Iranian Journal of Blood and Cancer
Volume:8 Issue: 2, Jun 2016

Burkitt lymphoma is a common subtype of non-Hodgkin lymphoma in children. It has a rapid and aggressive clinical course with frequent involvement of bone marrow and central nervous system. Systemic chemotherapy is the mainstay of the treatment for this malignancy in children. In this systematic review, we discuss autologous and allogeneic hematopoietic stem cell transplantation (HSCT) and its indications in pediatric patients with Burkitt lymphoma.
The Medline (PubMed) database was searched using all keywords and phrases. The studies were identified by utilizing a combination of MeSH terms, such as Burkitt lymphoma, stem cell transplantation, autologous transplantation and allogeneic transplantation. Articles which were not published as full articles (conference proceedings excluded) were excluded. Relevant articles published during 2000-2015 were included.
13 articles met the inclusion criteria and were discussed.
Both autologous and allogeneic HSCT may improve survival in patients with BL. Autologous HSCT is mainly considered for patients with high-risk features of BL at presentation; however, allo-HSCT with non-myeloablative conditioning regimens are preferred for advanced stages and relapsed/refractory disease.

Tumor lysis syndrome (TLS) is a major metabolic complication in patients with malignancy after initiation of chemotherapy or spontaneously without treatment. The role of Rasburicase (a recombinant urate-oxidase enzyme) in prevention and treatment of TLS has been demonstrated in recent years. We aimed to investigate the efficacy of a single dose of rasburicase in reducing the risk of TLS in children at high risk.
we conducted a retrospective analysis of 560 children with various malignancies in a single referral center. On the basis of the reference values previously established in our center hyperuricemia and TLS were defined. Tumor lysis syndrome development was the primary outcome. 48 children with a mean age of 7.1 years (range: 3 months to 15.8 years) developed tumor lysis syndrome. The most common malignancies were B-precursor acute lymphoblastic leukemia (ALL) (45%) followed by non-Hodgkin lymphoma (NHL) and Wilms’ tumor (each 10.4%), respectively. They received normal saline intravenously at a rate of 4-5 L/m2/day in 24-48 hours prior to initiating chemotherapy. Plasma samples were drawn to detect uric acid, calcium, phosphate, potassium, creatinine and blood urea nitrogen (BUN) 4 hours before administering a single dose of IV rasburicase (0.2 mg/kg over 30 minute). Laboratory markers were evaluated again 4 and 24 hours after administering rasburicase.
All patients with diagnosis of TLS had significantly decreased uric acid levels following single dose of rasburicase except 1 patient (2.1%) (P Rasburicase is a safe and highly effective drug in children with hyperuricemia in the setting of malignancy at risk of developing TLS.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most known enzyme defects in Iran with various genetic mutations. We aimed to study the predisposing factors of hemolysis in children with G6PD deficiency.
This study was done during 2007-2012 in two referral centers of Mofid Children’s Hospital and Baqiyatallah Hospital, Tehran, Iran. The hospital records of the patients were fully reviewed and questionnaires for each patient were filled for the date of admission, initial symptoms, initial laboratory results, family history and history of any drug consumption, infection or fava bean ingestion.
Medical records of 192 children with mean age of 4.2 years (1 month to 14 years) were extracted. 68.2% of the cases were male. Hemolytic crises were significantly more common in spring which is the peak time for fava bean consumption and occurred more frequently in those with a family history of G6PD deficiency especially in females. The most common initial symptoms were jaundice (71%), dark color urine (49%), fever (34.4%), and pallor (24.5%), followed by abdominal pain (16.7%). Fava bean intake (93%) was the first etiological agent triggering hemolysis followed by infectious agents and drug consumption. Initial hemoglobin level was significantly lower in male patients.
Regarding the high prevalence of G6PD deficiency in Iran, we should emphasize on education of parents and physicians about the disease and prevention of fava bean ingestion in people with G6PD deficiency.

Bladder cancer accounts for about 7% of all cancers among adults in Iran. We aimed to evaluate epidemiological and clinicopathological features of bladder cancer in Kermanshah province, west Iran.
In a descriptive retrospective study, records of patients with bladder cancer referring to Imam Reza Hospital, Kermanshah city, Iran during 2011-2013 were analyzed. Sex, age, smoking habits, patients’ symptoms, histopathology and staging of the tumor were extracted from the records. The grading of the tumor was performed according to the Ash grading system.
220 patients were registered. 179 (81.4%) were patients men. The mean±SD age for patients at diagnosis was 62.5±15.1 years (range: 14-90 years). 71 (43%) patients were smokers. 165 (75%) patients were from rural areas. Hematuria, dysuria, and frequent or painful urination were reported in 74.5%, 5.5%, and 41.8%, respectively. Transitional cell carcinoma was the most common pathology in 93.2% of the patients. Grade І, ІІ, ІІІ and ІV tumors were observed in 0.5%, 41.5%, 37% and 21% of the patients, respectively.
Bladder cancer occurs most commonly in the elderly. Bladder cancer in Asia is more common in men compared with Europe and Africa. Since hematuria was the main presenting manifestation of the patients with bladder cancer, elderly patients with hematuria should be referred at the earliest convenience for investigation.

Acute lymphoblastic leukemia (ALL), the most common childhood cancer with a peak incidence in children from 2-5 years old, might be associated with poor prognosis and resistance to therapy in specific cytogenetic backgrounds. FoxO3a, a member of the forkhead class ‘O’ (FoxO) transcription factors, is a main downstream target of PI3K/AKT pathway which regulates different variety of biological processes and is overactivated in several human cancers. We aimed to evaluate the aberration of the FoxO3a gene in mRNA level in childhood ALL and compare them with healthy controls.
Real-time quantitative RT-PCR (qRT-PCR) was used to detect FOXO3a expression in 30 new cases of pediatric ALL and 30 age- and sex-matched healthy children as the control group.
the expression level of the FoxO3a gene was significantly lower in ALL patients compared to healthy controls (P Our study suggested that decreased FoxO3a expression may play an important role in the development of pediatric ALL. FoxO3a could be considered as a molecular target of therapy in ALL malignancy.

Allogeneic Hematopoietic Stem Cell Transplantation (allo-HSCT) is used as treatment of choice for patients with acute myeloid leukemia (AML). We aimed to evaluate the prognostic factors in 2-year overall survival of patients with non-M3 AML who underwent allogenic HSCT.
This is a Cross sectional retrospective study. Demographic data and study of variables such as age, sex, complete remission status, Karnofsky performance status scale at baseline and at time of transplantation, occurrence of GVHD (acute and chronic), relapse and 2-year survival were extracted from records of 49 patients who underwent allogenic HSCT from years 2006-2013 at BMT center in Imam Khomeini Hospital. All Autologous SCTs and M3 cases were not included. All data were analyzed with SPSS software. P The overall survival rate was 55% in the patients. There was no significant difference in overall survival between complete remission (CR) 1 and CR2. Relapse rate was 6%. Mean 2-year Karnofsky scale was 93.7. Mean admission time following BMT was 22 days which was significantly related to 2-year performance status (P=0.02). Admission complication rate was 22.5% (GVHD 12%). Rate of overall GVHD was 55%, 40% of whom developed chronic GVHD which had a positive effect on 2-year overall survival (OS2).The patient’s first performance state (K1) had a significant correlation with 2-year performance state (P Chronic GVHD and initial Karnofsky performance status scale can be considered as good prognostic factors in patients with AML who undergo allo-HSCT.

Congenital factor X deficiency is a rare autosomal recessive bleeding disorder that presents with variable bleeding tendency and prolonged coagulation tests, prothrombin time, and partial thromboplastin time. Thromboembolic events have not been reported in patients with factor X deficiency yet. Herein, we report a patient with factor X deficiency who had recurrent venous thromboembolic events.