Ferenc Sipos, Hungary
Semmelweis University 2nd Department of MedicinePresenter of 1 Presentation
DIAGNOSIS OF NON-HODGKIN LYMPHOMA PRECEDING THE RECOGNITION OF THE UNDERLYING PRIMARY IMMUNODEFICIENCY
Abstract
Background and Aims
CVID is a clinically relevant type of PID with manifestations including recurrent infections, autoimmune diseases, and malignancies. The risk of B-cell NHL, diffuse, and high grade with extranodal primary sites is especially increased.
Methods
Patients. A 60-yr-old female has been suffering from sinobronchial infections and splenomegaly for years. Later due to persistent lymphocytosis NHL/CLL was diagnosed with sec. immunodeficiency. Six cycles of FC were administered. A 41-yr-old male displayed recurrent infections, splenomegaly, pulmonary infiltrates, and hypogammaglobulinemia for years. Pulmonary biopsy indicated NHL/MZL. Six cycles of CVP were administered. A 33-yr-old male with recurrent fever, pulmonary infiltrates, and lymphadenopathy starting 3 years ago was diagnosed with NHL/DLBCL by lymph node biopsy, and with sec. immunodeficiency. Six cycles of R-CHOP were administered. A 39-yr-old female with a 3-month presentation of splenomegaly, pancytopenia, and low Ig-levels was diagnosed upon crista biopsy with NHL/THRLBCL. Six cycles of R-CHOP were administered. The patients were referred to our division for evaluation of the persisting Ig-abnormalities.
Results
Upon the ESID criteria we diagnosed all 4 patients with CVID; (marked reduction of Ig-isotypes and class-switched CD27+IgD−IgM− memory B-cells, increased number of CD19+CD21− immature B-cells.) In their cases NHL probably developed on the basis of immune dysregulation. Their PID status now is stable with regular, monthly given IVIG (Intratect).
Conclusions
The association of CVID with malignancy is well established. In general, NHL develops after a period of the CVID onset. Our cases reflect the difficulty of CVID diagnosis, mainly in respect of potential sec. causes of immunodeficiency, like NHL.