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Introduction: We present the case of a 60-year-old female patient with generalized weakness, dyspnea, unintentional weight loss, and gastrointestinal symptoms, including intermittent epigastric pain and gastroesophageal reflux. The initial diagnosis suggested a possible gastrointestinal malignancy. Despite an initial approach to rule out infectious or neoplastic pathology, the direct Coombs test was positive for warm IgG antibodies, leading to a diagnosis of autoimmune hemolytic anemia (AIHA).
Management and treatment: Treatment was initiated with methylprednisolone and other supportive medications. Abdominal ultrasound and computed tomography revealed splenomegaly. Subsequently, the patient was transferred to a tertiary care center, where bone marrow aspiration confirmed infiltration by splenic marginal zone lymphoma (SMZ), with AIHA as the initial clinical presentation.
Results: The patient responded positively to treatment with Rituximab, showing clinical improvement, regulation of hemoglobin and bilirubin levels, and resolution of splenomegaly.
Conclusion: It is important to consider hematological diagnoses in patients with atypical AIHA presentation, as in this case, where SML debuted with AIHA. Early diagnosis and appropriate treatment with Rituximab allowed for substantial clinical improvement
