95% and 75%. Two patients had 2ry malignant neoplasms, one had AML and died, the other had malignant fibrous histocytoma and still alive. None of our patients had fertility problem. Also, they did not experience chronic pulmonary or cardiotoxicity. Classic Hodgkin's lymphoma: nodular sclerosis subtype was more prominent (55%) than mixed cellularity subtype (22.5%), which is similar to several European and US studies, lymphocyte rich (11.25%) and lymphocyte depleted (0%), while nodular lymphocyte predominant Hodgkin's lymphoma (11.25%). CONCLUSIONS: Our study provided unique descriptive study of childhood HL, in Saudi Arabia, with valuable insight into the long-term outcome and late toxicity. Our results are comparable to other studies in the Middle East and European countries.

Diagnostic accuracy of reticulocyte parameters on the sysmex XN 1000 for discriminating iron deficiency anaemia and thalassaemia in Saudi Arabia.

INTRODUCTION: Iron deficient erythropoiesis and Thalassaemia are both associated with microcytic erythropoiesis albeit from different pathological mechanisms. Given the high prevalence of Hemoglobinopathies in the Mediterranean region, discriminating these two conditions is important. Several algorithms using conventional red cell indices have been developed to facilitate diagnosis, however, their diagnostic accuracy is low. The new generation haematology analyzers enabled the use of more innovative parameters such as reticulocyte parameters. We aimed to evaluate the diagnostic performance of the reticulocyte parameters on the Sysmex XN 1000 to distinguish between IDA and Thalassemia in our population. METHODS: We performed a retrospective analysis of blood samples sent to our laboratory for haemoglobin electrophoresis screening. We categorized our cohort into Thalassemia and Iron Deficient patients based on known diagnostic criteria. We analyzed the reticulocyte parameters using receiver operator curve analysis (ROC) and determined the cut off value for each parameter. RESULTS: Reticulocyte parameters most accurate for discriminating IDA from Thalassemia patients was: RET, RET-HE and IRF. The RET-HE had the best statistical significance for IDA patients with AUC = 0.69 for cut off 22.25. The RET-HE for dual positive patients was more accurate with AUC = 0.78 for cut off 21.25. The IRF had the best statistical significance for Alpha Thalassemia with AUC = 0.66 for cut off value 18. CONCLUSION: An IRF cut off below 15.5 and RET-HE cut off below 22.25 was the most accurate variable in predicting IDA with a sensitivity of 59.4% and 68.3%.

The Impact of COVID-19 Pandemic in Children With Cancer: A Report From Saudi Arabia.

In January 2020, the WHO declared the novel coronavirus (2019-nCoV) outbreak as a public health emergency of international concern. Due to the rapid spread of 2019-nCoV, all countries started preventive and precautionary measures to prevent COVID-19 infection spread. These measures limited the population mobility and services provided, which subsequently Impact of on children with cancer and cancer care delivery in the many health centers in Saudi Arabia. We did a cross-sectional study to assess the impact of this outbreak on children with cancer concerning all aspects of life including medical services provided, the specific precautions to prevent spread in cancer patients, mental, psychological effects, and its effect on the quality of life. We collected 204 responses during a survey that assessed the impact on the treatment of cancer children at a tertiary institution during the COVID-19 pandemic. The majority of patients were receiving ongoing chemotherapy for leukemia/lymphoma. The majority of these patients (60.5%) reported a delay in treatment received due to hospital cancellation of appointments due to the pandemic. Although the majority of patients in our cohort complained of delayed treatment, fortunately, none of the delays led to fatalities. In the context of global lockdowns and physical distancing to help flatten the COVID-19 curve, telemedicine has proved fundamental to keeping patients and their healthcare providers connected and safe. Children also faced multiple other difficulties such as psychosocial issues during the COVID-19 pandemic. Our long-term goals are to develop new programs that will enable children with cancer to emerge successfully during a pandemic.

Rare cytogenetic abnormalities and their clinical relevance in pediatric acute leukemia of Saudi Arabian population.

BACKGROUND: Childhood Acute Leukemia (AL) is characterized by recurrent genetic aberrations in 60% of AML cases and 90% of ALL cases. Insufficient data exists of rare cytogenetic abnormalities in AL. Therefore, we tested rare cytogenetic abnormalities occurring in childhood AL and its effect on clinical prognosis in patients diagnosed at our institution from 2010 to 2017. RESULTS: Among 150 cases of AL, we detected 9 cases with rare chromosomal abnormalities. We found two hypodiploid (2n-) cases: 2n-,t (5;14)(q31;q32) and t (3;11;19)(q21;q23;q13.1) in ALL patients. AML patients showed t (7;14)(q22;q32), t (11;17)(p15;q21), t (11;20) (p15;q11), t (12;17)(q15;q23) and t (11;20)(p15;q11). Both t (1;15)(q10;q10) and t (17;19)(q21;p13.3) occurred in a case with biphenotypic AL. Complete remission (CR) status was attained in 3 patients and 6 patients never attained CR or relapsed/demised. CONCLUSION: The study highlighted that rare cytogenetic abnormalities are associated with a poor prognosis. This finding is not well reported in the literature suggesting that ongoing cytogenetic studies for rare abnormalities associated with pediatric leukaemia are warranted.

Routine Preoperative Coagulation Tests in Children Undergoing Elective Surgery or Invasive Procedures: Are They Still Necessary?

INTRODUCTION: Preoperative coagulation screening tests in pediatric patients was once routine clinical practice globally and still used as standard practice in some countries before surgical procedures to assess of perioperative bleeding risk. OBJECTIVE: The study aimed to evaluate unselected routine preoperative coagulation testing in children undergoing elective or invasive surgery to predict abnormal perioperative bleeding. The study also aimed to provide a rational approach of determining bleeding and family history of coagulation disorders as a predictive risk for bleeding. METHODS: This retrospective study conducted between 2014 and 2015 (1 year) on normal healthy children aged under 15 years admitted to the hospitals for elective mild to intermediate surgery or invasive procedures. We reviewed and collected the details of the clinical history, previous surgery, trauma, family history, detail of anti-thrombotic medication and coagulation tests performed (prothrombin time (PT), the activated partial prothrombin time (APTT), and international normalized ratio (INR)) at the time of admission. RESULTS: Among 2078 cases, 1940 cases had normal coagulation tests (93.4%), 77 cases had abnormal coagulation results (3.7%), and 61 patients underwent surgery without preoperative coagulation screening (2.9%). In 15 of 77 patients, coagulation tests were normal on repeat testing. A total of 52 were confirmed to have abnormal screening testing. Among these 52 cases, 45 had normal factors assay; where seven patients had abnormal factors assay. Postoperative bleeding occurred only in three cases (0.14%), two cases due to surgical procedures with normal preoperative testing and one due to hemophilia A which was detected postoperatively as no preoperative testing was performed. CONCLUSIONS: Routine coagulation screening before surgery or invasive procedures to predict perioperative bleeding in unselected patients is not recommended. Our study emphasizes that selective preoperative testing is more appropriate. Selective criteria for consideration of the latter includes physical examination, type of surgery, family and bleeding history, and concomitant use of antiplatelet and anti-thrombotic therapy.

Natural Killer Cell Lymphoblastic Leukaemia/Lymphoma: Case Report and Review of the Recent Literature.

Natural killer (NK) cell lymphoblastic leukaemia/lymphoma is a rare haemopoietic tumour currently defined in the 2008 WHO classification under the category of acute leukaemias of ambiguous lineage. A diagnosis of this type of leukaemia is considered in cases expressing CD56 along with immature T-cell-associated markers such as CD2 and CD7 with absence of B-cell and myeloid markers; in addition, blastic plasmacytoid dendritic cell leukaemia should be excluded. Prior to 2008, these precursor NK cell lymphoblastic leukaemias/lymphomas were categorized as myeloid/NK cell acute leukaemia with a phenotype identical to acute myeloid leukaemia with minimal differentiation. While the new classification has merit in having excluded myeloid expression, there is still persistent confusion in the literature and on a practical level with regard to precursor NK cell neoplasms. There is a paucity of recent case reports in the literature after the new WHO classification of this neoplasm. Due to the rarity of this neoplasm, an accurate pathological diagnosis is often difficult. In this article, we describe a case of precursor NK cell lymphoblastic leukaemia/lymphoma presenting with unique morphological features and conflicting immunophenotypes. We also review all case reports of this neoplasm after the WHO 2008 classification.

Bone marrow aspirate microscopy v. bone marrow trephine biopsy microscopy for detection of Mycobacterium tuberculosis infection.

BACKGROUND: Tuberculosis (TB) remains a global health problem. According to the 2013 Global Report on Tuberculosis, 8.6 million people developed TB in 2012 and 1.3 million died from the disease. An estimated 13% of people who developed TB in 2012 were HIV-positive, and 75% of these lived in Africa. While pulmonary TB is the commonest form of Mycobacterium tuberculosis infection, extrapulmonary TB is increasingly being detected in HIV-positive patients. Definitive diagnosis of disseminated TB is a challenge owing to atypical presentations and diagnostic difficulties (negative chest radiograph and sputum microscopy and culture). A rapid diagnosis of disseminated TB is desirable, as early initiation of treatment can reduce mortality. Although TB culture is the gold standard for diagnosis of TB, it has a long turnaround time (up to 6 weeks). OBJECTIVES: To identify a potentially faster and more effective diagnostic strategy for disseminated TB. METHODS: A retrospective 18-month review, conducted at a tertiary hospital, comparing histological findings of an auramine O-stained bone marrow aspiration (BMA) smear and a bone marrow trephine (BMT) biopsy specimen with the gold standard of TB culture. RESULTS: Microscopic examination of BMA smears and BMT biopsy specimens offers a rapid diagnostic strategy, with results available on the same day for the former and within 4 days for the latter. BMT histological examination had a significantly higher detection rate than BMA auramine O staining compared with TB culture. CONCLUSION: We recommend that BMT biopsies remain an essential part of the diagnostic work-up for disseminated TB.