Medical Journal of Dr. D.Y. Patil Vidyapeeth, 2023 Background: The cluster of differentiation 200 (CD200) is a recently introduced marker, used to differentiate various lymphoproliferative disorders (LPDs) and is a potential target for chemotherapy.
Pavuluri Divya +8 more
doaj +1 more source
Journal of Pediatric Gastroenterology and Nutrition, EarlyView.Abstract Objectives Shwachman‐Diamond syndrome (SDS) is an inherited bone marrow failure disorder, and its hepatic phenotype is poorly defined. Our objective was to systematically characterize the prevalence, features, and outcomes of liver injury in a multicenter SDS cohort. Methods Retrospective registry study of 171 patients with biallelic Shwachman‐
Jane Koo +16 more
wiley +1 more source
Fatal lymphoproliferation and acute monocytic leukemia-like disease following infectious mononucleosis in the elderly [PDF]
, 1981 Three elderly patients are reported, in whom serologically confirmed recent infectious mononucleosis is followed by fatal lymphoproliferation (case 1), by acute monocytic leukemia (case 2), and by acute probably monocytic leukemia (case 3)
Deinhardt, F. +5 more
core +1 more source
Therapeutic Options for IgA Pemphigus in Patients Refractory to Dapsone
JEADV Clinical Practice, EarlyView.ABSTRACT IgA pemphigus is a rare autoimmune blistering disorder with limited therapeutic guidance available due to its rarity and complex presentation. This comprehensive analysis evaluates the therapeutic outcomes of 67 published patients, emphasising the significant role of dapsone as the first‐line treatment.
Lorenz Frasheri +2 more
wiley +1 more source
A mutation in caspase-9 decreases the expression of BAFFR and ICOS in patients with immunodeficiency and lymphoproliferation [PDF]
, 2015 Lymphocyte apoptosis is mainly induced by either death receptor-dependent activation of caspase-8 or mitochondria-dependent activation of caspase-9. Mutations in caspase-8 lead to autoimmunity/lymphoproliferation and immunodeficiency. This work describes
Boggio, E +15 more
core +2 more sources
Sjögren Syndrome Complicated by Mucosa-Associated Lymphoid Tissue Lymphoma and Lymphocytic Interstitial Pneumonia. [PDF]
, 2015 Sjögren syndrome (SS) is an autoimmune disease with exocrine glands dysfunction and multiorgan involvement. It is associated with increased risk of lymphoproliferative disorders, especially B-cell marginal zone lymphoma.
Ahmed, Fatma +5 more
core +4 more sources
Refractory Eczema as a Presenting Feature of Common Variable Immunodeficiency
JEADV Clinical Practice, EarlyView.ABSTRACT Common variable immunodeficiency (CVID) is the most prevalent symptomatic inborn error of immunity (IEI) in adults. It presents with recurrent infections and non‐infectious complications, including autoimmunity, lymphoproliferation and dermatitis.
Mercedes Sanchez‐Diaz +2 more
wiley +1 more source
Somatic Mutations in Acquired Pure Red Cell Aplasia: Incidence and Implications
American Journal of Hematology, EarlyView.
Robert T. Means Jr
wiley +1 more source
Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. [PDF]
, 2020 Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification ...
Ailal, Fatima +18 more
core
An unusual unifocal presentation of Castleman’s disease in a young woman with a detailed description of sonographic findings to reduce diagnostic uncertainty: a case report [PDF]
, 2013 Background: Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal masses and causes a wide range of clinical symptoms.
Maden, Zerrin, Wagner, Norbert
core +1 more source