Archil Jaliashvili: Novel Rescue in Hyperhaemolysis Syndrome of Sickle Cell Anaemia
Archil Jaliashvili, Co-owner, General Manager of SkinAI, Co-Chief Executive Officer at GeoBio Pharm, shared on LinkedIn:
‘‘Novel Rescue in Hyperhaemolysis Syndrome of Sickle Cell Anaemia (SCA)
Hyperhaemolysis syndrome (HHS) remains one of the most fatal complicatios of transfusion in SCA, with rapid destruction of both transfused and autologous RBCs.
Conventional therapies (steroids, IVIG, rituximab, eculizumab) often fail, leaving clinicians with limited options.
Case (Epstein and Hadley, J Clin Pharm Ther)
• 19-year-old male with SCA developed HHS post partial exchange transfusion.
• Hb fell to 4.0 g/dL despite methylprednisolone, darbepoetin, rituximab.
• Severe hemoglobinuria, LDH 7,120 U/L, Coombs negative.
New oxygenation therapeutic Breakthrough:
Hemopure (BHOC – our new gen bio analog)
• Single infusion restored oxygen delivery instantly.
• Clinically, the patient regained energy and responsiveness despite critical Hb.
• Effect lasted ~5 hours.
Bortezomib (26S proteasome inhibitor)
• 1.3 mg/m² SC q72h × 4 doses.
• Downregulated NF-κB–driven macrophage activation and hemolysis.
• Hb rose from 4.0 → 7.0 g/dL, reticulocyte count >20%.
Outcome: Patient stabilized and discharged. No alloantibodies detected later.
Why important:
•Hemopure has demonstrated value as a bridge oxygen carrier when hemoglobin (Hb) levels are critically low.
• Bortezomib represents a novel immune-modulating option in transfusion-refractory hyperhemolytic syndromes (HHS).
• This case supports the expansion of HBOCs in clinical emergencies and the initiation of clinical trials of proteasome inhibitors in hemolytic syndromes.
About future possibility:
Our next-generation bio-similar BHOC can serve as a bridge to recovery.
We believe it can transform the approach to Sickle Cell Anemia (SCA) treatment by providing supportive, low-volume infusion therapy before HbS levels become critical.
Much like insulin regulates blood sugar and HbA1c levels, BHOC can help support and control HbS polymerization level, stabilizing oxygen delivery and patient condition.
Contact us for collaboration or discussion about clinical applications and partnerships.”
Article: Successful management of potentially fatal hyperhaemolysis syndrome of sickle cell anaemia with a regimen including bortezomib and Hemopure
Authors: Stacy S. Epstein, Terence J. Hadley
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