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Treatment of severe Immune Thrombocytopenic purpura (ITP) accompanied by life-threatening bleeding events is challenging and a combination of treatment measures should be undertaken to rapidly increase the platelet count. Herein, we report a case of severe ITP in a seven-year-old boy who suffered from massive bleeding which was totally unresponsive to conventional therapeutic interventions. Since the patient was still symptomatic with severe thrombocytopenia after splenectomy, the patient underwent plasma exchange (PE) which was successful. To best of our knowledge this is the first case of severe ITP in children managed with plasma exchange due to unresponsiveness to standard treatments and splenectomy. Keywords: Immune mediated thrombocytopenia, Plasma exchange, Children, refractory

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Background: Considering the increasing number of patients with hemophilia and infrastructure requirements for a comprehensive approach, development of a recombinant factor has become a milestone. The objective of this study was to assess the safety, efficacy and non inferiority of Safacto (Recombinant factor VIII) compared with plasma-derived factor in the treatment of hemophilia A. Methods: 10 patients with severe hemophilia A were enrolled in this study. Each patient was treated by a 40-50 IU/kg infusion of either plasma derived or recombinant factor VIII after initiation of each of 4 consecutive hemarthrosis episodes in a triple-blind prospective crossover permuted block randomizing method. Clinical efficacy scale score and in vivo recovery of factor VIII was assessed in each of the treated bleeding episodes. Any adverse event was also recorded. Results: The mean±SD level of factor VIII in the plasma versus recombinant groups was 111.5±39 and 115±39, respectively without any significant difference. Response scaling method which assessed pain and range of motion revealed equalized scores along with in vivo recovery, hence treatment success rate was comparable in both groups. One non-recurring, mild skin rash reaction occurred simultaneous with the administration of plasma derived factor. Conclusion: Safacto (r-FVIII) is safe and effective and non-inferior to plasma derived factor VIII in the treatment of hemophilia A related bleeding events.

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Acute leukemia which is the most common cancer in children is a heterogeneous group of clonal malignancies. The conversion of the leukemic cell lineage during the course of the disease or later is termed lineage switch. It has been rarely reported in the literature. In leukemia lineage switch, conversions from lymphoblastic leukemia to myeloid leukemia or vice versa are reported. Herein, we report a 7-year-old child with acute lymphoblastic leukemia which switched to acute myeloid leukemia upon relapse.

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Background: Bleeding events in hemophilic patients with inhibitors are managed by bypassing agents. Currently available agents in Iran are recombinant activated factor VII (rfVIIa; Aryogen, Aryoseven) and Feiba (factor eight inhibitor bypassing agent). No standardized and accurate assay is currently available for monitoring the effectiveness of bypassing agents. We suggested that history of the patients’ response and also their preference could be a reliable method for assessing the efficacy of bypassing agents; therefore, we designed a multi-centric discrete choice experiment study to assess the factors that affect the efficacy of bypassing agents.
Methods: Hemophilic patients older than 2 years with inhibitors who required bypassing agents for the treatment of bleeding episodes were eligible to participate in the study. Patients’ preference toward treatment with either Feiba or Aryoseven was measured with a DCE (discrete choice experiment) design on a phone interview.
Results: 80 patients were enrolled from 5 centers in Iran. At enrollment, the mean age was18.6 years (range, 2-50 years). 47 patients (58%) preferred to receive FEIBA, 21 patients (21.2%) favored Aryoseven and 12 (14.8%) patients claimed no difference between the two products.
Conclusion: Our results indicated that according to the DCE method, patients preferred Feiba to Aryoseven while the main reason was their higher efficacy. In addition, adverse reactions in both groups were almost equal. As a result, it seems that presence of both products in the market for hemophilic patients with inhibitors is absolutely essential.

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Background: Glial fibrillaryacidic protein (GFAP), an intermediate filament protein, is mainly expressed by astrocytes, but some other cells like enteric glia and non-myelinating Schwann cells can also express GFAP. GFAP elevation has been reported in some types of meningioma and malignant brain tumors. In the present study, we analyzed the association between serum levels of GFAP with meningioma. 
Methods: Sixty-eight newly diagnosed patients with meningioma and 28 healthy individuals (control group) were included. Serum levels of GFAP were measured by ELISA.
Results: There was no significant difference in GFAP serum levels between the two groups. Subdivision of the patients also revealed no significant association between GFAP and meningioma. 
Conclusion: We studied serum levels of GFAP in meningioma in Iranian patients for the first time. We did not observe a significant association between meningioma and GFAP. A larger study including a larger number of different subtypes of meningioma patients may discover a weakly significant difference if it exists.

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Background: The Philadelphia chromosome (Ph) characterized by t (9; 22) (q34; q11.2) is a reciprocal translocation giving rise to a chimeric BCR-ABL fusion gene. Incidence of Ph chromosome is over 98% in Patients with Chronic Myeloid Leukemia (CML) and around 20% in acute lymphoblastic leukemia (ALL). The finding of this fusion gene is essential for diagnosis of CML by detection of various fusion transcripts such as b2a2 and b3a2 transcripts and Ph positive ALL by detection of e1a2 (p190) transcripts. We conducted this study to determine the frequency of various BCR-ABL fusion transcripts in the west Azerbaijani patients with CML.
Methods: RNA was isolated from peripheral blood samples by standard protocols. BCR-ABL fusion gene detection was carried out with one-step multiplex RT-PCR in 41 west Azerbaijani patients with CML.
Results: Among patients with CML, the frequencies of b2a2 and b3a2 transcripts were 52.5% and 12.5%, respectively. Co-expression of b3a2 and b2a2 transcripts was found in 12.5% of the patients. 
Conclusion: The findings of this study showed that multiplex RT-PCR is a suitable technique to identify the typical BCR-ABL fusion transcripts in the west Azerbaijani patients with CML. Atypical transcripts possibly run away while using multiplex PCR.

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Background: Oral Aprepitant, a neurokinin-1 receptor antagonist, is suggested in combination with other antiemetic agents in preventing chemotherapy-induced nausea and vomiting(CINV) associated with emetogenic chemotherapeutic regimens in adolescents, but its efficacy and safety in pediatric patients more than six months are unknown. in this study, we used abitant drug (a generic name of Aprepitant produced by Exir pharmaceutical company) for preventing CINV in children. 
Methods: In this triple-blind clinical trial, patients aged between 6 months to 15 years were randomly assigned to receive 3 mg/kg (maximum of 120 mg per dose) Abitant 60 minutes before receiving moderate to highly emetogenic chemotherapy and 2 mg/kg (maximum 80 mg per dose) in days 2 and 3 or placebo plus ondansetron. The primary efficacy endpoint was the percentage of patients who obtained complete response (stated as no retching, no vomiting, and no urge for rescue medication) during 25–120 hours (delayed phase) after initiation of emetogenic chemotherapy. The secondary endpoint was the proportion of children who attained complete response throughout the acute (0–24 h) and the total phase(0-120 h). Efficacy and safety analyses were done with randomly assigned patients who received at least one study treatment dose. 
Results: twelve patients of the Abitant group (66/7%) and 11 patients of the placebo group (64/7%) showed complete response (P=0.186). There was no significant adverse effect observed in both groups. Complete response on day 5 was 83.3% in the Abitant group and 86/7% in the placebo group. 
Conclusion: The efficacy of a combination of abitant along with ondansetron in controlling CINV was not inferior to the ondansetron alone in our pediatric population study group.

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Background: This study aimed to estimate the incidence of thyroid cancer and completeness of thyroid cancer case registration in Kerman province, Iran using three source capture-recapture approach during years 2008-2015.
Methods: All new cases of thyroid cancers reported during 2008-2015 which were registered by pathology, nuclear medicine and medical records were included in this study. Using the personal identifiers, common cases between sources were linked. The log-linear Poisson regression analysis was applied to estimate the number of unregistered cases. The incidence rate per 100,000 population was calculated. To study the trend, Joint-point regression analysis was performed. 
Results: Overall, 1153 new cases of thyroid cancer were reported by three sources. Most cases were female (81/7%). The mean age at diagnosis was 41.53±15.69 years. The estimated number of thyroid cancer cases was 1323. The estimated incidence per 100,000 population varied from 4.5 in 2011 to 11 in 2015. The female to male ratio varied from 3.1 in 2015 to 6.7 in 2011. The completeness of registration for all three sources varied from 33.7% in 2008 to 95.1% in 2013. The completeness of medical records, pathology reports, and nuclear medicine reports were 33.1%, 50.7%, and 66.2%, respectively.
Conclusion: Despite some improvements in completeness of thyroid cancer registration during the study period, there are still fluctuations within the study period. Furthermore, considering each source separately, the completeness of registration is not satisfactory. Therefore, more effort should be done to increase the completeness of thyroid cancer registration specially through the pathology source.

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Background: Educational evaluation is a broad concept that is related to all elements of the educational system. This concept is the result of the interaction of all values that are implemented with different titles and forms inside and outside the educational system to increase the performance of the educational system. The field of pediatric hematology and oncology is one that is constantly evolving due to extensive and numerous researches in various fields. These changes must be in line with changes in the health care delivery system. In this study, among the decision models, the CIPP model which is an evaluation model for curriculum evaluation given by Stufflebeam in 1983 which includes four elements: C- Context, I- Input, P- Process and P- Product., was selected to evaluate the educational curriculum of Iranian pediatric hematology and oncology fellowship.
Methods: The present study has two quantitative and qualitative aspects and a quantitative cross-sectional, descriptive-analytical study. This analysis was conducted in 2021 by the strategic group of the Iranian pediatric hematology and oncology association. Its statistical population consisted of members of this association. Most members have a degree in pediatric hematology and oncology. The research was conducted by census method. Data were collected using a researcher-made questionnaire. In general, the training course was examined in 4 areas of education and research, hardware facilities of the training and current environment, and professional abilities other than the content of the course. The efficacy of the evaluation questionnaire of the Pediatric hematology and oncology fellowship course was a combination of open and closed questions based on the “Kirk Patrick evaluation model”. This questionnaire had 20 questions. The internal evaluation based on Cronbach’s alpha was 0.92. The items surveyed in the questionnaire were: learning modern medical principles such as evidence-based medicine and clinical reasoning, learning the principles of medical ethics, study skills, understanding of legal procedures, ability to electronically research and adequacy of educational subjects. Satisfaction of the faculty members, students ‘satisfaction and the need for supplementary courses, the current method of evaluating students’ communication skills, the scientific ability of the eligible faculty in teaching current topics and participation in educating the students were among the other items of the questionnaire.
Results: In the internal validity study, Cronbach’s alpha coefficient of 0.92 was obtained for the current situation and 0.96 for the optimal situation. Descriptive statistics (mean and standard deviation) and one-group and independent t-test were used to analyze the data. Findings indicated that there was a significant difference between the current and desired status of free and absentee university exams in the areas of purpose, design, implementation, modification and feedback and the three components of each of these axes. According to the obtained results, changing the current educational curriculum of the subspecialty fellowship in pediatric blood and cancer is necessary and inevitable.
Conclusion: Corrective suggestions for writing a new curriculum in accordance with modern sciences and medical needs of the country were extracted and applied in the new curriculum.

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Cytomegalovirus (CMV) is the leading cause of viral-associated congenital infections. Moreover, it can also be acquired. Between 50 to 80 percent of the world’s population is seropositive for CMV and most clinical disease occurs in individuals previously infected with CMV. Rarely, serious CMV infection has occurred in individuals with healthy immune system. In contrast to immunocompetent patients, higher morbidity and mortality of CMV end organ disease is considered in immunocompromised patients. According to available evidence, gastrointestinal (GI) disease has lower prevalence in case of CMV-induced organ involvement, especially in pediatric non-transplant acute leukemia. In this report, we present a 12-year-old girl, known case of acute lymphoblastic leukemia (ALL) receiving maintenance chemotherapy with manifestations of gastroenteritis and significant weight loss. Initial laboratory data, demonstrated mild pancytopenia especially lymphopenia and thrombocytopenia. After excluding more common etiologies, colonoscopy with multiple biopsies were taken which was indicative of CMV-colitis. Intravenous (IV) ganciclovir for 3 weeks and oral valganciclovir for about 9 months were initiated. Follow-up courses for CMV surveillance included blood qualitative CMV polymerase chain reaction (PCR) and colonoscopy with biopsy which were negative for CMV but tissue qualitative CMV PCR was positive for CMV in about 7 months after initiation of treatment. Oral treatment was decided to be continued. To sum up, plenty of guidelines have been developed in stem cell transplantation and human immunodeficiency virus (HIV) patients but non-transplant leukemic setting, is a neglected area in the field of CMV infection management.

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Iron overload can adversely affect thyroid and parathyroid function in patients with transfusion-dependent thalassemia. Iron deposition in both glands or the pituitary gland, which controls thyroid function, can lead to their destruction and dysfunction. Hypothyroidism can cause symptoms such as fatigue, weight gain, and depression, while hypoparathyroidism can cause symptoms such as numbness and tingling in the hands and feet, muscle cramps, and seizures. Regular thyroid and parathyroid function monitoring is essential in thalassemia patients to detect any dysfunction early and provide appropriate treatment. Treatment may include medications to replace thyroid hormone or calcium and vitamin D supplements to manage hypoparathyroidism. A comprehensive approach to managing endocrine complications in thalassemia patients can improve outcomes and quality of life for these individuals. To provide professional healthcare members with clear and concise recommendations for diagnosing and treating hypothyroidism and hypoparathyroidism in transfusion dependent thalassemia patients, a practical national guideline should be developed.

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Thalassemia major hemoglobinopathy requires regular blood transfusions, often leading to iron overload due to repeated transfusions and increased intestinal iron absorption. The association between thalassemia major and metabolic complications, including diabetes and metabolic syndrome, has been recognized due to iron overload, insulin secretion impairment, insulin resistance, hepatic dysfunction, and other endocrine complications. These hormonal imbalances can also influence glucose metabolism and contribute to the development of metabolic syndrome. It's essential for individuals with thalassemia major to undergo regular monitoring of their glucose metabolism, including periodic assessments of fasting blood glucose, oral glucose tolerance tests, and measurement of Fructosamine. Early detection and management of diabetes and metabolic syndrome in thalassemia major patients are crucial to minimize complications and optimize overall health. Medical management may involve a combination of regular blood transfusions, iron chelation therapy to reduce iron overload, lifestyle modifications such as a healthy diet and physical activity, and, if needed, pharmacological interventions for glycemic control. Close collaboration between hematologists and endocrinologists is often necessary to provide comprehensive care for individuals with thalassemia major and metabolic complications. 

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