Sirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study.

Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune-mediated destruction of haematopoietic cell lines. Effective and well-tolerated treatment options for relapsed-refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7 months (range, 0-9 months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow-up and two of them had remission with low-dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed-refractory immune cytopenia.

Is There a Clinical Significance of Very Low Serum Immunoglobulin E Level?

PURPOSE: High serum immunoglobulin (Ig) E levels are associated with allergies, parasitic infections, and some immune deficiencies; however, the potential effects and clinical implications of low IgE levels on the human immune system are not well-known. This study aims 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 patients with an IgE level < 2 IU/mL were included in this study. Demographic data, immunoglobulin levels, autoantibody 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 130 patients had IgE levels < 2 IU/mL. Fifty-seven patients were children (0.26%); 73 were adults (0.56%). There was a malignant disease in 34 (9 of them children) (26%), autoimmune diseases in 20 (3 of them children) (15.4%), and immunodeficiency in 17 (14 of them children) (13.1%) of the patients. The most common reasons were other diseases, immunodeficiency 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 deficiencies. Patients with very low IgE levels should be carefully monitored for systemic disorders.

Subarachnoid-pleural fistula in a child: the cause and treatment.

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.

Variant Guillain-Barré syndrome in a patient with Hodgkin lymphoma: AMSAN.

Lymphomas are solid tumors characterized by the malignant proliferation of lymphoid cells. Neurologic signs encountered in patients with Hodgkin's lymphoma can be due to the direct spread of tumor to the nervous system, secondary to chemotherapy or radiation, secondary to tumor mass compression, infectious causes and paraneoplastic syndromes. Paraneoplastic neurologic syndromes are rarely encountered in patients with Hodgkin's lymphoma and non-Hodgkin's lymphoma. Except for paraneoplastic cerebellar degeneration in Hodgkin's lymphoma and dermato/polymyositis in both Hodgkin's lymphoma and non-Hodgkin's lymphoma, other paraneoplastic syndromes are uncommon and have only been reported as isolated case reports or short series. Here, we present a patient with Hodgkin's lymphoma with symptoms of bilateral lower extremity weakness and loss of sensation before the start of therapy, who was eventually diagnosed as having motor and sensory axonal neuropathy.