Objective: It is well known that increased oxidative stress leads to tissue damage in beta-thalassemia (β-thal) patients. Thiols are one of the most important antioxidant agents, and thiol/disulfide (SH/SS) homeostasis is a novel oxidative stress marker. This study aimed to investigate the relationship of thiol levels, SH/SS homeostasis, and ischemia-modified albumin (IMA) in patients with β-thal. Materials and Methods: A hundred transfusion-dependent β-thal patients and 41 healthy controls were included in the study. Results: Native thiol, total thiol, disulfide, catalase, and IMA levels were significantly higher in the β-thal group compared with the control group (P<0.02). There were no correlation between serum ferritin level and SH/SS homeostasis, and weak positive correlations were found between serum ferritin and IMA (r=0.242, P=0.022). Conclusions: Our study results suggest that antioxidant systems try to compensate for peroxidative damage in the patients’ group and serum IMA level was found increased because of increased oxidative status. To the best of our knowledge, there has been no report evaluating plasma dynamic SH/SS homeostasis in β-thal patients.

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Hydrothorax of the cerebrospinal fluid after a subarachnoid-pleural fistula is a rare condition. Subarachnoid-pleural fistula may appear after a trauma at the thoracolumbar vertebral column or iatrogenically after thoracotomy. A two years and four months old boy who was operated because of ganglioneuroblastoma was admitted to hospital due to respiratory distress. The chest roentgenogram obtained two months after thoracotomy, showed a pleural effusion at the left side and a chest tube was inserted. The craniospinal magnetic resonance imagining revealed subarachnoid-pleural fistula and lumbar external cerebrospinal fluid drainage was performed. The chest tube was removed by application of tetracycline between pleural layers. After the patient was discharged, respiratory distress reoccurred after 3 weeks and a chest tube was reinserted due to fluid at the left hemithorax. An external lumbar drainage was reapplied. The location of the fistula was determined by craniospinal magnetic resonance imagining and myelography. The fistula was surgically restored by a posterior approach and laminectomy. The cerebrospinal fluid drainage and chest tube was removed three days and seven days after the operation respectively. The patient was discharged at the 13 days after the operation. During periodical outpatient follow up the patient has no symptoms and is neurologically intact. Subarachnoid-pleural fistulas, usually do not regress spontaneously or respond to conservative methods. Invasive approaches including surgery may be needed to treat patients with subarachnoid-pleural fistulae.

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Objective: Hepatic hemangiomas (HH) are the most common vascular tumors of the liver. It is important to distinguish hemangiomas from malignant liver tumors. Materials and Methods: The patients 0 to 1 years old, were diagnosed with HH and followed up in the oncology outpatient clinic between 2009 and 2020 were included in the study. Results: A total of 127 patients with the diagnosis of HH were included in the study. Of the patients, 99 (78%) had focal, 20 (15.7%) had multifocal, and 8 (6.3%) had diffuse HHs. Surgery was performed and the diagnosis was confirmed histopathologically in 6 patients (4.7%). During the follow-up, 16 (12.5%) patients received medical treatment. Thirteen (10.2%) were treated with propranolol, 2 (1.5%) with corticosteroids, and 1 (0.8%) with propranolol and corticosteroids. Complete response was obtained in 9 (9/16) patients and partial response was obtained in 6 (6/16) patients with medical treatment. Conclusion: Although HH is a benign tumor, it is important to make its differential diagnosis with malignant tumors of the liver. Over the years, the need for histopathologic examination for diagnosis has decreased. The success rate of propranolol is high, and the need for other treatment options with a high side-effect profile has decreased significantly since 2008.

Purpose: It has been well known that high serum immunoglobulin (Ig) E levels are associated with allergies, parasitic infections and some immune de ciencies; however, the potential effects and clinical implications of low IgE level on the human immune system are not well known. To determine the disorders accompanying very low IgE levels in children and adults.Methods: The patients whose IgE levels were determined between January 2015 and September 2020 were analyzed, and the ones with an IgE level <2.5 IU/mL were included in the study. Demographic data, immunoglobulin levels, auto-antibody results, and the diagnoses of the patients were noted from the electronic recording system of the hospital. Result: The IgE levels were measured in 34,809 patients (21,875 children, 12,934 adults), and 180 patients had IgE levels <2.5 IU/mL. Eighty patients were children (0.37%), 100 were adults (0.77%). There was a malignant disease in 45 (11 of them children) (25%), autoimmune diseases in 30 (4 of them children) (16.7%) and immunode ciency in 19 (16 of them children) (10.6%) of the patients. The most common reasons were other disases, immunode ciency and malignancy in children; and malignancy, autoimmune disorders and other diseases in the adults, in rank order. The IgE level did not show any correlation with the levels of other immunoglobulins. Conclusion: Although rare, a low IgE level has been shown to accompany malignancies, autoimmune disorders and immune de ciencies. Patients with very low IgE levels should be carefully monitored for systemic disorders.

Lenfomalar, lenfoid kökenli hücrelerin anormal çoğalması sonucu ortaya çıkan tümörlerdir. Hodgkin lenfomada nörolojik bulgular; sinir sistemine yayılım, kemoterapi ve radyoterapiye ikincil, kitle basısı sonucu, enfeksiyon ile ilişkili olarak ya da paraneoplastik sendrom şeklinde ortaya çıkabilir. Paraneoplastik nörolojik sendromlar Hodgkin ve Hodgkin-dışı lenfomalarda nadiren görülür. Hodgkin lenfomada görülen serebellar sendrom ve dermato-polimiyozit dışında diğer paraneoplastik nörolojik bulgular olgu bildirimi düzeyindedir. Bu yazıda Hodgkin lenfoma tanısı ile izlediğimiz, tedavi başlama aşamasında iken her iki alt ekstremitelerden başlayan güçsüzlük ve duyu kaybı gelişen, klinik bulgularına ve yapılan tetkiklerine dayanarak Guillain-Barré sendromunun bir alt tipi olan akut motor duyusal aksonal nöropati tanısı alan bir olgu sunulmuştur.

This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity.