Non-Hodgkin's lymphoma (NHL) is the predominant malignancy in CVID patients, accounting for 47 to 64% of all tumors. The coexistence of immunodeficiency and malignancy presents a challenge for the diagnosis and treatment of this group of patients. Herewith we present a pediatric patient who developed NHL early after CVID diagnosis.
laboratory and immunological tests
A six year old boy was diagnosed with CVID based on the clinical manifestations for recurrent respiratory infections, oligoarthritis and agammaglobulinemia. One year after diagnosis and initiation of IVIG therapy, abdominal pain, fever and diarrhea occurred. Laboratory tests made show leukocytosis, elevated CRP and ESR. The laparotomy revealed generalized abdominal lymphadenomegaly. The histological evaluation pointed to NHL - EBV+ high-grade B-cells lymphoma. The administration of Rituximab, CHOP and IVIG had a good effect.
Lymphoma, may develop in pediatric CVID patients with no previous signs of lymphoid hyperplasia and even early after CVID diagnosis. Therefore, strategies for cancer prevention and/or early diagnosis are required in pediatric CVID patients. This case is an example of good collaboration between immunologist, pediatricians, oncologist and other professionals.