Primary adrenal non-Hodgkin's lymphoma associated with autoimmune hemolytic anemia: a case diagnosed by ultrasound-guided fine needle biopsy

Abstract

Primary localized adrenal non-Hodgkin's lymphomas are extremely rare. Only 28 observations have been reported so far, all with a very poor prognosis: a median survival of 12.5 weeks. The authors report the case of a 78-year-old male with primary non-Hodgkin's lymphoma of the right adrenal gland. Disease onset was characterized by severe autoimmune hemolytic anemia, and diagnosis was made by echo-guided biopsy. The patient was admitted to our Department with severe autoimmune hemolytic anemia; the hemoglobin value was 6.5 g/dL and both indirect and direct Coombs' tests were positive. Steroid treatment with methylprednisolone 2 mg/kg/day did not improve the hemolytic process. Abdominal ultrasound examination disclosed a right hypoechogenic suprarenal mass of 10 x 9 cm; imaging techniques such as computed tomography and magnetic resonance imaging were not useful in the etiologic diagnosis of the right suprarenal mass; fine needle aspiration and tissue-core biopsy revealed low-grade non-Hodgkin's lymphoma. Staging procedures, including clinical examination, total body computed tomography scan, bone-marrow biopsy, gallium scan, abdominal magnetic resonance imaging, did not disclose other sites of involvement and strongly supported a diagnosis of primary non-Hodgkin's lymphoma of the right adrenal gland. Hormone assays were within normal limits. The patient was treated with chemotherapy, cyclophosphamide-vincristine-prednisone regimen, with good regression of the adrenal mass after 6 courses, and normalization of hemoglobin level and negativity of Coombs' tests. The importance of this case lies in the very rare occurrence of this disease, its association with autoimmune hemolytic anemia, the diagnosis made by ultrasound-guided biopsy, and good response to treatment with respect to cases reported in the literature. The patient remains in clinical remission 12 months after onset of the disease.