Plasmapheresis combined with somatostatin is a successful treatment of porphyrias.
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.
Medenica
R
R
International Immuno-Biology Research Laboratory, Hilton Head Island, South Carolina, USA.
Lazovic
G
G
Long
P
P
Corbitt
W
W
Powell
D
D
D000328:Adult; D005260:Female; D006727:Hormone Antagonists / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D011164:Porphyrias / Q000628:therapy*; D013004:Somatostatin / Q000627:therapeutic use*
D006727:Hormone Antagonists; D013004:Somatostatin