Plasmapheresis Combined with Somatostatinis a Successful Treatment of Porphyrias(Symposium 3: A New Indication and Innovation of Plasma Therapy)

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During acute attacks of hepatic porphyria, levels of polypeptides, vasoactive intestinal peptides, neurotensin, substance P, pancreatic polypeptide, gastrin-releasing polypeptide, gastrin and motilin increase in the circulation while the clinical symptoms are evident in acute porphyria attacks. However, no somatostatin decrease is detected. Somatostatin belongs to a group of regulatory peptides, which antagonizes the action of endogenous steroid hormones and by decreasing their bioavailability it decreases the rate of synthesis of S-aminolevulinate synthase (ALA) and PPS. Plasma exchange, conducted in courses for 2 consecutive days every 28 days (total 6 courses) removed more than 100% of patient plasma each time. Between the two courses of plasmapheresis, subcutaneous injections of somatostatin (100-500 mcg) were administered. A durable disappearance of pain and complete remission were obtained in all 7 patients treated. Plasmapheresis combined with somatostatin may be considered as a treatment in porphyria exacerbation.
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