1/5. porphyria cutanea tarda in human immunodeficiency virus-seropositive men: case report and literature review.porphyria cutanea tarda (PCT) is a disorder of heme synthesis characterized by (a) a diminished activity of uroporphyrinogen decarboxylase biochemically and (b) cutaneous lesions secondary to a delayed type of photosensitivity clinically. A human immunodeficiency virus (hiv)-infected patient with PCT is reported and the world literature is reviewed. To date, 17 hiv-seropositive men with PCT have been described. The initial appearance of PCT occurred before or concurrent with the diagnosis of hiv infection in 71% of these individuals (12 men). The median age at onset of PCT was 36 years (range of 20 to 69 years); the median age for the detection of hiv infection was 35 years (range of less than 20 to 71 years). All of these patients had elevated levels of urine porphyrins and blisters on their dorsal hands. Abnormal liver function tests, erosions, hyperpigmentation, hypertrichosis, and skin fragility were also present in some of the men. polycythemia, serologic evidence of increased iron stores, scarring, milia, and sclerodermoid changes were rarely observed. Successful therapeutic approaches for PCT in men with hiv infection included (a) elimination of PCT-precipitating agents, (b) avoidance of sun exposure, and (c) periodic phlebotomy. Multiple hypotheses for an etiologic role of the hiv and/or an hiv-associated infection, directly or indirectly, in the pathogenesis of PCT in hiv-seropositive men have been suggested.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
2/5. Successful treatment of hemodialysis-related porphyria cutanea tarda with deferoxamine.End-stage renal failure and long-term hemodialysis treatment promote the development of genetically conditioned porphyria cutanea tarda (PCT). The clinical manifestation is triggered off by unknown factors coexisting with renal insufficiency and hemodialysis. iron overload is often associated with the disease and is thought to play a key role in its pathogenesis. Iron removal by deferoxamine infusions is regarded as the treatment of choice for patients who cannot undergo repeated phlebotomy procedures and has been successfully used in patients with normal renal function. We report a case of hemodialysis-related PCT and iron overload in whom repeated venesections were contraindicated on account of severe anemia and treatment with deferoxamine led to a striking improvement of symptoms.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
3/5. phlebotomy treatment in porphyria cutanea tarda combined with beta-thalassaemia.In 74 patients with porphyria cutanea tarda, 11 (14.9%) cases of beta-thalassaemia were found. The incidence of beta-thalassaemia in porphyrics is not greater than in non-porphyrics and it seems that beta-thalassaemia is not a factor triggering the development of porphyria cutanea tarda. The phlebotomy treatment is also a convenient method for porphyria cutanea tarda combined with beta-thalassaemia.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
4/5. porphyria cutanea tarda: response to vitamin e. A review and two case reports.porphyria cutanea tarda is a disease characterized by a triad of cutaneous manifestations: "fragile" skin, usually involving the dorsal aspects of the hands, forearms, legs, or feet; mild hyperpigmentation; and hypertrichosis, especially of the face. The condition is due to a metabolic defect of liver function involving heme synthesis, resulting in the formation of abnormal amounts of uroporphyrin, and sometimes, coproporphyrin or both, which can be measured quantitatively in the urine. Present methods of treatment, including repeated phlebotomy, alkalinization, or chloroquine leave much to be desired. Based on recent experimental and clinical reports and on our personal experience with two patients, we suggest a more logical therapeutic approach in the form of large doses of vitamin e, which apparently corrects the metabolic defect causing the disease.- - - - - - - - - - ranking = 1keywords = phlebotomy (Clic here for more details about this article) |
5/5. Large phlebotomy in variegate porphyria.There are no reports on effects of large blood losses in acute hepatic porphyria. In the present study we report the experiences of repeated large therapeutic phlebotomies in a patients with porphyria cutanea tarda coexisting with variegate porphyria. Neither a series of 12 phlebotomies, 300 mL each, resulting in a 17% decrease in blood haemoglobin, nor a single 400 mL phlebotomy activated the acute porphyric condition. It is concluded that the increased bone marrow metabolic throughput resulting from blood loss, in acute types of porphyria does not overload the normoblast or leukocyte precursor haem synthetic pathways in a way which will increase porphyrin precursor excretion or trigger acute porphyric symptoms.- - - - - - - - - - ranking = 5keywords = phlebotomy (Clic here for more details about this article) |