PubMed · 7901653
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D Fuchs, G Weiss, C Murr, H Wachter. 1993-11-27. Interleukin-10 for common variable immunodeficiency.. https://doi.org/10.1016/0140-6736(93)92272-u
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We evaluated the T-cell repertoire and the thymic output in two infants, one with Omenn Syndrome (OS) and another with complete DiGeorge Syndrome (DGS), who developed generalized dermatitis. The patients shared common T-cell abnormalities, as demonstrated by the low response to mitogenic stimulation, by an unusual usage of specific T-cell receptor (TCR) segments, and by a reduction of TCR diversity in both alpha/beta and gamma/delta populations. Furthermore, they both showed an impaired thymic function, as assessed by the low number of TCR recombination excision circles, which are formed from excised DNA during the rearrangement of TCR genes. These data indicated that generalized erythrodermia may be present in different forms of T-cell immunodeficiency and may reflect intrinsic defects in either V(D)J recombination or in thymic development, leading to the peripheral expansion of T-cell clonotypes, that bear peculiar TCR chains.
Paraneoplastic pemphigus (PNP) is a rare multisystem, autoimmune disease with prominent mucocutaneous manifestations, occurring most commonly in association with haematological malignancies. It is characterised by the presence of circulating autoantibodies against epithelial adhesion proteins. We report a 46-year-old woman with common variable immunodeficiency who developed paraneoplastic pemphigus after receiving radiotherapy for relapsed non-Hodgkin's lymphoma. Flaccid bullae covering approximately 70% of the skin, painful oropharyngeal ulceration and periocular erosions were prominent clinical features. Despite supportive treatment and attempts at disease control using high-dose corticosteroids and cyclophosphamide, the patient became increasingly debilitated, developed septic shock secondary to Pseudomonas aeruginosa septicaemia on two occasions and died of respiratory failure 6 weeks after presentation. We highlight the need to be aware of (PNP) and to perform appropriate immunological investigations. In addition, we emphasise the importance of a multidisciplinary approach to the management of such patients.
Primary effusion lymphoma (PEL) is a newly described high-grade B cell lymphoma developing in association with human herpes virus type 8 (HHV-8) in human immunodeficiency virus (HIV)-infecting individuals. Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by reduced serum immunoglobulin and heterogeneous clinical features. The risk of cancer in CVID patients is increased. Here, we describe a PEL that developed in the pleural and pericardial cavities of an HIV-negative and HHV-8-negative patient with CVID.