Predicting Stage Progression in Binet Stage a Chronic Lymphocytic Leukemia.

INTRODUCTION: The variable clinical course of chronic lymphocytic leukemia (CLL) and the lack of consensus on follow-up and treatment strategies have necessitated a prognostic model for identifying high-risk patients at the time of diagnosis. METHODS: We involved a retrospective analysis of demographic and clinical characteristics of 212 patients diagnosed with Binet stage A CLL and thus eligible for risk stratification by both the International Prognostic Score for Early-stage CLL (IPS-E) and the alternative IPS-E (AIPS-E). We evaluated the applicability of these prognostic indices in our young, Middle Eastern cohort (median age 59 at diagnosis). RESULTS: During the study period with a median follow-up of 3.5 years, 67 patients (32 %) experienced progression to first treatment and cumulative incidence of treatment was 13 % at 1 year and 28 % at 3 years after diagnosis. Sixty-nine (51 % of the 136 with a known value) patients harbored an unmutated immunoglobulin heavy chain gene (IGHV) and 21 (10 %) an 11q or 17p deletion with 11 % lacking FISH results. For each early-stage CLL prognostic index, more patients were identified as high-risk for disease progression (51 % of 124 patients evaluable for IPS-E; 42 % of 109 patients evaluable for AIPS-E) than intermediate-risk and low-risk. Multivariable models involving the IPS-E and AIPS-E components revealed that unmutated IGHV and elevated absolute lymphocyte count were significant predictors of earlier treatment requirement. Both prognostic scores were discriminative of time to first treatment (log-rank p < 0.001; c-statistics of 0.74 for IPS-E and 0.69 for AIPS-E). CONCLUSION: Although clarity on clinical behavior with regard to initiation of treatment remains elusive, IPS-E and AIPS-E are valuable tools for identifying high-risk patients.

Epidemiology of de novo Acute Myeloid Leukemia in Kuwait per the 2016 WHO Classification.

OBJECTIVE: Acute myeloid leukemia (AML) is a hematological malignancy that arises from the clonal proliferation of immature myeloid cells. Although the number of AML cases has dramatically increased worldwide, information on its prevalence and incidence in Kuwait is lacking. This study reports the incidence of AML and patient demographics in the country from 2014 to 2020, based on the 2016 WHO classification of AML. SUBJECTS AND METHODS: Data on patients with AML, including acute promyelocytic leukemia (APL), were collected from a clinical cohort with 281 cases analyzed in this study. RESULTS: The overall median age of the population was 47 years with a 1.1:1 male-to-female ratio. Over the study period, the incidence of AML demonstrated a general increasing trend, with the highest and lowest overall incidence occurring in 2018 and 2015, respectively. The frequency of APL in our cohort was 8.9%. Regarding the 2017 European LeukemiaNet (ELN) risk stratification of patients with AML, 37%, 46%, and 17% of patients had a favorable, intermediate, and adverse risk, respectively. A total of 57% of cases achieved complete remission post-induction, and the median overall survival was 37 months. CONCLUSION: Our study may help predict the future trends of AML in Kuwait to help improve clinical management and patient outcomes.

Analysis of Common Driver Mutations in Philadelphia-Negative Myeloproliferative Neoplasms.

BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (MPNs) are a group of hematopoietic stem cell disorders that include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). This study examines the driver mutations among patients with MPNs in Kuwait. PATIENTS AND METHODS: This study was a retrospective review of 942 MPN cases with a driver mutation from July 2007 to June 2019 to examine their demographic, clinical, and laboratory attributes. RESULTS: The annual incidence of MPNs is 1.6 per 100,000 persons, and ET is the most common subtype. The median age of our cohort was 55 years, and the patients were predominantly male. We found that the most frequent gene mutation of MPNs in our cohort was the JAK2V617F mutation, which was present in 90% of cases, followed by the CALR exon 9, MPLW515L/K, and JAK2 exon 12 mutations. In our cohort, thrombotic events were observed in 18.7% of cases. CONCLUSION: Although Philadelphia-negative MPNs are rare hematologic malignancies, thrombosis is a relatively common initial presentation. The JAK2V617F mutation was the driver mutation in the majority of patients with MPN.

Philadelphia-negative myeloproliferative neoplasms among Kuwaiti Nationals.

The epidemiology, genetics, and thrombosis risk of MPNs among Arabs are largely unknown. This may be attributed to scarce epidemiological data, particularly from our region. Our study included 381 Kuwaiti nationals with Ph-negative MPNs and a confirmed driver mutation involving JAK2 (exon 12 14), CALR, or MPL. This first regional study examines the demographics, clinical parameters, and thrombosis-related attributes of the participants. This study reported a median age of 58 years, with females and males representing 54.9% and 45.1%, respectively. ET was the most frequent subtype of Ph-negative MPNs in our population, accounting for 52.0% of the cases, followed by PV, found in 34.6% of the participants, and PMF, found in 8.4% of participants. The crude annual cumulative incidence of Ph-negative MPNs in Kuwait ranged from 0.674 to 3.177 per 100,000 population across the study period. The most common driver mutation was JAK2V617F, with a frequency of 89.5%. At diagnosis, 19.2% of the patients presented with unexplained thrombosis, and almost half were of arterial origins. Males were more likely to present with arterial thrombosis than females (61.5% vs. 35.3%), whereas venous thrombotic events were more common in females than in males (47.1% vs. 17.9%; p-value = 0.025). Ph-negative MPNs in Kuwait are rare; however, thrombosis is a frequent complication, being documented in up to 19.2% of cases at presentation, more commonly at arterial sites. These findings call for thorough evaluation of patients with unexplained derangements in their hematological parameters during follow-ups.

Food advertisements on UK television popular with children: a content analysis in relation to dental health.

Objective To quantify the prevalence of advertising for foods and beverages potentially detrimental to dental health on UK television watched by children.Design Content analysis of pre-recorded television advertisements (adverts).Materials and methods Three hundred and fifty-two hours of television were recorded (one weekday and one weekend day, 6 am - 10 pm) from the main commercial channel (ITV1). All adverts were coded using pre-defined criteria.Setting UK television recorded between January and December 2012.Results Of 9,151 adverts, foods and beverages were the second most commonly advertised products (16.7%; n = 1,532). Nearly two-thirds of food adverts were for items that are potentially harmful to dental health (61%; n = 934). Of these, 96.6% were cariogenic and 11% were acidogenic foods. During peak children's viewing hours, the proportion of foods that are potentially harmful to dental health was significantly higher than for non-harmful foods (65.9% vs. 34.1%; p = 0.011). Adverts for foods potentially harmful to dental health were rare around children's programmes, but significantly more frequent during other programmes watched by children (p <0.001).Conclusion UK children are exposed to a particularly high proportion of advertisements for foods that are potentially detrimental to their dental health during their peak viewing hours and around the programmes they watch the most.

Nutritional rickets and osteomalacia in school children and adolescents.

OBJECTIVE: To review experiences of nutritional rickets and osteomalacia in school children and adolescents at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia. METHODS: Records of children and adolescents aged 6-18 years, seen at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia, during the period January 1994 through to December 1999, who were diagnosed to have rickets or osteomalacia were reviewed. The diagnosis was based on clinical, biochemical and radiological data. Data extracted and analyzed included age, sex, presenting symptoms and signs, dietary history and sun exposure, blood count, bone profiles, renal and liver profile, and 25-hydroxy vitamin D3 and 1, 25 dihydroxy vitamin D3. Hand and wrist x-rays were carried out for all patients while bone density of lumbar spine and 3 femoral sites and bone scan were performed on the majority of patients. RESULTS: Forty-two children and adolescents (25 females and 17 males) were diagnosed. Their age ranged between 6-18 years with a mean of 13.5. Non specific symptoms, such as bone pain and fatigue were the most presenting symptoms, while skeletal deformities and fractures were the presenting symptoms in only 5 and 3 patients. Lack of direct sun exposure and poor calcium intake was evident. Bone profiles at the time of diagnosis revealed mean serum calcium of 2.1 mmol/L, range 1.5 2.3 (Normal=2.2-2.7), phosphorus 1.1 mmol/L, range 0.7 1.9 (Normal=1.4 2.1) and alkaline phosphatase activities of 1,480 U/L, range 834-2,590 (N=<600). Serum concentrations of 25-hydroxy Vitamin D were low (<10 mg/L) while that of 1, 25 Dihydroxy Vitamin D varied between low to normal (<10-45 ng/L). Bone density of the lumbar spine and 3 femoral sites were performed in 26 patients and showed markedly reduced values, while bone scan demonstrated a high uptake of tracer throughout the skeleton "super scan". Multiple stress fractures were evident in 8 children. CONCLUSION: Although a community-based study to assess the magnitude of the problem is needed, it seems that rickets and osteomalacia of nutritional origin are not that uncommon and deserves special attention from all pediatricians and practicing physicians. They also suggested that further studies are needed to help understand the pathophysiology, and identify the contributing factors for the development of the disorder.

Juvenile rheumatoid arthritis: The Saudi Experience.

We retrospectively studied the charts of 115 Arab children with juvenile rheumatoid arthritis (JRA), all of whom satisfied the American College of Rheumatology (ACR) criteria for JRA. They were followed between 1978 and 1993 at King Faisal Specialist Hospital and Research Centre and King Khalid University Hospital in Riyadh, Saudi Arabia. All patients were followed up for at least 18 months. The female to male ratio was 1.2:1, and the mean age of onset of the disease was six years. Ninety of the patients were Saudis and the remainder were Middle Eastern Arabs. The mode of onset was systemic in 44%, polyarticular in 30%, and periarticular in 26%. Chronic uveitis was found in only two of the children (1.7%). Antinuclear antibody (ANA) was determined in 96 patients and was positive in 29 (30%). Amyloidosis was not detected in this study population. The clinical and laboratory manifestations of this disease are presented. This review shows that the spectrum of clinical presentation differs in Arab children from those in the West. Systemic and polyarticular onset subtypes were more common, and the incidence of uveitis and amyloidosis was lower. Whether this reflects a genuine difference in the pattern of the disease, or is due to bias in referral pattern, remains to be detected. The answer is currently being sought through an ongoing prospective study.

Down's syndrome in Saudi Arabia: incidence and cytogenetics.

A study has been carried out in Riyadh to determine the incidence and distribution of Down's syndrome births during a 9-year period from July 1982 to June 1991. Down's syndrome was ascertained in 42 (23 females and 19 males) of 23,261 consecutive babies born alive to Saudi women, giving an incidence of 1 in 554 live births (1.8 per 1,000). A trend towards an increased incidence of Down's syndrome with advanced maternal age or increased maternal parity was found. Cytogenetic studies were performed on 37 cases of which all but 1 were non-disjunction trisomy 21, while the remaining infant had a translocation. This study provides the first step for further epidemiological surveys of Down's syndrome in the Kingdom of Saudi Arabia in order to prepare the ground for an effective antenatal screening programme for chromosomal disorders.

Childhood brucellosis: a deceptive infectious disease.

Human brucellosis is a multisystem disease that may notoriously mimic many other illnesses leading to misdiagnosis and increased morbidity. Six pediatric cases of brucellosis who had no epidemiologic evidence of the infection escaped early or correct recognition. The diagnosis of brucellosis was later made on the basis of significant brucella serology and positive blood or bone marrow culture. In endemic areas, a high index of suspicion should prevail in the evaluation of patients with vague or unexplained symptoms.