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Master Thesis

Étude de 43 patients adultes présentant un lymphome et un déficit immunitaire de phénotype « Déficit Immunitaire Commun Variable ».

Abstract : In patients with a diagnosis of Common Variable Immunodeficiency (CVID), about 5% will develop a lymphoma during the course of the disease. Little is known about the characteristics of these patients and lymphomas. To date, lymphoma remains an exclusion criteria for CVID if preceding or revealing this condition. This descriptive unicentric retrospective study analysed 43 adult patients presenting both a CVID-like immune deficiency and a lymphoma, whatever the chronology of events. The aim of the study was to describe the clinical and biological phenotypes of these patients, as well as available genetic data and biopsy reports. We report here on 48 lymphomas with a high frequency of extranodal disease, including 65% of non-Hodgkin B-cell lymphomas, 27% of Hodgkin lymphomas, and 8% of T-cell lymphoma, with an EBV association rate of 50%. Diffuse large B cell lymphoma was the most common diagnosis (35%). Patients usually had an impaired humoral immunity, but above all a severe T-cell defect was present in 49% of them. CVID extra-infectious complications occurred with a high frequency (72%). With the current definition of CVID, 53% of patients were excluded of this diagnosis, as lymphoma preceded the diagnosis of hypogammaglobulinemia. Comparison between these patients and those who developed lymphoma during the follow-up of CVID showed significant differences concerning lymphoma subtypes distribution, but similar clinical and immunological characteristics in the two groups of patients. In conclusion, a diagnosis of CVID-like primary immune deficiency should not be systematically ruled out in patients presenting with hypogammaglobulinemia revealed or preceded by a lymphoma.
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Master Thesis
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Vincent Allain. Étude de 43 patients adultes présentant un lymphome et un déficit immunitaire de phénotype « Déficit Immunitaire Commun Variable ».. Médecine humaine et pathologie. 2017. ⟨dumas-02474488⟩



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