Question

A 40-year-old woman is referred to an immunology clinic for evaluation of her immune system. She has had frequent infections throughout her life, with childhood otitis, sinusitis, tonsilitis, and intermittent diarrhea. In her adult life, she has had recurrent respiratory and gastrointestinal infections. At 25 years of age she was diagnosed with thyroid insufficiency and has been on thyroid replacement hormone therapy. Her spleen is enlarged. Blood tests show low levels of all immunoglobulin isotypes, including IgM. She failed to make antibodies against the pneumococcal vaccine. B cell and T cell numbers were normal. No anti-nuclear antibodies were present, but antithyroid antibodies were present. Her mother and sister, both deceased, had hypogammaglobulinemia. Her mother had passed away from non-Hodgkin’s lymphoma and her sister died from gastrointestinal cancer. What is this patient’s likely diagnosIS?

Answer 1

A familial background of non-Hodgkin’s lymphoma (NHL) has repeatedly been reported to constitute a significant risk factor for lymphoma development. In an evaluation of first-degree relatives of lymphoma patients,observed an increased rate of both NHL as well as Hodgkin’s lymphoma

A negative ANA test effectively excludes a diagnosis of ANA associated rheumatic disease such as drug-induced lupus and, in the majority of cases, SLE, systemic sclerosis/scleroderma and mixed connective tissue disease,