Pathophysiology and management of hyperviscosity syndrome as the first oncological emergency in Waldenström’s Macroglobulinemia: A case report and literature review

Background: Waldenstrom macroglobulinemia (WM) is an exceptionally rare B-cell lymphoma, accounting for less than 2% of all non-Hodgkin lymphomas. Its unique features, including anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and the occurrence of hyperviscosity, make it a significant focus in the medical community. The extreme rarity of this condition underscores its unparalleled importance and the need for further research. Hyperviscosity syndrome (HVS) is a critical oncological emergency in this disease, leading to increased plasma viscosity and reduced blood flow. Case presentation: We present a case of a 56-year-old man with Waldenström's macroglobulinemia who, following induction therapy, developed a rare hyperviscosity syndrome. Through a combination of plasma exchange therapy and a maintenance program involving Ibrutinib and Lenalidomide, we successfully reduced the patient’s serum viscosity. This successful outcome not only demonstrates the potential for effective treatment strategies in such cases but also instills confidence in the effectiveness of the treatment, offering a beacon of hope for similar cases in the future. Conclusion: The importance of choosing a suitable treatment strategy for Waldenström macroglobulinemia patients is highlighted by the fact that HVS is one of the first oncological emergencies that require timely treatment. This underscores the crucial role of medical professionals, particularly hematologists and oncologists, in promptly diagnosing and treating such cases. The urgency and significance of the issue and the need for swift action are emphasized, as HVS is a condition that cannot be delayed in its management.