INTRODUCTION
Common variable immunodeficiency (CVID) has a high incidence of gastrointestinal manifestations and an increased risk of malignancy. From 2 to 10% of patients with CVID develop malignant lymphoid type, usually non-Hodgking B-cell lymphomas. They frequently involve extranodal sites, which includes the gastrointestinal tract.
CLINICAL CASE
A 33-year-old (y/o) male patient.
Evolution:
14 y/o: autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura.
20 y/o: 2 abscesses one malar and axillary. Severe pneumonia, so protocol is performed for primary immunodeficiency and concludes CVID.
30 y/o: intermittent diarrhea, steatorrhea with foamy stools.
Colonoscopy: chronic inflammation with lymphoid hyperplasia.
Celiac disease is diagnosed, gluten-free and dairy-free diet begins, with symptom improvement.
33 y/o: Hematochezia and diarrhea.
Colonoscopy and endoscopic capsule: non-Hodgkin's lymphoma of B cells in ileum by biopsy.
Treatment with chemotherapy is started. 6 months later due to complications of chemotherapy, he presents massive hemorrhage of the lower digestive tract and dies.
DISCUSION
The manifestations of gastrointestinal malignancy in patients with CVID are the 2nd cause of death, so patients should be suspected and evaluated routinely when presenting symptoms, since celiac disease or the syndrome of irritable bowel can progress to malignancy. Patients with CVID may also develop gastrointestinal nodular lymphoid hyperplasia, leading to misdiagnosis of indolent gastrointestinal lymphoid malignancies.
CONCLUSION
The monitoring of patients is recommended endoscopies every 24 months in patients with normal histology, every 12 months in patients with gastritis atrophy or intestinal metaplasia and every 6 months with dysplasia.