Acquired hemophilia A (AHA) is a rare autoimmune condition characterized by spontaneous antibodies formation against coagulation factor VIII (FVIII). In the United Arab Emirates (UAE), the treatment paradigm of AHA varies, leading to heterogeneous patient outcomes. Because AHA cases often present to clinical practitioners with insufficient experience in the disease, diagnosis is sometimes delayed. This document aims to provide recommendations to clinical practitioners in the UAE to bridge the gap of heterogeneous practice in the clinical management of AHA. Structured literature searches in PubMed and Google Scholar with a three-stage Delphi method were conducted. Evidence suggests that prolonged activated partial thromboplastin time before surgery or coupled with acute or recent onset of bleeding is a hallmark of AHA. Treatment using bypassing agents is recommended in AHA patients with bleeding. Corticosteroids and rituximab with (out) a cytotoxic agent represent the first-line treatment in patients with FVIII: C <1 IU/dL. Second-line therapy involves agents not utilized in the first round of treatment. Thromboprophylaxis is recommended if FVIII: C has returned to normal levels. These recommendations are intended to improve the clinical practice and awareness of this disorder among hematologists and nonhematologists in the UAE and provide practical diagnosis and treatment advice.

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Coronavirus disease 19 (COVID-19) is a systematic multitropic disease. Sustaining blood homeostasis is a mission of multiple factors includes procoagulant and anticoagulant systems. Subsequently, maintaining a precise equilibrium between these antagonists' systems is crucial to prevent hemostasis. Frequently, during severe forms of COVID-19 patients, studies reported a discrepancy between the procoagulant and anticoagulant that usually results in fatal outcomes through multiorgan ischemia by thrombotic events. The proper interpreting of the anticoagulant status changes in COVID-19 patients is sufficient for effective and safe anticoagulant treatment. Our postulation is the first to address the pathophysiology of anticoagulant status under COVID-19.

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Hematopoietic progenitor cell transplant (HPCT) is found throughout the body. HPCT is a set of undifferentiated cells that are capable of indefinite self-renewal and generation of a functional progeny of highly specialized cells. Allogeneic HPCT presents a distinct set of challenges for blood banks and transfusion services. When considering transfusion for an HPCT recipient, one has to take into account not only the complexities associated with the patient's underlying condition, but also potential problems associated with recipient alloantibodies, donor passenger lymphocytes, and the conversion of blood group substance after successful allogeneic HPCT. For an allogeneic HPCT recipient, it is vital that the blood transfusion service keep a detailed record of the donor and recipient's that include donor blood group and antibody screening results and recipient's pre- and posttransplant blood group and antibody screening results. The management of nonidentical and incompatible HPCT requires a unique strategies when major, minor, and bidirectional differences exist which have the potentiality to improve patient outcomes and allow for effective management of the blood and blood products inventory.

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BACKGROUND: Hematopoietic Stem Cell Transplantation (HSCT) increases demand on Paediatric Intensive Care Units (PICU) and survival outcomes has emerged as an area of interest in HSCT research. AIMS AND OBJECTIVES: The aim of this retrospective case-control study is to estimate the incidence and survival outcomes, and identify variables associated with survival amongst HSCT patients: MATERIALS AND METHODS: Patients aged ≤15 years transplanted during January 2016 to December 2019 and requiring PICU admission(s). Statistical analysis involved calculating incidence of PICU admission, 2-year overall survival using Kaplan-Meier method; univariate and multivariate logistic regression analysis performed to identify factors associated with outcomes and P-value of <0.05 considered significant. RESULTS: Total of 130 patients underwent 150 HSCT, 31 (24%) of patients required 50 PICU admissions, discharge following PICU admission was observed in 36 (72%) admission episodes, while 14 (28%) associated with patient demise. Two- year Overall Survival (OS) amongst HSCT patients requiring PICU admission was 58% compared to 96.6% amongst patients not requiring PICU (P<0.001). subgroup analysis of cases with malignant disorders and non-malignant disorders showed similar results (P<0.001). Univariate analysis revealed reduced intensity conditioning, no engraftment at the time of PICU admission, and sepsis associated with decreased survival, however multivariate analysis revealed sepsis as the single independent prognostic factor for decreased survival. Our study reports 24% incidence of PICU admission amongst patients undergoing HSCT with a 28% mortality rate following PICU admission. CONCLUSION: Sepsis identified as the single prognostic factor associated with decreased survival. Therefore, efforts to optimize early identification and management of sepsis in the high-acuity setting of pediatric HSCT is warranted

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BACKGROUND: Anemia is one of the most common conditions in women during pregnancy. Fetal hemoglobin (HbF) levels are usually <1.0%. There are several genetic loci that have a significant influence on HbF levels. AIMS AND OBJECTIVES: The aim of this study is to determine the association of HbF level and DNA polymorphism at BCL11A rs1186868, HMIP rs9376090, and XmnI rs7482144 in anemic pregnant women. MATERIALS AND METHODS: Blood samples were collected from 164 anemic pregnant women at Obstetrics and Gynecology Clinic, Hospital Universiti Sains Malaysia. High-performance liquid chromatography was used to determine the HbF and HbA2 levels. RESULTS: Multiplex amplification-refractory mutation system-polymerase chain reaction (PCR) and gap-PCR were performed for 44 samples with a high HbA2 level (>3.2%) and normal HbA2 level (≤3.2%) to detect mutation at b-globin gene cluster. Allelic discrimination for rs1186868, rs9376090, and rs7482144 was performed using the real-time PCR technique for samples with no mutation detected. The mean age of patients was 31 ± 5.9 years. The HbF and Hb levels showed a strong positive correlation (r = 0.61). Out of 22 samples, 15 mutations at the b-globin gene and no mutation at the δβ-globin gene were detected. The mean difference of HbF level of patients with and without b-globin gene cluster was 1.07 ± 0.6 and 1.79 ± 3.5, respectively. The minor allele frequency (MAF) for the studied single nucleotide polymorphisms as follows: rs1186868 (MAF = 0%), rs9376090 (MAF = 19%), and rs7482144 (MAF = 32.8%). CONCLUSION: In conclusion, the baseline HbF levels were unaltered by BCL11A, HMIP, and XmnI genetic polymorphisms.

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BACKGROUND: Sickle cell disease and its variants result from an abnormal hemoglobin, hemoglobin S (HbS), caused by a single point mutation in the Beta-Globin gene. Hypoxia causes polymerization and distortion of Hb S containing red blood cells resulting in sickle crisis and hemolytic anemia. Common diagnostic methods include high-performance liquid chromatography (HPLC) and hemoglobin electrophoresis. The purpose of this research is to determine the frequency of sickle cell hemoglobin in HPLC samples and its geographical distribution in Pakistan. MATERIALS AND METHODS: Data was collected from the samples received from 1^st February 2020 to 31^st January 2021. Proforma included demography, complete blood count parameters, and variants of sickle cell disease which were extracted from hospital records. Analysis was done using the SPSS (version 26). RESULTS: Out of 14,740 samples, 295 (2%) revealed HbS. These patients had a mean age of 14.2 years. The male-to-female ratio was 1.5:1. The samples were received from Baluchistan (43%), followed by Sindh (32.1%), Khyber Pakhtunkhwa (16%), and Punjab (8.9%). Mean hemoglobin was 8.6 ± 2.6 g/dl, lowest and highest were 2.4 g/dl and 16.9 g/dl, respectively. Sickle cell trait was found in 21.3% of the patients, homozygous HbSS in 27.7%, sickle beta thalassemia in 30.8%, 4.4% were compound heterozygotes for Hb S and Hb D, whereas 15.5% were posttransfusion samples. CONCLUSION: Our analysis showed that the highest frequency was of sickle beta thalassemia and other variants being low. This study also proved it to be more prevalent in Baluchistan with relatively high male preponderance.

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BACKGROUND AND AIM: Combining Hydroxycarbamide (hydroxyurea) therapy and L-Carnitine (HU+LC) was proved effective and tolerable in patients with thalassemia intermedia; however, its role in sickle cell anemia (SCA) was not investigated. This study aimed to assess various aspects of treatment with HU + LC in adult patients with severe forms of SCA. PATIENTS AND METHODS: Ninety-one adult SCA patients were recruited, then distributed into four treatment groups: Group 1 – 37 patients treated with HU + LC; Group 2 – 16 patients treated with HU; Group 3 – 18 patients treated with LC; and Group 4 – 20 patients received supportive treatment only. Their baseline data were recorded then reassessed 8–12 weeks later. Their compliance was assessed subjectively. RESULTS: Patients were nearly matched as regards gender, age, baseline hematologic, and biochemical data. Compared to baseline, HU + LC treatment significantly increased hemoglobin and reduced reticulocytes, white blood cells, mean corpuscular hemoglobin concentration, and lactate dehydrogenase (LDH). Compared with other lines, HU + LC significantly reduced number of painful episodes, blood transfusions, and in hospital days per year. Adding LC to HU improved patient compliance, with mild side effects. Residence, side effects, baseline hemoglobin (Hb), and Hb F% affected patients' compliance. Both gender and age did not show any influence on response to treatment while side effects, compliance, baseline Hb, and LDH displayed a significant effect. CONCLUSION: Combining LC with HU for the treatment of adult SCA patients showed superior hematologic and clinical effectiveness. The response was more obvious in severe cases. However, health education about the combination is needed to improve patients' compliance and therapeutic responses. Trial registration: Clinical Trials.gov, NCT05081349. https://clinicaltrials.gov/ct2/show/NCT05081349.

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CONTEXT: Patient's sense of control over their disease management (patient activation) is reported to be associated with better disease outcomes in many chronic conditions. AIMS: The aim of this study is to assess patient activation levels among adult transfusion-dependent patients with hemoglobinopathies. The secondary aims were to determine whether the level of patient activation is associated with different disease outcomes and whether an association exists between higher levels of activation and specific patient characteristics. SETTINGS AND DESIGN: Participants were recruited from the day-care unit in the period between November 2019 and April 2021. Patients who agreed to participate were asked to provide demographic and socioeconomic data and complete the patient activation measure (PAM-10) in Arabic. Hospital records were reviewed for the retrieval of clinical data. RESULTS: The total number of participants was 84. The mean PAM score was 52.90 ± 10. Of all the participants, 21 (25%) had very low levels of activation (Level 1), 38 (45.2%) were categorized as Level 2, 21 (25%) were categorized as Level 3, while 4 (4.8%) were categorized as Level 4 (highest activation level). Disease outcomes were not different among patients by level of patient activation. CONCLUSIONS: Our results show that transfusion-dependent patients with hemoglobinopathies have low levels of activation. No association was found between patient activation and clinical disease outcomes.

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BACKGROUND: Thrombosis and acute respiratory failure are among the leading causes of mortality in COVID-19 patients. The von Willebrand factor (VWF) is one of the main components in the blood coagulation system. It has been hypothesized that the increased VWF level results from vascular damage because VWF is stored in endothelial cells. Infection of endothelial cells with SARS-CoV-2 can stimulate the release of VWF. This study aimed to determine the level of VWF activity among the Saudi population affected by COVID-19 and investigate the correlation between VWF level and D-dimer level. MATERIALS AND METHODS: This is an analytical, retrospective, observational study conducted in a single-center tertiary hospital at King Fahad Military Medical Complex (KFMMC). A study sample of 80 hospitalized patients (aged ≥18 years) diagnosed with COVID-19 confirmed by nasopharyngeal SARS-COV was randomly included in this study. Frozen blood samples were used to measure VWF antigen (Ag) level and activity for all patients. Historical data on hemostasis markers such as platelets (PLTS), fibrinogen, and D-dimer were obtained retrospectively, as well as demographic and clinical data. RESULTS: Data of 80 patients were analyzed to assess VWF Ag level and activity in correlation to D-dimer level. The mean age of the patients was 58.3 (standard deviation 16.18), with nearly two-thirds being females (62.6%). The most common comorbidities were arterial hypertension (67.5%), diabetes (56.3%), and dyslipidemia (22.5%). Only 10% of patients were obese. The mean values of were 3.25 for D-dimer, 3.55 for fibrinogen, 226.4 for PLTS, 9.88 for white blood cell, 237.04 for VWF Ag, 253.21 for VWF activity, and 70.45 for C-reactive protein. Pearson's correlation coefficient revealed that the correlation between D-dimer and VWF Ag and D-dimer and VWF activity was statistically insignificant (P > 0.05). Only six patients had overt thrombosis, but this has no significant correlation with VWF Ag, activity, D-dimer, or platelet count. VWF Ag and activity were higher in intensive care unit (ICU) than non-ICU patients. However, it is not significant statistically. VWF Ag and activity were significantly higher in deceased patients than in survivors. CONCLUSIONS: The results of this study showed that there was no significant correlation between VWF Ag and activity with D-dimer among Saudi patients with confirmed COVID-19.

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BACKGROUND: In T-acute lymphoblastic leukemia (T-ALL), multi-parametric flow cytometry can serve to detect minimal residual disease (MRD) by using immature or aberrant antigens expression as well as the altered expression of T-cell antigens. The latter approach has been specifically introduced to overcome the absence of leukemia-associated antigens. However, there is no agreed-upon method for the use of T-cell antigens in T-ALL MRD testing. AIMS AND OBJECTIVES: To compare the expression of classic T-cell antigens on T-lymphoblasts and T-lymphocytes to establish a protocol for their use in MRD analysis. MATERIALS AND METHODS: Flow cytometric data of PB or BM samples from 63 adults with T-ALL were collected. We assessed the frequency and degree of brightness or dimness of each T-cell marker, in addition to studying the uniformity of the events scatter of a total of 287 follow-up BM samples from 50 patients. RESULTS: Significant differences in expression intensity of T-cell markers were found between T-lymphoblasts and T-lymphocytes; they were reasonably stable on blasts in follow up samples. This detailed study has nominated the conjoint use sCD3neg/dim and CD5dim/neg in the identification of residual cells, to be supported by other T-cell markers. CONCLUSION: The suggested gating sequence showed an acceptable level of accuracy in detecting residual leukemia, supporting their use in T-ALL MRD especially when other distinguishing markers might be absent in the diagnosis sample, or susceptible to be lost with induction therapy.

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BACKGROUND: Many systematic reviews (SRs) and meta-analyses (MAs) have been published regarding the incidence of venous thromboembolism (VTE) in coronavirus disease 2019 (COVID-19) patients, which is acknowledged as high. Therefore, we aimed to assess the methodological quality of any SR/MA involving VTE and COVID-19. METHODS: This is a meta-epidemiological study evaluating the methodological quality of SR/MA. A structured search in PubMed and Web of Science was done for all SRs/MAs in VTE and COVID-19 from inception till April 29, 2021. We evaluated the methodological quality using A Measurement Tool to Assess SRs-2 (AMSTAR-2) checklist. RESULTS: We included 29 SRs/MAs where only one SR/MA was rated as high (3.44%), 12 (41.37%) were rated as moderate, and the rest were rated as low/critically low (55.17%). No statistical significance was observed in Spearman's correlation analysis between the rate of methodological quality and the number of authors, searching period, publication month, and publishing journal's impact factor. CONCLUSIONS: The number of published SRs/MAs in the VTE and COVID-19 subject is rising rapidly. The readers should pay attention to the low methodological quality of most published SRs/MAs.

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Hemophilia B is an X-linked inherited bleeding disorder caused by either the absence or reduced biosynthesis of clotting factor IX (FIX). This disorder affects approximately 1 in 30,000 male individuals worldwide. Patients with severe form (FIX <1%) account for 30%–45% of hemophilia B cases and usually report of spontaneous bleeds. Treatment includes FIX replacement prophylactically to prevent complications. However, the patient may develop inhibitors against FIX, which is rare and a serious complication, reported to occur in 1.5%–3% of hemophilia B patients. Immune tolerance induction is a therapeutic strategy to eliminate inhibitors. We report a 14-month-old-male child with severe hemophilia B on primary prophylaxis, presented with right knee swelling. He developed anaphylactic reaction while receiving recombinant FIX concentrate. Investigation revealed that FIX inhibitor titers were 1.0 Bethesda Units. He was managed with immune tolerance induction successfully.

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Congenital thrombocytopenia results from genetic mutations in genes implicated in megakaryocyte differentiation and/or platelet formation and clearance. We report the case of an 11-month-old girl who presented with severe macrothrombocytopenia since birth and subsequently developed an intracranial bleed. She was diagnosed to have GNE gene mutation. GNE gene encodes the key enzyme in sialic acid biosynthesis, glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE/MNK). Its mutation is responsible for the reduction in sialic acid biosynthesis and consequently leads to severe congenital thrombocytopenia and/or myopathy. Although no sign of myopathy is observed in our patient; it is possible myopathy can be developed later, thus long-term follow-up with neurology is highly advisable. We recommend the genetic counseling and a segregation analysis of this variant in other affected individuals in the family.

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BACKGROUND: This study aimed to determine the association of single-nucleotide polymorphisms (SNPs) of interleukin-6 (IL-6) gene with the survival and disease status of patients after allogeneic hematopoietic stem cell transplantation. MATERIALS AND METHODS: It was a prospective cohort study of total 102 participants, 51 patients along with their donors who had human leukocyte antigen-identical-matched allogeneic hematopoietic stem cell transplantation (aHSCT). Their pretransplant and posttransplant blood samples were collected for extraction of DNA for genotyping by sequence-specific primers polymerase chain reaction and gel electrophoresis followed by sequencing. The pre- and posttransplant levels of IL-6 were measured using enzyme-linked immunosorbent assay technique. RESULTS: The mean age of our patients was 19.83 ± 12.5 years having males 65 (63.7%) and females 37 (36.3%). The frequency of −174G/C SNP among acute graft-versus-host disease (aGVHD) group was GG = 53.8%, GC = 34.6%, and CC = 11.5%, and for −597G/A SNP, it was GG = 69.2%, GA = 15.4%, and AA = 15.4%. Our results showed that the presence of G allele in both homozygous and heterozygous forms was associated with increased aGVHD incidence, while the homozygous CC and AA mutant genotypes correlated with the lowest number of cases of aGVHD. The polymorphism −597G/A was significantly associated with the incidence of aGVHD with P = 0.04. The GG genotype in −174G/C and −597G/A was found to be significantly associated with aGVHD with P < 0.0001. High serum levels of IL-6 pre- and posttransplant were found to be significantly associated with the incidence of aGVHD with P < 0.0001. CONCLUSION: We concluded that the two SNPs − 174G/C and − 597G/A in the promoter region of IL-6 gene present either in donors or recipients predisposed to increase the risk of development of aGVHD following aHSCT. Their evaluation in our transplant setting may help in the risk stratification of transplant recipients early in time and predict the onset of aGVHD and mortality.

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Rare cases of plasma cell myeloma with morphological variants have been reported. Here we report a case of a plasma cell myeloma with a very unusual presentation diagnosed based on flow cytometry, immunohistochemistry, and cytogenetic studies.

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CONTEXT: Low-density lipoprotein cholesterol (LDL-C) is the classical target in cardiovascular (CV) disease management and is usually estimated by Friedewald's formula. However, this formula may over- or underestimate LDL-C levels. AIMS: Our aim is to compare eight LDL-C-estimating formulas to the direct LDL-C measurement and validate their use in the Saudi population. SETTINGS AND DESIGN: This was a retrospective study. SUBJECTS AND METHODS: A blood sample of fasting 669 Saudi subjects was tested for lipid profiles in King Abdulaziz University Hospital Laboratory, from which directly measured LDL-C was obtained. LDL-C was then estimated from eight different formulas: Friedewald's, Cordova's, Hata's, Puavilai's, Chen's, Ahmadi's, Hattori's, and Vujovic's, which were all compared to direct LDL-C. STATISTICAL ANALYSIS USED: Mean and standard deviation, paired t-test, and Pearson's correlation were used for statistical analysis. RESULTS: The mean differences between the direct LDL-C and Hattori and Chen's LDL-C were 0.03 and 0.08 mmol/L, respectively; P < 0.001, while the mean difference observed with Hata, Friedewald, Puavilai, and Vujovic's formulas were higher in comparison: 0.15, 0.24, 0.29, and 0.33 mmol/L, respectively, P < 0.001. Ahmadi and Cordova's LDL-C were estimated to be 0.60 and 0.64 mmol/L more than direct LDL-C levels, respectively, which showed the highest discordance with direct LDL-C, P < 0.001. At a triglyceride (TG) level of <4.5 mmol/L, Hattori also had the best agreement with direct LDL-C, with a mean difference of 0.04 mmol/L, and with TG >4.5 mmol/L, their mean difference was 0.21 mmol/L. All estimated LDL-C strongly correlated with direct LDL-C, except for Ahmadi's. CONCLUSIONS: Hattori's LDL-C had the best agreement with the direct LDL-C, and across all TG levels. However, we recommend directly measuring LDL-C in those with high CV risk.

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BACKGROUND: Determining the prognosis of Hodgkin's Lymphoma (HL) is crucial to avoid overtreatment and undertreatment, both of which have dangerous effects on the patient's health. This justifies the many studies to find prognostic factors, which in turn contribute to making the appropriate therapeutic decision. International Prognostic Score (IPS) and lymphocyte/monocyte ratio (LMR) are the most common prognostic indicator in HL, but they have some limitations in their application at some stages of the disease. The aim of this research was to study the efficiency of combining IPS and LMR to predict prognosis at both stages of the disease (early and advanced). METHODS: This study included 84 newly classical HL patients treated by ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) with radiotherapy. RESULTS: IPS showed a poor ability to predict 18-month progression-free survival (PFS) in both early-stage disease (P = 0.162) and advanced-stage disease (P = 0.191); LMR showed high performance in predicting 18-month PFS in early-stage patients treated with ABVD (HR = 5.456, confidence interval [CI] =1.04–28.715, P = 0.045). The combination of IPS and LMR was able to predict 18-month PFS in both early- and advanced-stage disease (HR = 12.21, CI = 1.24–120.1, P = 0.032) (HR = 2.84, CI = 1.04–5.04, P = 0.041), respectively. CONCLUSIONS: The combination of IPS and LMR defines two important risk groups: the high-risk group (IPS ≥3 and LMR <2.9) and the low-risk group (IPS <3 and LMR ≥2.9); it can therefore be used to predict prognosis and adapt treatment to suit each patient's condition.

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BACKGROUND: Langerhans cell histiocytosis is a rare disorder leading to destruction of tissues as well as causing lesions in the body. LCH can be self-limiting to disseminated life threatening. OBJECTIVES: We wanted to review data on presentation and outcome of our treatment efforts on patients with LCH diagnosed and treated at our institution. MATERIAL AND METHODS: In this retrospective study involving 75 pediatric patients with LCH diagnosed between 1998 and 2018, we evaluated clinicopathological features, laboratory findings, treatment modalities and outcome. RESULTS: In males median age at the time of diagnosis was 2.4 years. The median follow up was 63+/- 7.9 months. The most common presenting symptom was a bone lesion-related symptom. 57.3% were above 2 years of age at presentation while 38.7% below 2 years of age. 53% of cases had multisystem disease at diagnosis. Liver found to be most common organ involved. Treatment consisted of observation, surgery or chemotherapy. Vinblastine with steroid was the most common used chemotherapy regimen. 3 patients with progressive refractory disease underwent stem cell transplantation. CONCLUSION: Overall survival was significantly lower in patients with liver involvement.

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