CVID, represents the most common symptomatic PID in adults in Costa Rica. It is characterized by hypogammaglobulinemia causing an heterogenous clinical phenotype including infectious, autoimmune, atopic and malignant complications.
We present a 37 years old male presenting CVID since his was 35 years old. The diagnostic of his PID was in contex of an episode of cryptococcal meningitis. At that moment, he showed low IgG (150 mg/dL), low IgM (16 mg/dL) and normal IgA, absent isohemagglutinins, poor response to vaccines and slight low B cells count (80 uL). He was succesfully treated.
Two years after, the patient consulted for subacute right upper quadrant pain and jaundice. The blood test revealed cholestasic and liver enzyme alteration. A thoracoabdominal CT was performed. At least 16 liver suggesting tumors images were found. Also CT manifested enlarged retroperitoneal and mediastinal lymph nodes.
The patient was admitted to the internal medicine service. A liver biopsy and an endobronchial ultrasound with transbronchial aspiration needle of a lymph node were performed. A flow citometry of the extracted lymph node requested.
The liver biopsy revealed germinal center DLBCL. Immunochemestry (IHC) was positive for HANS algorithm (Bcl-6, MUM1, CD10+).
Lymph node flow citometry reported the presence of 6.7% of mature monoclonal B lymphocytes (KAPPA +, LAMBDA-), in the absence of normal B cells.
Bone marrow biopsy resulted with DLBCL infiltration.
R-CHOP chemotherapy regimen was started.
PID is not only about infectious diseases. Unfortunately, we do not have the possibility of genetic squencing for PID diagnostic.