Frequency of lymphomas in a cohort of Common Variable Immunodeficiency (CVID) patients
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1
University of São Paulo Medical School, Division of Clinical Immunology and Allergy and Laboratory of Clinical Immunology and Allergy, LIM-60, Brazil
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2
University of São Paulo Medical School, Laboratory of Genetics and Molecular Hematology (LIM31), Brazil
Our objective was to evaluate the frequency of lymphomas in a cohort of CVID followed at the PID Clinic. Methods: Analysis of data obtained from medical records. Results: We analyzed 144 CVID patients followed between 1985 and 2012, being 80 men, aged 15-72 years at diagnosis. Seven lymphoma cases were diagnosed (0.5% - 5 men, 2 women) being 1 Hodgkin lymphoma, 6 non-Hodgkin lymphomas. Age of onset of CVID symptoms ranged from 2 - 53y, age at CVID diagnosis from 5 - 55y, age of lymphoma diagnosis from 19 - 53y. The time period between CVID onset of symptoms and lymphoma diagnosis ranged from 7 to 24 years in 4 patients and in 3 the diagnosis of CVID and lymphoma nearly overlapped, preceding CVID diagnosis in only 1 to 6 months. Lymphoma´s manifestations like fever of unknown origin, weight loss, splenomegaly and lymphadenopathy occurred in 100% of patients. Other manifestations observed: renal infiltration (2), hepatic infiltration (4), pulmonary infiltrates (3) and mediastinal mass (1). Three deaths due to lymphoma occurred during the study. Two patients presented lymphocytosis with inversion of CD4/CD8 ratio and one lymphopenia. Increased NK cells were observed in 3. Three patients in whom diagnosis of lymphoma and CVID were simultaneous presented undetectable B lymphocytes while in one, B cells were present at CVID diagnosis but undetectable at lymphoma diagnosis. Conclusions: Patients with absence of B cells in recently diagnosed CVID or vanishing B cells in long term follow-up CVID demands a screening for the presence of lymphomas.
Keywords:
CVID,
humoral immunity,
Lymphoma,
B lymphocytes,
Lymphopenia
Conference:
15th International Congress of Immunology (ICI), Milan, Italy, 22 Aug - 27 Aug, 2013.
Presentation Type:
Abstract
Topic:
Adaptive Immunity
Citation:
Sini
B,
Kokron
CM,
Levy
D,
Pereira
J,
Oliveira
AB,
Cohon
A,
Kalil
J,
Bydlowski
S and
Barros
MT
(2013). Frequency of lymphomas in a cohort of Common Variable Immunodeficiency (CVID) patients.
Front. Immunol.
Conference Abstract:
15th International Congress of Immunology (ICI).
doi: 10.3389/conf.fimmu.2013.02.00989
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Received:
28 Jun 2013;
Published Online:
22 Aug 2013.
*
Correspondence:
Dr. Cristina M Kokron, University of São Paulo Medical School, Division of Clinical Immunology and Allergy and Laboratory of Clinical Immunology and Allergy, LIM-60, São Paulo, Brazil, cmkokron@usp.br