Consequences of delayed diagnosis in treatment of retinoblastoma

One of the primary factors in managing patients with retinoblastoma is early diagnosis. The main idea of this study was to recognize the consequences of delay in diagnosis on therapy of the disease. A retrospective review of all children with proven retinoblastoma, who had presented to MAHAK hospital in Tehran, from April 2007 to Dec 2011, was performed. Grouping of intraocular tumors was applied as A to E according to International Classification of Retinoblastoma. There were 157 [91 boys] children eligible for study. The mean age was 1.21 +/- 0.11 years with average delay in diagnosis of 3.4 +/- 0.53 months. Classification of D group in both unilateral [93 patients] and bilateral tumors was the largest category. A significant relation [P=0.05] between delayed diagnosis time and tumor grouping was evident. The most frequent symptoms were leukocoria and strabismus. Age was significantly lower in the subgroup of bilateral tumors than in unilateral retinoblastomas [0.6 +/- 0.12 year vs 1.6 +/- 0.15 years]. The diagnosis was delayed in subgroup of extra ocular retinoblastoma more than in intraocular tumors [8.7 +/- 2.9 months vs 2.9 +/- 0.52 months]. The authors recommend early referring of suspected cases to ophthalmologists and pediatric oncologists and to organize educational programs to publisize signs and symptoms of the disease such as leukocoria, strabismus and ocular inflammatory disorders through national media. In conclusion, early diagnosis of retinoblastoma can be the primary factor in managing the patients as the delay in diagnosis accounts for highly advanced disease and poor prognosis

[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

[Survey of the relationship between of thrombophilic factors and the rate and severity of bleeding in patients with severe hemophilia A]

Hemophilia is the most common disorder of deficiencies in thrombotic factors. In most patients, severities of symptoms are in proportion of the seriousness of deficiency in the thrombotic factors. But in some patients with severe hemophilia, having less than 1% in factor level; the clinical symptoms are lower and slighter than the other hemophilic patients. Even in some cases, thrombotic events in the severe hemophilic patients have been accrued. The underlying causes of these findings are unknown. The aims of this study were to determine the role of some factors and also the type of genetic mutation of these factors in the rate and severity of bleeding and also the symptoms of the severe hemophilic patients. Sixty hemophilia A patients [FVIII<1%] with having records in Hemophilic Patients Center were divided on the basis of the received factor rate items per year, bleeding score, orthopedic score and radiologic score, in three groups with mild, moderate and sever clinical presentations [each group with 20 patients]. And then the mutation tests in the gene of Leiden V factor, PG20210A, MTHFR, level of thrombotic factors [II, V, VII, VIII, IX, X, XI, XII, XIII, and Fibrinogen], Protein C, S, Antithrombine III, Phospholipid Ab and Hemosisteine and number of platelets were performed. Data using the x2, Fischer, ANOVA and Tukey test and SPSS-14 were analyzed. Of the studied factors, the differences among 3 groups from view point of daily activity [P<0.002], antithrombine III [P<0.013], number of platelets [P<0.007], protein C [P<0.013] and level of factor XII [P<0.01] was significant. No significant differences were found in three groups in other tested factors. In this study, there were significant differences only in the daily activity, antithrombine III, number of platelets, protein C and level of factor XII. Thus, further studies are required to determine the role of other factors that may contribute to differences in the clinical presentations of the severe hemophilic patients

Immunophenotypic subtyping of leukemic cells from Iranian patients with acute lymphoblastic leukaemia: association to disease outcome

Immunophenotypic characterization of the leukemic cells has been widely used as a tool for diagnosis, classification, stratification and prognosis of leukaemia. To investigate the immunophenotypic subtype profiles of Iranian patients with acute lymphoblastic leukemia [ALL] and its association to disease outcome. In this study, a total of 60 Iranian patients with ALL were immunophenotyped by flow cytometry using a panel of monoclonal antibodies specific for CD2, CD3, CD5, CD10, CD13, CD14, CD19, CD20, CD33, CD34, CD45, HLA-DR and TdT molecules. The samples were initially categorized into T-ALL [n=9], B-ALL [n=50] and mixed lineage [n=1] based on the expression patterns of CD3 and CD19 molecules. B-ALL patients could further be classified into four subtypes, including Pro-B [n=7, 11.7%], Pre-B I [n=28, 46.7%], Pre-B II [n=13, 21.7%] and immature/mature B cells [n=2, 3.3%] on the basis of expression of CD10, CD19, CD20, HLA-DR and TdT. Clinical manifestations and laboratory findings of the patients did not reveal association with immunophenotypic sub-types of ALL, with the exception of mediastinal mass and WBC count at the time of diagnosis which were found to be significantly higher in patients with T-ALL compared with B-ALL [p=0.001 and 0.014], respectively. Our results indicate that overall the immunophenotypic profile of Iranian ALL patients is similar to previous reports and it might be used for monitoring of minimal residual disease and prognosis

Gonadal function and fertility in male survivors treated for Hodgkin's disease in Iran

To investigate the effect of chemotherapy on gonadal function of young men cured of childhood Hodgkin's disease. Young adult males surviving Hodgkin's disease, aged 17 and over at least 2 years after therapy were studied in Ali Asghar Children's Hospital, Tehran, Iran from March 2000 to March 2005. Clinical evaluation for secondary sexual characteristics, semen analysis, follicle stimulating hormone [FSH], luteinizing hormone [LH], and testosterone was studied in 33 survivors of Hodgkin's disease. The age at diagnosis was 5-15 years, median 9 years, age at study 17-29 years, median 19 years old. The median duration off therapy was 7 years [2-20 years]. All 33 patients received chemotherapy as follows: 32 patients received nitrogen mustard [mechlorethamine], vincristine [Oncovin], procarbazine, prednisone [MOPP] / doxorubicin [adriamycin], bleomycin, vinblastine, dacarbazine [ABVD] 6-8 cycles, 5 of whom after relapses received other protocols. One received only MOPP. Twenty-seven [81.8%] had azoospermia, 2 had severe oligospermia, 3 had oligospermia, and one had normal sperm count [58000,000]. All patients had normal secondary sexual characteristic. The FSH, and LH in 6/33 patients were above normal. Testosterone in 3/33 was below normal. A prepubertal status does not protect the gonads from the harmful effect of chemotherapy, and approximately 87% of male survivors of Hodgkin's disease develop azoospermia or severe oligospermia