---

Although rare, synovial sarcoma is the most common malignant non-rhabdomyosarcomatous soft tissue sarcoma in children and adolescents. Synovial sarcoma typically involves the soft tissues of the extremities, especially near large joints, but it can occur anywhere in the body in locations far from joint spaces. Although this tumor typically affects adults in their fourth decade of life, nearly half of the reported cases have been children and adolescents. We report a rare case of head and neck synovial sarcoma presented with tonsilar hemorrhage and painful facial contracture. Cervical computed tomography (CT) scan with contrast injection showed an asymmetrical respiratory tract image. Significant thickening of right (anterior) parapharyngeal soft tissue was revealed indicating a soft tissue mass. The mass was completely resected in a surgical procedure. In pathological examination of the mass, biphasic synovial sarcoma was reported. At first, we treated the patient with VIE (Vincristin, Ifosfamide, Etoposide),but because of the severe neutropenia and hemorrhagic cystitis, we changed the protocol to VAC (Vincristin, Actinomycine, Cyclophosphamide). The girl is 6 years old now and in a good condition without any pain or bleeding. Also, she has a normal cervical CT scan after more than two years. She is followed up every week and receives monthly Vincristin.

---

Background: Hydroxyurea (HU) is a well known chemotherapeutic agent that has been used largely for various myeloproliferative diseases over the past 20 years. In β-thalassemia, the effect of HU is much less clear and remains controversial. This study was undertaken to describe the hematologic and clinical responses of thalassemia major and intermediate patients to HU treatment during 2 years.

Materials and Methods: Seventy one major and twenty transfusion-dependent intermediate thalassemia participants were selected among 150 β-thalassemia patients. All patients underwent laboratory tests, and the state of energy, social activity, tolerance, and mood were recorded in the beginning of the study. Echocardiography was carried out before and during treatment with HU. All patients were treated with HU the initial dose was 10- 15mg/kg/day given once a day.

Results: All the patients tolerated HU well and showed a dramatic response to the drug. Nine of 20 intermediate and 8 of 71 major patients became completely transfusion free. In 6 intermediate and 15 major patients, transfusion interval prolonged more than 50%. After treatment, 95% of intermediate and 81% of major patients described an increase in social activity. HU therapy was also associated with a marked decrease in serum ferritin level in major thalassemia patients.

Conclusion: HU may be administered in thalassemia major and intermediate patients to minimize or obviate the need for regular transfusion and concomitant iron overload. HU therapy appears to be safe and effective when administrated in thalassemia patients.

---

Background: The advances in treatment regimes for thalassemic patients have increased the survival among them therefore osteoporosis has emerged as an important cause of morbidity. The aim of this study was to determine the prevalence of osteoporosis and osteopenia in patients with thalassemia from Zafar Adult Thalassemia Clinic, Tehran, Iran. Patients and Methods: In this cross sectional investigation, we studied 239 patients with β thalassemia major and 87 patients with thalassemia intermedia with a mean age of 29±8 years. All demographic data including age, weight, height, sex, age at diagnosis, age at blood transfusion initiation, chelating agent therapy and serum ferritin level were obtained from patients’ history. Bone mineral density of the lumbar spine (L1-L4) and femoral neck was determined using dual-energy X-ray absorptiometry (DEXA). Results: The prevalence of osteoporosis was 65.6% (214 out of 326 patients). Osteoporosis was present in 10.7%, 11% and 43.9% of patients in the lumbar spine alone (L1-L4), femoral neck alone and both places, respectively. In the rest of patients 18.7% showed osteopenia and only 15.7% were normal. Osteoporosis was more prevalent in patients with thalassemia intermedia compared to thalassemia major (p<0.001). Also higher age of patients, longer duration of transfusion and longer intervals between transfusions had a positive correlation with osteoporosis. Conclusion: The prevalence of osteoporosis among Iranian thalassemia patients is similar to prevalence reported elsewhere. Bone Mineral density is a good index of bone status in patients with thalassemia and recommended to be done for thalassemic patients annually. Key words: Thalassemia, bone mineral density, osteoporosis, DEXA.

---

Background: Red cell distribution width (RDW) is a routine laboratory measure that could be used as a predictor of mortality in critically ill patients. Identification of patients at risk for mortality early in the course of PICU admission is an important step in improving the outcome. We aimed to assess the use of RDW as an early biomarker for outcome in pediatric critical illnesses.
Methods: A retrospective study by extracting administrative and laboratory data from patients admitted to PICU of an academic pediatric teaching hospital was accomplished. After exclusion of 64 patients according to our exclusion criteria, 304 pediatric patients with PICU admissions over the 6 months of study period were included in the study. 
Results: The mean RDW for all patients was 14.9%±2.5%. PICU mortality was 13.3%. The rate of mortality in the quartile of RDW>15.7% was 20.1%. Elevated RDW was associated with longer duration of PICU admission (P<0.001). Tracheal intubation and ventilator support was needed in 34.2% of the patients. This was also correlated with elevated RDW (P=0.043). 
Conclusion: We observed that higher RDW was strongly linked to higher mortality risk in pediatric patients admitted in PICU. Higher RDW was associated with longer duration of PICU admission. 

---

The consequence of repeated blood transfusions in thalassemia is iron overload in different organs. Magnetic resonance imaging (MRI) is a reliable, non-invasive and accurate method for iron detection in various tissues, hence the introduction of MRI has revolutionized the management of these patients and improved the life expectancy of them. Cardiac MRI T2* has a profound effect not only on estimation of severity of cardiac siderosis, but on intensification of chelation regimens and survival of patients. Liver hemosiderosis is also a common morbidity among thalassemia patients, since the liver represents the dominant iron storage organ in the body; however, the relationship between total body iron and liver iron concentration (LIC) is challenging. Pancreatic iron overload occurs in 75-100% of patients with thalassemia major, but the association between pancreatic R2* and development of diabetes mellitus has not been established in the studies. On the other hand, there is a strong correlation between pancreatic R2* and cardiac R2*, so pancreatic R2* could predict left ventricular function. The most prevalent endocrinopathy in patients with thalassemia is hypogonadotropic hypogonadism which has been correlated with pituitary iron overload. Published data about kidney and adrenal MRI is limited, and further studies are needed to determine their clinical significance.

---

In the last few decades, the prognosis of patients with β-thalassemia has improved dramatically. However these patients suffer from many serious complications. One of the most important morbidities in patients with thalassemia are thromboembolic events that are mainly observed in thalassemia intermedia. Profound hemostatic changes have been observed in patients with thalassemia intermedia and numerous factors have been attributed to the pathogenesis of hypercoagulable state among patients with thalassemia intermedia. In this review the different mechanisms resulting in hypercoagulability in patients with thalassemia, the clinical importance, prevention and treatment of thromboembolic episodes have been discussed.

---

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.

---

Introduction: CLL is one of the most common leukemias, which is categorized by the accumulation of mature CD5⁺ B-lymphocytes in the peripheral blood, bone marrow, and secondary lymphoid organs. In this study, the status of rs6449182 polymorphism of the CD38 gene and its association with clinical and laboratory parameters of CLL patients was evaluated.
 Methods: Genomic DNA extraction was performed using the salting out method. The CD38 gene polymorphism (rs6449182) was studied in 70 patients with CLL and 70 healthy individuals using the PCR-RFLP method.
Results: The results of this study showed that the control group had 86% wild-type rs6449182 (CC), 12% heterozygous (CG), and 2% homozygous (GG) genotypes. In the case group, 62% had wild-type genotype (CC) 26% were heterozygous (CG), and 12% were homozygous (GG). Statistical analysis showed that the heterozygous genotype for the CD38 gene  was significantly associated with CLL. It was also understood that this polymorphism had a significant relationship with hemoglobin, age, and organomegaly of patients.
Conclusions: The CD38 gene polymorphism of rs6449182 SNP G allele had the highest frequency. Moreover, based on the results, this polymorphism has a significant relationship with organomegaly, which indicates the importance of these markers in the pathogenesis and prognosis of the disease.
Keywords: Polymorphism, Single Nucleotide Polymorphisms, Chronic lymphocytic leukemia (CLL), CD38
 

---

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.

---

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. 

---

---

---