Plasmapheresis Combined with Somatostatin is a Successful Treatment of Porphyrias

Abstract

During acute attacks of hepatic porphyria, levels of polypeptides, vasoactive intestinal peptides, neurotensin, substance P, pancreatic polypeptide, gastrin releasing polypeptide, gastrin, and motilin increased in the circulation while the clinical symptoms were evident. However, somatostatin decrease was not detected. Somatostatin belongs to a group of regulatory peptides that antagonize the action of endogenous steroid hormones, and decreasing their bioavailability decreases the rate of synthesis of delta-aminolevulinate synthase, alpha-aminolevunilic acid (ALA), and polypeptides. Plasma exchange was conducted in courses for 2 consecutive days every 28 days (total of 6 courses), removing more than 100% of the patient plasma each time. Between the 2 courses of plasmapheresis, subcutaneous injections of somatostatin (100-500 mcg) were administered. A lasting disappearance of pain and complete remission were obtained in all 7 patients treated. Plasmapheresis combined with somatostatin may be considered as a treatment of porphyria exacerbation.

The Management of Motor Neuropathy With Plasmapheresis in a Patient With Acute Porphyria: A Case Report

“…There is also documented evidence that the levels of peptides increase during acute attacks of porphyrias [11]. Combination therapy of somatostatin and plasmapheresis has proven to be beneficial in these cases [12]. Plasmapheresis certainly plays an important role in the management of chronic neuropathy of acute porphyrias.…”