Management of rapidly progressive symptomatic splenomegaly with associated pancytopenia: a rare case of splenic hamartoma

Authors

• Dimitra Taprantzi Department of Internal Medicine, University General Hospital of Patras, Rio, Greece
• Irene Terzi Department of Internal Medicine, University General Hospital of Patras, Rio, Greece; Medical School of Patras, University of Patras, Rio, Greece 0000-0001-5765-533X
• Dimitrios Velissaris Department of Internal Medicine, University General Hospital of Patras, Rio, Greece; Medical School of Patras, University of Patras, Rio, Greece
• Evgenia Verigou Division of Haematology, Department of Internal Medicine, University General Hospital of Patras, Rio, Greece
• Nikolaos Mpenetatos Department of General Surgery, University General Hospital of Patras, Rio, Greece
• Charalampos Potsios Department of Internal Medicine, University General Hospital of Patras, Rio, Greece

gter41@otenet.gr

Keywords

Abstract

Introduction: Splenomegaly with focal splenic lesions represents a complex diagnostic entity, encompassing a broad spectrum of haematological, infectious, autoimmune, infiltrative and malignant disorders. Distinguishing benign from malignant pathology is particularly challenging when splenomegaly is accompanied by cytopenias and a rapidly progressive clinical course.
Case description: A 43-year-old woman presented with severe left upper quadrant abdominal pain and persistent vomiting. Physical examination revealed splenomegaly, and laboratory investigations showed pancytopenia. Abdominal ultrasonography, computed tomography scan and magnetic resonance imaging demonstrated a large heterogeneous splenic mass with progressive enlargement and mass effect on adjacent organs. ¹⁸F-fluorodeoxyglucose positron emission tomography scan showed no abnormal metabolic activity. Extensive laboratory evaluation and image-guided biopsy failed to establish a definitive diagnosis. Despite conservative management, the patient’s clinical condition progressively deteriorated, with persistent symptoms and worsening hypersplenism. Owing to the ongoing diagnostic uncertainty and clinical deterioration, splenectomy was performed for both diagnostic and therapeutic purposes. Histopathological examination confirmed the diagnosis of splenic hamartoma.
Conclusion: This case highlights the limitations of non-invasive diagnostic modalities in the evaluation of large splenic masses and underscores the importance of considering rare benign entities in the differential diagnosis of clinically aggressive splenic pathology. In carefully selected patients with progressive symptoms and inconclusive investigations, splenectomy remains a valuable strategy to achieve diagnostic clarity and symptom control.

References