Plasma Exchange in ANCA‐Associated Vasculitis With Kidney Involvement: Differential Outcomes by Kidney Function in a Colombian CohortEscorcia‐García, Clara; Restrepo‐Escobar, Mauricio; Aguirre, Lina; Carvajal, José Nelson; Rodelo‐Ceballos, Joaquín
doi: 10.1002/jca.70099pmid: 41731336
ANCA‐associated vasculitis (AAV) frequently involves the kidneys and may progress to end‐stage kidney disease (ESKD). The benefit of plasma exchange (PLEX) in this setting remains uncertain. We conducted a retrospective cohort study of 163 patients with AAV and serum creatinine ≥ 1.5 mg/dL from two tertiary centers in Colombia, comparing PLEX versus no PLEX. The primary outcome was a composite of all‐cause mortality or ESKD. Overall, PLEX was not associated with a reduction in the composite outcome (adjusted hazard ratio [HR] 1.04, 95% CI 0.61–1.76). Among patients with baseline creatinine > 5.7 mg/dL, PLEX was associated with a lower risk of ESKD (adjusted HR 0.38, 95% CI 0.14–0.97) and a borderline reduction in the composite outcome (HR 0.45, p = 0.05), with no benefit in those with lower creatinine levels. PLEX was associated with increased in‐hospital infections (adjusted risk ratio 4.66, p = 0.001). These findings suggest a potential role for PLEX in selected patients with severe kidney dysfunction.
Efficacy of Combined Plasma Exchange With Plasma Adsorption/Hemoperfusion Artificial Liver Support System (ALSS) in Treating Acute‐on‐Chronic Liver Failure due to Hepatitis BLi, Dingchun; Liu, Ye; Zhou, Ju; Duan, Zhiwen; Yang, Ruidong; Chao, Chunmei; Li, Wu
doi: 10.1002/jca.70058pmid: 41673904
Acute‐on‐chronic liver failure (ACLF) is the most prevalent type of liver failure in Asia, with hepatitis B virus (HBV) infection being the primary cause in our country. This study aimed to evaluate the effectiveness of the artificial liver support system (ALSS) in treating HBV‐induced ACLF (HBV‐ACLF) and assess the efficacy and safety of a combined ALSS model with small‐volume plasma. A retrospective analysis compared the 4‐week and 12‐week survival rates of patients receiving comprehensive medical treatment versus ALSS treatment and identified factors associated with mortality. Patients undergoing the combined ALSS model (plasma exchange + double plasma adsorption system/hemoperfusion, PE + DPMAS/HP) were categorized into small‐volume plasma and half‐volume plasma groups. Changes in liver function, renal function, coagulation function, and blood ammonia levels following PE + DPMAS/HP treatment were analyzed. Mortality rates at 4 and 12 weeks were compared between the two groups, and factors influencing 4‐week and 12‐week mortality in HBV‐ACLF patients were examined. The results indicated no significant difference in 4‐week mortality between the ALSS and medical treatment groups; however, the ALSS group showed a significantly improved 12‐week survival rate. Both small‐volume and half‐volume plasma ALSS models effectively eliminated bilirubin, bile acids, urea, and creatinine. No significant differences were observed in 4‐week and 12‐week mortality between the small‐volume and half‐volume plasma groups, regardless of the severity of HBV‐ACLF (early, middle, or late stages). COX proportional hazard regression analysis revealed that the amount of plasma used in ALSS treatment was not a significant factor influencing HBV‐ACLF prognosis. The combined ALSS model with reduced plasma usage may help alleviate the limited plasma supply and merits further application and promotion.
Safety and Efficacy of Cyclophosphamide With Dual Mecapegfilgrastim and On‐Demand Plerixafor for Salvage Mobilization in Patients With Initial Mobilization Failure: A Retrospective CohortHuang, Fang; Cui, Ke; Zhao, Jie; Li, Zhichao; Qin, Yun; Hao, Siguo; Wan, Jiangbo
doi: 10.1002/jca.70091pmid: 41603596
Initial hematopoietic stem cell (HSC) mobilization failure remains a critical barrier to autologous stem cell transplantation (ASCT) in patients with lymphoma or multiple myeloma (MM). This retrospective cohort study (August 2015 to March 2023) evaluated the efficacy, safety, and cost‐effectiveness of a novel salvage regimen—split‐dose cyclophosphamide (CTX: 1.5 g/m2 for 2 days) + Mecapegfilgrastim (PEG‐rhG‐CSF) + on‐demand plerixafor (PXF)—compared to conventional strategies (CTX + granulocyte colony‐stimulating factor [G‐CSF] or G‐CSF + PXF) in 118 patients with initial mobilization failure. Patients were stratified into three groups: CTX + PEG‐rhG‐CSF + on‐demand PXF (n = 46), CTX + G‐CSF (n = 34), and G‐CSF + PXF (n = 38). The CTX + PEG‐rhG‐CSF + on‐demand PXF group achieved superior mobilization efficacy: median CD34+ cell yield was 9.2 × 106/kg, which is significantly higher than that of CTX + G‐CSF group (4.1 × 106/kg; p < 0.05) and G‐CSF + PXF group (4.6 × 106/kg; p < 0.05). Rates of achieving the thresholds of ≥ 2 × 106 CD34+ cells/kg and ≥ 5 × 106 CD34+ cells/kg were significantly higher in the CTX + PEG‐rhG‐CSF + on‐demand PXF group. This superior mobilization efficacy also brought fewer apheresis sessions: only 15.2% of patients in the CTX + PEG‐rhG‐CSF + on‐demand PXF cohort required ≥ 2 sessions, compared to 70.5% in CTX + G‐CSF group and 57.9% in G‐CSF + PXF group (p < 0.001). Safety profiles were comparable across cohorts: febrile neutropenia occurred in 13% of patients in CTX + PEG‐rhG‐CSF + on‐demand PXF group (vs. 26.5% in CTX + G‐CSF; p = 0.33). Severe neutropenia (Grade 3–5) was common in CTX‐containing groups (91.3% vs. 90.2%; p = 0.88) but mostly being transient, with no treatment‐related mortality. Neutrophil engraftment times were similar (median 10–11 days; p = 0.406) in all groups. Cost analysis revealed that the CTX + PEG‐rhG‐CSF + on‐demand PXF regimen was more cost‐effective than G‐CSF + PXF regimen (5511 vs. 8761; p < 0.05) but more expensive than CTX + G‐CSF regimen ($3012; p < 0.05). In conclusion, CTX + PEG‐rhG‐CSF + on‐demand PXF is a highly effective salvage mobilization strategy for patients with initial HSC mobilization failure, yielding higher CD34+ cell counts with fewer aphereses and acceptable safety. With balanced advantages in efficacy, safety, and cost‐effectiveness, the CTX + PEG‐rhG‐CSF + on‐demand PXF regimen can be a preferred salvage option for ASCT candidates with prior mobilization failure.
Cerebral Fat Embolism Syndrome in Homozygous Sickle Cell Disease Treated With Therapeutic Plasma Exchange and Simple TransfusionsVajdic, Tyler; Jacob, Reuben; Chonat, Satheesh; Sheehan, Vivien
doi: 10.1002/jca.70105pmid: 41735820
Fat embolism syndrome (FES) is a rare, life‐threatening complication of sickle cell disease (SCD) associated with high mortality and neurologic morbidity. We report a 17‐year‐old male with homozygous SCD (HbSS) who developed cerebral FES following vaso‐occlusive and acute chest syndrome (ACS). Diagnosis was supported by clinical findings and characteristic punctate lesions on brain MRI. He received multiple simple red blood cell transfusions and four sessions of therapeutic plasma exchange (TPE), followed by 1 year of chronic transfusions. ADAMTS13 activity was mildly reduced but inconsistent with thrombotic thrombocytopenic purpura. The patient experienced rapid hematologic improvement and full neurologic recovery. Including this case, only six reports describe cerebral FES in HbSS individuals, predominantly young males. Outcomes appear most favorable with early combined transfusion and TPE. This case highlights the diagnostic challenges and supports early multimodal therapy for cerebral FES in SCD while underscoring the need for multicenter studies to define optimal management.
When Strong Recommendations Rest on Weak Evidence: Lessons From Therapeutic Apheresis GuidelinesJacobs, Jeremy W.; Booth, Garrett S.; Adkins, Brian D.; Costa, Victoria; Raza, Sheharyar; Park, Yara A.; Schwartz, Joseph Yossi; Bloch, Evan M.
doi: 10.1002/jca.70098pmid: 41711290
The American Society for Apheresis (ASFA) guidelines serve as a global standard for therapeutic apheresis practice. However, our analysis of the 2023 guidelines reveals discordance between the strength of recommendation and the quality of evidence. Among 166 indications, one‐third carry strong recommendations, yet only 8% are supported by high‐quality evidence. Over half (55%) are informed by low‐ or very‐low quality evidence. This mismatch is most pronounced for Category I indications, where apheresis is considered first‐line therapy: nearly one‐third are based on low‐quality data, yet 89% receive strong recommendations. Weak evidence is nine times more likely to prompt a strong recommendation for Category I versus Category III indications. This misalignment risks overutilization of apheresis, introduces ethical hurdles for clinical trials by diminishing equipoise, and may mislead patient expectations during informed consent. We advocate for greater transparency by stating the rationale underlying strong recommendations despite low‐quality evidence, acknowledgment of uncertainty where applicable, and suggested research to strengthen the evidence.
Twenty‐Four Cases of Severe ANCA‐Associated Vasculitis Treated With Plasma Exchange: A Clinical Analysis and Long‐Term Follow‐UpChen, Zhou; Li, Wenhao; Guo, Zhiyong; Zhu, Chao
doi: 10.1002/jca.70087pmid: 41470023
Advances in the understanding of anti‐neutrophil cytoplasmic antibodies (ANCA)‐associated vasculitis (AAV) pathophysiology have expanded the application of plasma exchange (PE) in severe cases; however, robust evidence on its efficacy, safety, and long‐term outcomes remains scarce. This retrospective study evaluated the short‐term efficacy, safety, and long‐term prognosis of PE in patients with severe AAV. A total of 24 patients receiving PE alongside standard induction therapy (glucocorticoids plus cyclophosphamide or rituximab) were analyzed. Clinical and laboratory parameters—including ANCA titers, Birmingham Vasculitis Activity Score (BVAS), C‐reactive protein (CRP), and hemoglobin (Hb)—were assessed before and after treatment, together with adverse events and survival. The cohort comprised 10 males and 14 females, mean age 62.5 ± 16.1 years (range 29–84). PE was associated with significant reductions in ANCA levels, BVAS, and CRP, alongside increased Hb. Over long‐term follow‐up, mortality rose progressively, mainly attributable to severe infections, respiratory failure, and heart failure. In conclusion, PE exhibits short‐term effectiveness and a tolerable safety profile in severe AAV, yet long‐term survival outcomes warrant further attention.
Red Blood Cell Depletion in Patients With Hyperferritinemia: Comparison of Two Apheresis SystemsNeureiter, Heidrun; Schroeder, Nadja; Kartal, Orkan; Laner‐Plamberger, Sandra; Lauth, Wanda; Rohde, Eva; Grabmer, Christoph
doi: 10.1002/jca.70097pmid: 41724909
Red blood cell (RBC) apheresis is, alongside phlebotomy, a standard treatment for iron overload in hereditary hemochromatosis (HH). We compared the serum ferritin (SF) reduction, process parameters, duration, and side effects of two apheresis systems: Spectra Optia apheresis system (Optia) and Alyx apheresis collection system (Alyx). Forty‐three patients were RBC depleted with one of the two separators, Optia or Alyx. In total, 186 procedures were performed. The main diagnoses were HH (n = 20) and dysmetabolic iron overload syndrome (DIOS) (n = 21). Around two thirds of the procedures were done with Optia (n = 143) and one third with Alyx (n = 43). A mean volume of 405 and 442 mL of RBCs was withdrawn per single treatment with the Optia and Alyx, respectively. The procedure took 12 min (Optia) versus 26 min (Alyx) with a hematocrit (Hct) reduction of 5% versus 7.5% (p < 0.001). The SF reduction 3 weeks after RBC depletion was not significantly different between the two systems. The amount of anticoagulant used with the Optia was almost half of what was used with the Alyx (63 mL compared to 123 mL). There were no significant adverse events. The advantages of the Alyx include the lower cost and the easy portability. The Optia, on the other hand, has a shorter procedure time, a smaller extracorporeal volume, a lower anticoagulant consumption, a lower rate of complications, and allows a precise Hct adjustment.
Determining the Optimal Albumin Concentration in the Replacement Fluid for Double Filtration Plasmapheresis in Autoimmune Neurological Disorders: A Preliminary StudyLoke, Kit Mun; Hiew, Fu Liong
doi: 10.1002/jca.70101pmid: 41733190
Pre‐set fixed‐dose albumin replacement fluid for double filtration plasmapheresis (DFPP) can result in progressive albumin deficit, along with other untargeted protein, leading to loss of osmotic pressure, and subsequently hypotension. This can occur in up to 6% of DFPP procedures. In this study, we aim to determine the serum albumin deficit with fixed‐dose replacement regime and estimate the required practical supplementary regime for the discrepancy. We compared the change in serum albumin level among patients treated with DFPP given fixed‐dose 10% plasma replacement volume with albumin 5% concentration versus patients given additional replacement with albumin 20% concentration. Seventy‐seven DPFF procedures were performed in 16 patients with various neurological disorders. In average, each patient received 4.8 DFPP procedures (Range 3–7) per treatment. Eight patients were given fixed‐dose albumin replacement and remaining 8 had additional albumin 20% replacement. Twenty‐one (27.3%) procedures had additional replacement. The average change in serum albumin post‐DFPP procedures was a reduction of 2.4 g/L (SD 3.0) with fixed‐dose replacement and an increment of 0.1 g/L (SD 2.8) for procedures with additional human albumin 20% replacement (p = 0.003). In average, additional 78.6 mL (50–500 mL in up to 5 divided replacements) of albumin 20% replacement given to patients with post‐DFPP serum albumin lower than 34 g/L, or in those with post‐DFPP negative serum albumin balance depending on severity effectively reduces post‐DFPP albumin deficit. In conclusion, fixed‐dose albumin replacement for DFPP results in progressive albumin deficits with successive procedures. Addition albumin supplementation may be required.