Splenectomy links guillain-barré syndrome, autoimmune haemolysis and ebv viraemia: unveiling a hidden diagnosis

Abstract

A 64-year-old man presented to the emergency department of a district general hospital with a widespread rash. He received antihistamine treatment and was discharged. He subsequently returned with peripheral paraesthesia and severe back pain. Neurological assessment showed pronounced bilateral ascending lower limb motor weakness, and further review found diplopia and dysphagia. A diagnosis of Guillain-Barré syndrome (GBS) with the Miller Fisher variant was established, and treatment with high-dose intravenous immunoglobulin (IVIG) was initiated. The patient was transferred to a tertiary centre for nerve conduction studies. During repatriation, the patient developed severe hyponatraemia, and paired serum and urine osmolality testing indicated syndrome of inappropriate antidiuretic hormone secretion (SIADH). A staging CT discovered numerous borderline enlarged lymph nodes, seemingly consistent with a viral screen, which identified an Epstein-Barr virus (EBV) viraemia. A rapid drop in the patient’s haemoglobin alongside a positive direct antibody test prompted a further diagnosis of autoimmune haemolytic anaemia (AIHA). High-dose steroids and rituximab both failed to abate the haemolysis, necessitating an ITU admission for stabilisation and emergency splenectomy. Histopathological analysis of the spleen was consistent with angioimmunoblastic T-cell lymphoma (AITL), providing a unifying explanation for the patient’s diverse autoimmune manifestations. The patient was initiated on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, and he achieved a complete metabolic response on interim PET-CT, and remains in remission at nine months of follow-up. This presentation of AITL with concurrent GBS and AIHA highlights the potential for this disease to present primarily with features of immune dysregulation.