PORPHYRIA From: Druid71@aol.com I have recently seen a 50yo Hispanic male who is on chronic hemodialysis because of diabetic nephropathy and who has severe ASHD with angina. Five years ago, prior to the development of his chronic renal failure and angina, the patient underwent a series of therapeutic phlebotomies for PCT. After this treatment and the successful treatment of his alcoholism, the PCT went into a prolonged remission. Unfortunately, in the past couple months he has noted the return of blistering on his face and upper extremities, marked skin fragility, and hyperpigmentation. His plasma uroporphyrins are 39X normal. His HCT = 35, his serum Fe is normal and his ferritin level is slightly elevated. After much discussion with his cardiologist and nephrologist we have elected to treat his PCT by cautiously lowering his dose of epogen to decrease his rate of hematopoesis while minimizing the risk of exacerbating his angina. I would welcome hearing the advice and experiences of any dermatologists who have treated patients in similar circumstances. Does anyone know if low-dose chloroquin would be a reasonble alternative? Is the Chloroquin-porphyrin complex dialysible? ============ From: "L.J. Gregg" <73477.361@compuserve.com> I have used minute doses of hydroxy chlorquine (1/4 tab twice weekly with success in otherwise normal but already anemic pts. I do not know if the compound is dialysable but would guess it is since it crosses the glomerular membrane in individuals with normal kidneys. ============ From: A Huntley Serum porphyrin levels can be rapidly lowered to normal by administration of activated charcoal by mouth, 1/2 oz BID. The serum level will return to normal in 2 days. Charcoal is odorless and tasteless, but the grittiness may be a little unpleasant. Our patients recommend taking the charcoal in juice, especially grape juice. The reason charcoal works is the level of porphyrins in the enterohepatic circulation is about 10 times that found in serum. Charcoal absorbs the prophyrins and they are then eliminated in the feces. Removing the excess porphyrins doesn't alleviate the overproduction, so for long term treatment, bloodletting is still treatment of choice for most patients. However, there are occasional patients who are already anemic, or for whom blood letting is otherwise impractical, and oral charcoal is helpful. If you are considering doing this for a long period of time, your patient may need some vitamin supplementation, otherwise it seems to be safe. Use activated charcoal powder and not the syrup that is used in emergency rooms. =========== From: Yelva@aol.com We managed such a case by depleting the iron with phlebotomy and continuing the epogen. Maybe this will work for you, too.