Evans Syndrome with Double-Positive Antiphospholipid Antibody Syndrome, NGS-Confirmed Cystic Fibrosis, Chronic Pancreatitis, Type-1 Diabetes Mellitus and Pulmonary Tuberculosis with Nocardiosis: A Rare Multi-System Overlap in a Young Adult Male

Abstract

Evans syndrome is an uncommon autoimmune hematological disorder characterized by the coexistence or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia. Its occurrence with antiphospholipid antibody syndrome, cystic fibrosis, chronic pancreatitis, insulin-dependent diabetes mellitus, pulmonary tuberculosis, and nocardiosis creates a highly complex diagnostic and therapeutic situation. We report the case of a 33-year-old male with multiple established autoimmune, genetic, metabolic, and infectious comorbidities who presented with acute left lower limb pain and swelling for three days. Clinical examination showed erythema, tenderness, and swelling of the affected limb. Doppler ultrasonography confirmed deep vein thrombosis. Laboratory evaluation showed anemia, thrombocytopenia, and markedly raised D-dimer. The patient had a background of Evans syndrome, double-positive antiphospholipid antibody syndrome, next-generation sequencing-confirmed cystic fibrosis, chronic pancreatitis, insulin-dependent diabetes mellitus, pulmonary tuberculosis on anti-tubercular therapy, and nocardiosis. He had recently received eltrombopag for severe thrombocytopenia following a hemolytic episode. The thrombotic event was considered to be temporally associated with eltrombopag use in the presence of an underlying prothrombotic antiphospholipid antibody profile. Management included anticoagulation with enoxaparin, discontinuation of eltrombopag, continuation of anti-tubercular therapy, immunosuppressive therapy, insulin treatment, analgesia, and supportive care. During hospitalization, limb pain and swelling improved, and the platelet count increased to 0.60 lac/cu.mm by Day 10. This case highlights the importance of individualized risk assessment when using thrombopoietin receptor agonists in patients with prothrombotic autoimmune disease and emphasizes the need for coordinated multidisciplinary management in complex multisystem overlap presentations.