Introduction: Primary immunodeficiency diseases (PIDs) are a group of heterogeneous disorders that result from one or more immune system abnormalities and have a wide range of clinical manifestations.
Aim: To evaluate the demographic, clinical, and radiological features of adult patients with PID.
Material And Methods: We retrospectively reviewed the data of adult patients with PID who had been receiving immunoglobulin therapy at the adult allergy and immunology outpatient clinic between November 2017 and April 2022.
Results: Of the 23 patients included, 4 (17.4%) were females and 19 (82.6%) were males. The mean age was 38.7 ±16.2 (range: 18-75) years. Time of delay in the diagnosis of immunodeficiency was 14.9 ±15.7 (range: 0.5-49) years. The most common complaint on admission was sinopulmonary infection, and 65% of the patients had bronchiectasis. A total of 6 patients had a history of lymphoma, and 3 of them were diagnosed during the study period. Recurrence of lymphoma was observed in 1 patient.
Conclusions: Patients with PID frequently have findings related to more than one organ system, and the diagnosis is often delayed in adults. Recognition and increased awareness of these manifestations is essential for early diagnosis and reducing morbidity and mortality.
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http://dx.doi.org/10.5114/ada.2022.120887 | DOI Listing |
Rev Alerg Mex
December 2024
Facultad de Medicina, Universidad Autónoma de Campeche, Campeche.
Background: Congenital neutropenia is a primary immunodeficiency characterized by quantitative anomalies in neutrophil counts. It is classified as mild, moderate, or severe. Hematopoietic stem cell transplantation stands as a potential therapeutic intervention; nevertheless, graft-versus-host disease emerges as a main complication.
View Article and Find Full Text PDFCureus
December 2024
Infectious Diseases, Hospital Garcia de Orta, Lisbon, PRT.
Extra-cavitary primary effusion lymphoma (PEL), often associated with human herpes virus 8 (HHV8) infection, represents a rare and aggressive form of non-Hodgkin lymphoma, which is predominantly found in individuals with severe immunosuppression. As an acquired immunodeficiency syndrome (AIDS)-associated lymphoma, PEL typically manifests in the context of advanced human immunodeficiency virus (HIV) infection, requiring tailored therapeutic approaches to manage both the lymphoma and underlying immunodeficiency. A 53-year-old male patient from Cape Verde presented with a three-day history of fever, night sweats, right iliac fossa pain, hematochezia, and an unintentional weight loss of five kilograms over the previous two months.
View Article and Find Full Text PDFBMC Nephrol
January 2025
Division of Nephrology, Department of Internal Medicine, Faculty of Health Sciences, University of the Free State, 205 Nelson Mandela Drive, Bloemfontein, 9300, South Africa.
Background: Continuous ambulatory peritoneal dialysis (CAPD) is one of the kidney replacement therapy (KRT) modalities used in patients with kidney failure. It is the preferred modality in most resource-limited settings as it is more accessible and cost-effective. CAPD technique failure remains a challenge and is associated with an increased risk of morbidity and mortality.
View Article and Find Full Text PDFJ Clin Immunol
January 2025
Department of Health Systems & Implementation Science, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA.
Purpose: Patients with inborn errors of immunity (IEI) have lifelong health complications including severe infections and physical impairments. Previous studies show that a patient's perception of their health is an important predictor of health outcomes. The purpose of this study was to understand factors related to patient reported health status.
View Article and Find Full Text PDFJAMA Oncol
January 2025
Department of Paediatric Haematology, Oncology and Immunodeficiency, University Hospital Justus-Liebig University Giessen, Giessen, Germany.
Importance: The current standard-of-care salvage therapy in relapsed/refractory classic Hodgkin lymphoma (cHL) includes consolidation high-dose chemotherapy (HDCT)/autologous stem cell transplant (aSCT).
Objective: To investigate whether presalvage risk factors and fludeoxyglucose-18 (FDG) positron emission tomography (PET) response to reinduction chemotherapy can guide escalation or de-escalation between HDCT/aSCT or transplant-free consolidation with radiotherapy to minimize toxic effects while maintaining high cure rates.
Design, Setting, And Participants: EuroNet-PHL-R1 was a nonrandomized clinical trial that enrolled patients younger than 18 years with first relapsed/refractory cHL across 68 sites in 13 countries in Europe between January 2007 and January 2013.
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