Anna Bobcakova, Slovak Republic
University Teaching Hospital in Martin Department of Pulmonology and PhthisiologyPresenter of 1 Presentation
ANTIBODY DEFICIENCY ASSOCIATED WITH LYMPHOMA AND BRONCHIECTASIS
Abstract
Background and Aims
Common variable immunodeficiency (CVID) is one of the clinically most important primary immunodeficiencies. Clinical picture of untreated patients usually includes complicated recurrent respiratory tract infections. Patients with CVID are in higher risk of lymphoreticular malignancies and gastric cancer. On the other hand, haematological malignancies and their therapy can lead to hypogammaglobulinemia development.
Methods
We present a case of 59-year-old female patient with the history of recurrent lower respiratory tract infections and bronchopneumonias since early childhood. The frequency and course of infections became worse after the diagnosis and the therapy of B-cell Non-Hodgkin Lymphoma. Laboratory tests revealed three-isotype hypogammaglobulinemia, but results could have been influenced by recent anti-CD20+ targeted therapy (rituximab).
Results
Since laboratory examinations performed in the past before lymphoma diagnosis and therapy have also shown hypogammaglobulinemia, and B cell depletion persists more than one year after the last administration of rituximab, we suspect primary immunodeficiency (CVID) to be the cause of antibody deficiency and later development of lymphoma. Moreover, detailed patient's history analysis (bronchiectasis since early childhood, heterotaxy) lead to suspicion of primary ciliary dyskinesia (PCD), which was confirmed by the analysis of ciliary kinematics.
Conclusions
We would like to point out that some of patients with lymphoreticular malignancy may represent undiagnosed CVID patients. The documentation of these patients from the period before manifestation of lymphoreticular malignancy has to be carefully analysed to discover possible clinical signs and laboratory findings typical for immunodeficiency. Moreover, we would like to raise the awareness about primary ciliary diskinesia as a cause of early bronchiectasis development.