Results 31 to 40 of about 215 (110)
Allergy, Volume 76, Issue 5, Page 1588-1591, May 2021.
Roy Gerth van Wijk +6 more
wiley +1 more source
Abstract Background Dyskeratosis congenita (DC) is a syndrome resulting from defective telomere maintenance. Immunodeficiency associated with DC can cause significant morbidity and lead to premature mortality, but the immunological characteristics and molecular hallmark of DC patients, especially young patients, have not been described in detail ...
Ting Zeng +9 more
wiley +1 more source
Abstract Mutations in SH2D1A, encoding the intracellular adaptor signaling lymphocyte activation molecule associated protein (SAP), are associated with X‐linked lymphoproliferative disease type 1 (XLP1). We identified a novel hemizygous SH2D1A c.49G > A (p.E17K) variant in a 21‐year‐old patient with fatal Epstein‐Barr virus infection–associated ...
Lamberto Torralba‐Raga +10 more
wiley +1 more source
Aspergilloma Superimposed Infection on Lymphoid Interstitial Pneumonia
We describe a case of a 27‐year‐old female without any prior underlying immunodeficiency syndromes who presented with hemoptysis secondary to subacute invasive pulmonary aspergillosis and subsequently diagnosed with lymphoid interstitial pneumonia (LIP). CT chest demonstrated bilateral interstitial disease with patchy opacities and multiple large cysts
Daniel Tran +5 more
wiley +1 more source
The SLAM family receptors contribute to diverse aspects of lymphocyte biology and signal via the small adaptor molecule SAP. Mutations affecting SAP lead to X-linked lymphoproliferative syndrome Type 1, a severe immunodysregulation characterized by ...
Bonnie Huang +5 more
doaj +1 more source
Follicular Helper T Cells in DiGeorge Syndrome
DiGeorge syndrome is an immunodeficiency characterized by thymic dysplasia resulting in T cell lymphopenia. Most patients suffer from increased susceptibility to infections and heightened prevalence of autoimmune disorders, such as autoimmune ...
Adam Klocperk +8 more
doaj +1 more source
The association between Kaposi's sarcoma and dysgammaglobulinemia [PDF]
The case of a patient with Kaposi's sarcoma and paraproteinemia is described and the relevant literature is reviewed. It is suggested that Kaposi's sarcoma is part of the spectrum of the lymphoproliferative diseases, and that both the marked angiogenesis and the synthesis of paraproteins in this disease are different expressions of the same ...
E, Ben-Chetrit, D, Ben-Amitai, Y, Levo
openaire +2 more sources
Patients with X‐linked lymphoproliferative disease 1 (XLP1) are exquisitely susceptible to Epstein‐Barr virus (EBV), with the first EBV infection often resulting in rapid death. In a manner not previously described, a 5‐year‐old patient with XLP1 presented solely with behavioral aggression, with no laboratory evidence of organ dysfunction or ...
Michelle M. Korah-Sedgwick +2 more
wiley +1 more source
Synchronous BALT Lymphoma and Squamous Cell Carcinoma of the Lung: Coincidence or Linkage?
A 72‐year‐old man presented with weight loss, fever, and malaise. Chest radiograph and CT revealed two large ill‐defined masses in middle and left lower lobes. CT‐guided biopsy of left lower lobe mass disclosed bronchus‐associated lymphoid tissue (BALT) lymphoma. Middle lobe mass was considered second deposit in contralateral lung. The patient received
Anastasia Oikonomou +11 more
wiley +1 more source
Multifaceted roles of IKZF1 gene, perspectives from bench to bedside
The IKZF1 gene encodes a transcription factor that belongs to the family of zinc-finger DNA-binding proteins associated with chromatin remodeling. The protein product, IKAROS, had been proved to regulate lymphopoiesis. Subsequent mouse model studies have
Lin Feng, Hang Zhang, Ting Liu
doaj +1 more source

