Reversal of refractory systemic lupus erythematosus-associated immune thrombotic thrombocytopenic purpura and cerebritis via optimized lymphoplasmapheresis: a case report and literature review.

BACKGROUND: Systemic lupus erythematosus (SLE) complicated by immune thrombotic thrombocytopenic purpura (iTTP) and lupus cerebritis is a severe clinical event. Standard therapeutic plasma exchange (TPE) frequently fails in this context due to a "rebound effect", as it clears circulating antibodies but spares the intravascular reservoir of autoreactive lymphocytes. CASE PRESENTATION: A 30-year-old male presented with refractory SLE, severe iTTP (platelet count: 5.00 × 10^9/L; ADAMTS13 activity: 1.49%), and imaging-confirmed lupus cerebritis. The patient underwent a unique "A-B-A" treatment trajectory. While five sessions of standard TPE at an outside facility did not fully halt the hyper-regenerative hemolysis and CNS progression, the implementation of an optimized lymphoplasmapheresis (LPE) regimen at our center induced rapid hematological remission, progressive radiological resolution of brain lesions, and the alleviation of immune-mediated renal injury, culminating in restored renal function and decreased proteinuria. MECHANISTIC INSIGHTS: The optimized LPE protocol utilized a novel dual-parameter modulation (synchronizing blood drawing speed and hematocrit) to precisely extract the pathogenic lymphocyte-rich buffy coat without exacerbating anemia. Paired quantitative analysis of the patient's peripheral blood and LPE effluent provided direct evidence of substantial cytokine (e.g., IL-10, IL-8, TNF-α) clearance. This facilitated the rapid seroconversion of antinuclear and anti-dsDNA antibodies. Furthermore, this targeted cellular depletion reduced the required allogeneic replacement plasma volume by up to 50% compared with standard TPE, conserving blood bank resources. CONCLUSION: Optimized LPE may interrupt the autoimmune cascade by simultaneously reducing circulating humoral mediators and lymphocyte-rich cellular components. This single-case observation supports its potential as a resource-sparing rescue strategy for refractory autoimmune microangiopathy, although further validation is required.