Common variable immunodeficiency (CVID) is an immune disorder characterized by a defect in immunoglobulin production secondary to compromised B cell differentiation. It is often diagnosed between the second and fourth decades of life with initial presentations varying widely from recurrent infections to autoimmunity. Those diagnosed with CVID have a higher incidence of malignancy including non-Hodgkin lymphoma.
A 36-year-old male presented to the hospital after labs for an outpatient workup of fatigue and bruising revealed a platelet count of 7 K/μL (150-350 K/μL). Significant past medical history includes asthma, eczema, and psoriasis. He was admitted to the hospital and treated for presumed immune thrombocytopenia with 1 g/kg IVIG and 4-day course of 40 mg dexamethasone. Platelets improved to 23 K/μL and IVIG was discontinued after one dose. During hospitalization, he was noted to have a serum IgG of 495 mg/dL (660-1690 mg/dL) before initiation of IVIG and IgA level of 24 mg/dL (57-543 mg/dL). After discharge, labs showed initial improvement of IgG to 1,850 mg/dL but decreased to 605 mg/dL on repeat testing; the patient was subsequently referred to an Immunologist for suspected CVID. He reported a 2-year history of recurrent cellulitis and upper respiratory infections and noted generalized fatigue. CT chest during workup showed multiple prominent mediastinal lymph nodes and left axillary lymphadenopathy. Repeat imaging 6 weeks later showed persistence of lymphadenopathy and the patient underwent lymph node biopsy with findings consistent with diffuse large B cell lymphoma. He completed 6 cycles of chemotherapy and achieved remission.
Due to the vast clinical presentations of CVID, initial screenings are often initiated based on presenting symptoms. This case highlights the necessity of radiographic chest imaging in newly diagnosed CVID. Although this patient did not present with pulmonary symptoms, fever, or night sweats, early imaging allowed for the detection and diagnosis of underlying lymphoma.
(A) Initial CT chest with mediastinal lymphadenopathy. (B) Repeat CT chest 6 weeks later with persistent lymphadenopathy.
(A) Initial CT chest with mediastinal lymphadenopathy. (B) Repeat CT chest 6 weeks later with persistent lymphadenopathy.
