Granulocyte progenitors (CFC) from 12 patients with idiopathic neutropenia were cultured in vitro and followed, in some cases, for several months. The results were similar whatever the degree of neutropenia and in the presence or absence of medullary hypoplasia. The medullary concentration of CFC was always clearly depressed, which correlated with a very low total number of CFC. In every case, when the cloning efficiency was low, there were few CFC per 10(5) metamyelocytes plated, which suggests an increase in mitotic amplification between the progenitor and the mature granulocytes. When circulating CFC were cultured, there was little or no growth, confirming the paucity of CFC in the host. No patient had an increase in cloning efficiency in marrow or blood that might have suggested ineffective granulopoiesis. In four patients, stimulatory or inhibitory activity in serum was studied; one serum was stimulatory, another, on two occasions, inhibitory. Possible machanisms for these results and their prognostic value are discussed.
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Immunol Res
January 2025
Laboratory of Clinical Immunology, Infectiology, and Autoimmunity (LICIA), Faculty of Medicine and Pharmacy of Casablanca, Hassan II University, Casablanca, Morocco.
Congenital neutropenia (CoN) is a heterogeneous group of inborn errors of immunity (IEI) characterized by recurrent infections and early onset of neutropenia (NP). This study aimed to investigate the demographic and clinical data of children with CoN and idiopathic neutropenia (IN) in Morocco. We performed a retrospective study of patients with CoN and analyzed the clinical and laboratory findings of patients with CoN and IN diagnosed between 1999 and 2018 in a clinical immunology unit of a large pediatric hospital.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
Center for Medical Experiments (CME), Guangming District People's Hospital, Shenzhen, 518106, China.
Background: Idiopathic CD4+ T lymphocytopenia is a rare immune dysfunction disease that is usually found after opportunistic infections. Mycobacterium abscessus is a rapidly growing mycobacterium that can cause pulmonary infections, lymphadenitis, skin and soft tissue infections, disseminated infections, among others, as a conditional pathogenic bacterium.
Case Presentation: We present the case of a 43-year-old Chinese woman who developed disseminated Mycobacterium abscessus infection due to idiopathic CD4+ T lymphocytopenia.
Arch Soc Esp Oftalmol (Engl Ed)
December 2024
Servicio Oftalmología, Hospital Alfredo Espinosa, Urduliz, Bizkaia, Spain.
A 66-year-old woman presents with unilateral orbital inflammation of several years of evolution, initially classified as idiopathic orbital inflammatory disease (IOID) subtype myositis. She presented with pain, eyelid inflammation and significant elevation of intraocular pressure, as well as radiological signs of exophthalmos and superior rectus myositis. She was treated with corticosteroids, with partial response.
View Article and Find Full Text PDFCureus
November 2024
Hematology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR.
Evans syndrome (ES) is a rare syndrome characterised by the association of autoimmune idiopathic hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) and, less commonly, autoimmune neutropenia (AIN). ES may be primary or secondary to some aetiology, including, exceptionally, tuberculosis. We describe a case of association between pulmonary and medullary tuberculosis and Evans syndrome with an effective response to antitubercular treatment and corticosteroids.
View Article and Find Full Text PDFReumatologia
November 2024
Department of Children's Diseases and Pediatric Surgery, I. Horbachevsky Ternopil National Medical University, Ukraine.
Systemic autoinflammatory diseases caused by dysregulation of the innate immunity are a known cause of recurrent fevers. We present the molecular diagnosis results of 12 children with recurrent fever, analyzing the correlation between molecular findings and clinical symptoms. No pathogenic variants confirming autoinflammatory disease were found.
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