The Effect of Phlebotomy Therapy in Porphyria Cutanea Tarda

Abstract

Abstract. Porphyrin excretion and clinical signs have been studied in 21 patients with porphyria cutanea tarda treated by phlebotomy and in a control series of 12 patients. Phlebotomy was performed until signs of depleted iron stores appeared. Clinical and biochemical remission occurred in every phlebotomy‐treated patient. In 18 patients who were bled frequently (usually 0.5 1 per week) and who were not given iron supplements in conjunction with phlebotomy, the urinary porphyrin excretion decreased in all to a level of 1 mg per day or less within 1/2 year. Remission ensued in patients with iron overload as well as in patients with quantitatively normal iron stores and in patients abstaining from alcohol as well as in patients with a persistent abuse of alcohol. A decrease in porphyrin excretion comparable to that of phlebotomy‐treated patients occurred in only two out of the 12 control patients. There was a significant relationship between the period required until iron stores were depleted and until the urinary porphyrin excretion fell to 1 mg per day. In one patient given iron supplement for 6 1/2 months in conjunction with phlebotomy, more blood had to be removed to obtain a remission than in any other patient, and this patient had the longest treatment (16 months). Thus the results have shown that phlebotomy therapy is effective in porphyria cutanea tarda. It seems probable that the effect is mediated through the phlebotomy‐induced reduction of iron stores.

Porphyria Cutanea Tarda: Effects and Risk Factors for Hepatotoxicity from High-dose Chloroquine Treatment

“…Half of the recurrences occurred within 3 years after treatment. Similar results have been reported after phlebotomy treatment (2,7). All patients having recurrence after chloroquine treatment had at least one known risk factor for overt PCT disease.…”

“…results from reduced activity of the enzyme uroporphyrinogen decarboxylase (UROD) (I). Three diflerent main therapeutic modalities are currently used: repeated phlebotomies; low-dose chloroquine; or a combination of both (2)(3)(4)(5)(6)(7). High-dose chloroquine has not been widely used in spite of positive reports about its etTect (8)(9)(10)(11).…”

A porphomethene inhibitor of uroporphyrinogen decarboxylase causes porphyria cutanea tarda

“…In both types of PCT, the clinical and biochemical phenotypes are identical. Hepatic iron overload is an almost universal finding, and depletion of hepatic iron stores by repetitive phlebotomy corrects the clinical phenotype (3,4). One or more of the same factors associated with expression of F-PCT are present in almost all sporadic cases.…”

Cutaneous porphyrias part II: treatment strategies

“…General phlebotomy guidelines suggest the removal of 7 ml of blood per kg body weight, not to exceed 550 ml in one session . Phlebotomy may be performed once or twice weekly until serum levels of ferritin reach the lower limit of normal and then once biweekly or monthly as dictated by pretreatment laboratory values and maintenance of a hemoglobin level appropriate for the patient . Phlebotomies are continued until 24‐hour porphyrin excretion falls to normal levels (<500 nmol), which usually occurs within 9–12 months …”