Differential WNT gene expression in various subtypes of acute lymphoblastic leukemia

Dysregulation of WNT signaling has been reported in many malignancies. This study was conducted to investigate the expression pattern of 14 members of the WNT gene family in different immunophenotypic subtypes of ALL. Semi-quantitative RT-PCR was performed on samples from 71 ALL patients and 36 age-matched healthy individuals. The ALL patients were categorized into B-ALL [76%], T-ALL [22.6%] and mixed lineage [1.4%] and the B-ALL cases were further classified into pro-B, pre-BI, pre-BII and immature/mature-B based on immunophenotypic results. Among the WNT genes, WNT-7B [p=0.026], WNT-9A [p=0.020] and WNT-16B [p=0.023] were significantly over-expressed, whereas WNT-2B [p=0.033], WNT-5A [p=0.016], WNT-7A [p<0.0001] and WNT-10A [p<0.0001] were down-regulated in B-ALL. Among the T-ALL subtype, however, significant down-regulation of WNT-2B, WNT-5B, WNT-7A, WNT-10A and WNT-11 was evident. Comparison between B-ALL subtypes showed significant over-expression of WNT-7B, WNT-9A and WNT-5B in certain subtypes. Our results suggest contribution of the WNT genes in leukemogenesis of ALL

[Evaluation of MYCN amplification in Iranian patients with neuroblastoma by conventional and real time PCR]

Background: There is an international consensus that evaluation of MYCNamplification should be done in all cases of newly diagnosed neuroblastoma. MYCN is the most important prognostic factor for neuroblastoma and determines treatment strategy

Materials and methods: In this study we evaluated paraffin embedded tissue block and bone marrow aspiration of 75 neuroblastoma patients with mean age of 4.1 years, including 32 female and 43 male, by conventional and also real time quantitative PCR

Results: Forty eight and 43 percent were MYCN amplification positive by Real time and conventional PCR, respectively. 28% of patients less than one year old and 48% of patients older than one year showed MYCN amplification.50 percent of cases with pathological diagnosis of small round cell tumor had MYCN amplification

Conclusion: PCR is a fast, reliable and cost effective method for the evaluation of MYCN amplification and can be performed using DNA extracted from small tissue samples and paraffin embedded blocks. As expected regarding detection power and convenience, Real time PCR was superior to conventional PCR

[Effects of L-asparginase administration on anticoagulant proteins and platelet function in patients with acute lymphoblastic leukemia]

Acute lymphoblastic leukemia is one the most common malignancies in children and adolescents. L-asparginase [L-ASP] is one of the leading medications in treatment of ALL. L.ASP interferes with the synthesis of some coagulation proteins and therefore causing disturbance in normal coagulation. In this study, the effects of L-ASP on anticoagulant proteins [protein C, protein S, and antithrombin III] and platelet function were assessed. This was a before-after study on 41 patients with ALL who refered to Mahak hospital [Tehran, Iran]. Before and after the injection of L.ASP, a bleeding time test was performed based on Ivy method. Protein C and protein S performance was assessed by turbidometry and antithrombin III performance was evaluated by chromogenic method. 48.8% of patients were female. Mean [ +/- SD] of age was 4.0 +/- 7.2. A significant reduction in the mean amount of protein C, antithrombin III and bleeding time was recorded. However, the reduction in protein S was not significant. No patient showed the symptoms of thrombosis. The results of this study showed that L. ASP drug reduced coagulation proteins [except the protein S]. This decrease along with other concomitant genetic factors can lead to thrombosis in some patients with ALL during induction therapy

role of periocular carboplatin in treatment of advanced intraocular retinoblastoma

To evaluate the results of combined chemotherapy and periocular carboplatin injection to control advanced intraocular retinoblastoma [Rb]. In this prospective interventional case-series, we included 8 eyes of 8 patients with advanced intraocular Rb [group C or D in International Intraocular Retinoblastoma Classification, IIRC]. In these patients, periocular carboplatin was injected as a primary adjuvant therapy combined with chemotherapy [VEC regimen] or as a secondary treatment in patients who had recurrent disease or did not respond to primary chemotherapy and local modalities. All patients were examined under anesthesia [EUA] and fundus photography was done by Ret-cam before treatment. Fifteen miligrams of carboplatin was injected into subconjunctival or subtenon space in 3-4 weeks interval. Eight eyes of eight patients were enrolled. Five patients were male and three patients were female. Mean age of patients at the time of treatment was 34 months and the mean duration between initial presentation of Rb and beginning of treatment was 7.5 months. Mean injection of periocular carboplatin in each eye was 3.1 times. Past medical history in our patients before starting of this treatment included: External Beam Radiotherapy [EBRT] in 3 eyes, cryotherapy in 4 eyes, transpupillary thermotherapy [TTT] in 4 eyes and brachytherapy in 2 eyes. Four eyes were in group C and four eyes were in group D. In 3 patients carboplatin was injected as primary treatment and in five patients it was injected for tumor recurrence following initial treatment failure or relapse. Three patients had vitreous, one had sub-retinal and remaining four had both vitreous and sub-retinal seeding. The degree of seeding was low in two patients, moderate in two patients and severe in four patients. At the end of study, tumor was regressed in six eyes [75%], relapsed in one [12.5%] and recurred in another [12.5%]. In both eyes with relapsed and recurrent tumors, enucleation was done. Combined chemotherapy and periocular carboplatin injection as well as focal treatment may be an effective method to control advanced intraocular Rb

Subtenon carboplatin in the management of intraocular retinoblastoma

To evaluate the efficacy of subtenon carboplatin as an adjuvant to systemic chemotherapy in the management of intraocular retinoblastoma. This study was conducted as a randomized, double-masked clinical trial. A diagnosis of intraocular retinoblastoma was made based on clinical examination, ultrasonography and orbital CT-scanning. The greatest basal dimension of the tumors was estimated in disc diameter [DD] by indirect ophthalmoscopy. Tumor thickness was determined by ultrasonography. Each eye was assigned to one of 10 blocks based on tumor stage [Reese-Ellseworth classification] and randomly received systemic chemotherapy alone [control group] or systemic chemotherapy plus 20mg subtenon carboplatin [case group]. Indirect laser photocoagulation or cryotherapy was performed as additional treatment. The study included 35 tumors in 17 eyes of 14 patients [19 tumors in 8 eyes of the control group and 16 tumors in 9 eyes of the case group]. There was 57.22% and 61.73% decrease in tumor thickness in the control and case groups, respectively [P=0.12]. The decrease in greatest basal tumor dimension in the control group [47.32%] was not significantly different from that in the case group [38.80%]. One eye [12.5%] in the control group and 3 eyes [33.3%] in the case group were enucleated. Adjuvant subtenon carboplatin does not seem to increase the efficacy of systemic chemotherapy in the treatment of intraocular retinoblastoma