Abstract

Background and Clinical Significance: Hyperviscosity syndrome (HVS) is a rare complication of primary Sjögren’s syndrome (pSS). While therapeutic plasma exchange (TPE) is the standard treatment to clear pathogenic immunoglobulins, its execution can trigger severe, atypical systemic risks. Case Presentation: A 60-year-old woman with pSS and extreme polyclonal hypergammaglobulinemia (total protein 100 g/L, IgM 41 g/L) presented with an acute hyperviscosity crisis, causing retinopathy, neurological deficits, and skin ischemia. Emergency TPE with 5% albumin replacement successfully reduced IgM by ~90% (to 6.39 g/L), resolving HVS symptoms. However, 20 min post-procedure, the patient suffered sudden hemodynamic collapse (BP 50/30 mmHg) and developed multiple massive, expanding soft-tissue hematomas. Laboratory tests revealed a coagulopathy consistent with plasma protein depletion following therapeutic plasma exchange, characterized by severe hypofibrinogenemia (1.35 g/L) and a 50% reduction in total serum protein. TPE was permanently discontinued. The patient was successfully stabilized using aggressive fluid resuscitation, vasopressors, and fresh frozen plasma (FFP) transfusions, followed by maintenance therapy with rituximab. Conclusions: In conclusion, clinicians should remain vigilant that severe hyperviscosity syndrome can be driven by a polyclonal increase in immunoglobulins rather than just monoclonal entities; furthermore, managing this condition requires careful balancing of TPE efficacy against its potential to trigger profound depletion coagulopathy.

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