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CEN Case Rep
January 2025
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
A 69-year-old Japanese man developed abdominal pain, purpura, proteinuria, and hematuria while receiving treatment for pulmonary tuberculosis. A skin biopsy revealed IgA-positive leukocytoclastic vasculitis, and a renal biopsy showed IgA-positive mesangial proliferative glomerulonephritis with crescent formation. Based on these findings, we diagnosed IgA vasculitis with nephritis (IgAVN) and initiated treatment.
View Article and Find Full Text PDFBackground: Germline haplodeficiency (RHD) is associated with thrombocytopenia, platelet dysfunction and predisposition to myeloid malignancies. Platelet expression profiling of a RHD patient showed decreased encoding for the A subunit of factor XIII, a transglutaminase that cross-links fibrin and induces clot stabilization. FXIII-A is synthesized by hematopoietic cells, megakaryocytes and monocytes.
View Article and Find Full Text PDFItal J Pediatr
January 2025
Pediatric Hematology and Oncology, SBU Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.
Background: Rare bleeding disorders (RBDs) include fibrinogen (Factor I), prothrombin (Factor II), Factor V(FV), combined Factor V and Factor VIII, Factor VII, Factor X, Factor XI, Factor XII, and Factor XIII deficiencies. This group accounts for 3-5% of all factor deficiencies. Different symptoms may occur, ranging from mild or moderate bleeding to serious and life-threatening bleeding, which may not be related to the factor level.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Academic Affairs, National Jewish Health, Denver, CO, United States.
Granulomas, organized aggregates of immune cells which form in response to (), are characteristic but not exclusive of tuberculosis (TB). Despite existing investigations on TB granulomas, the determinants that differentiate host-protective granulomas from granulomas that contribute to TB pathogenesis are often disputed. Thus, the goal of this narrative review is to help clarify the existing literature on such determinants.
View Article and Find Full Text PDFCureus
November 2024
General Medicine, Saveetha Medical College, Chennai, IND.
Acquired Factor XIII (FXIII) deficiency is a rare condition often associated with underlying medical conditions or medications. We present a case of a 23-year-old male, who presented with prolonged bleeding from a traumatic ulcer site on his left leg. Initial laboratory investigations revealed a severe deficiency in FXIII activity (30%) and antigen levels (25%), with no evidence of congenital disorders or other underlying pathologies.
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