Survival of a Severe Combined Immunodeficiency Patient from Transfusion-associated Graft-versus-host Disease: A Case Report

Patients with Severe Combined Immunodeficiency are at high risk of Transfusion Associated Graft Versus Host Disease (TAGVHD) if they receive a non-radiated blood product that has a high mortality rate. This Case study reports on the case of a premature baby who had anemia of prematurity with a normal level of WBC. He received packed red blood cells, but after a while, he developed severe nausea and skin lesions. Endoscopy was done and the biopsy from the gastrointestinal lesions as well as the biopsy from the skin lesions showed graft versus host disease when he was 6 months old. However, he has received Hematopoietic Stem Cell Transplantation and is well now after about 4 months from his transplant

Assessment of the First Presentations of Common Variable Immunodeficiency in a Large Cohort of Patients

BACKGROUND: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency syndrome resulting in recurrent infections, autoimmunity, and granulomatous manifestations. METHODS AND MATERIALS: This retrospective study was conducted on an Iranian national registry of immunodeficient patients from 2010 to 2021. The frequency of first presentations of CVID and its association with sex, age of onset, and family history of CVID was evaluated. RESULTS: A total of 383 patients entered the study, 164 of whom were female, and the rest were male. The mean age of the patients was 25.3 ± 14.5 years. The most frequent first presentations of CVID were pneumonia (36.8%) and diarrhea (19.1%). Patient sex, age of onset, and family history did not make significant differences in first presentations of this disease. CONCLUSION: pneumonia is the most common first presentation of CVID. Family history of CVID, the age of symptom onset, and sex made no differences in the first presentations of CVID

Terminal ileitis and cytotoxic lesion of corpus callosum as the presenting features of Multisystem inflammatory syndrome in children (MIS-C): a case report

Abstract Background Multisystem inflammatory syndrome in children (MIS-C) is a post-viral inflammatory vasculopathy characterized by persistent fever, multiorgan dysfunction, significant laboratory markers of inflammation, lack of an alternative diagnosis, and prior SARS-CoV-2 infection or exposure in children and adolescents. The most common early symptoms include a prolonged fever, as well as dermatologic, mucocutaneous, and gastrointestinal symptoms such abdominal pain, vomiting, and diarrhea. Case presentation We present a pediatric patient with multisystem inflammatory syndrome with the development of abdominal pain and seizure who was found to have a circumferential wall thickening of the terminal ileum and ileocecal junction in abdominal CT scan. The brain MRI of the patient showed cytotoxic lesions of the corpus callosum (CLOCC) which had hypersignal intensity with a few diffusion restrictions in the splenium of the corpus callosum. Conclusion This case is being reported to raise awareness of MIS-C presenting characteristics. Given the rising number of MIS-C patients and a lack of understanding regarding early diagnostic clinical characteristics and therapy, further research into clinical presentations, treatment, and outcomes is urgently needed