Exchange transfusion and pre-filter apheresis dilution for hyperviscosity syndrome refractory to conventional therapeutic plasma exchange in a patient with IgA multiple myeloma.

Abstract

Background: Significant serum paraprotein elevation leading to hyperviscosity syndrome (HVS) is a rare but serious medical complication that has been well documented in patients with hematological malignancies, including multiple myeloma and Waldenström's macroglobulinemia. This condition can result in severe neurological, renal, and cardiac complications. Standard management of symptomatic HVS includes therapeutic apheresis and the prompt initiation of chemotherapy. Although usually successful, apheresis failure due to extreme hyperviscosity has been documented.

Case report: A 73-year-old Caucasian male with neurological symptoms of HVS secondary to IgA monoclonal gammopathy was transferred to our institution for urgent therapeutic plasma exchange (TPE) and HVS management. Due to severe hyperviscosity, it was impossible to obtain blood analysis, and several attempts at standard TPE failed due to clogging in the apheresis tubing. In this life-threatening situation, manual blood exchanges were successfully performed, followed by pre-filter apheresis dilution. This unconventional approach proved to be effective, allowing subsequent laboratory analysis and the continuation of conventional TPE procedures. The patient remained hemodynamically stable throughout the procedure and was subsequently started on definitive chemotherapy treatment.

Conclusion: This case highlights the importance of prompt recognition of HVS in patients presenting with hematological malignancies. The use of manual exchange transfusion, in conjunction with pre-filter dilution, enabled the successful management of a patient with severe HVS in whom conventional TPE was not possible, illustrating an effective alternative approach in urgent hematological care. It also emphasizes the importance of initiating systemic treatment rapidly for the underlying hematological condition to ensure sustained improvement and prevent recurrence of HVS.