Transfusion therapy in a patient with erythropoietic protoporphyria

Bechtel, M. A.

doi:10.1001/archderm.117.2.99

Cited by 12 publications

(8 citation statements)

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Section: Therapeutic Optionsmentioning

confidence: 99%

“…Since excess protoporphyrin comes mainly from the bone marrow in patients with protoporphyria, transfusion therapy has been used to suppress erythropoiesis (48,49). Patients have received transfusions with red blood cells, and subsequently have had less photosensitivity as well as a decline in red blood cell protoporphyrin levels (48,49). The latter has resulted in part from the dilutional effect of cells containing normal levels of protoporphyrin, but this could not account for most of the change observed.…”

Section: Therapeutic Optionsmentioning

confidence: 99%

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The liver in protoporphyria

Bloomer

1988

Hepatology

106

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In 1961, Magnus and coworkers (1) reported the case of a 35-year-old man who for 27 years had had intense itching and swelling of his skin after exposure to sunlight. Urinary excretion of porphyrins and porphyrin precursors was normal, but the red blood cells and stool contained increased amounts of protoporphyrin. The authors proposed the name erythropoietic protoporphyria for this "hitherto undescribed erythropoietic condition."t Lancet is a widely distributed journal, and several gastroenterologists/hepatologists probably saw the title of the article. Some may have even read the summary, only to breathe a sigh of relief that this was not a type of porphyria which would enter into the differential diagnosis of obscure abdominal pain. Moreover, the liver was not the site of the metabolic disturbance, and the disorder would be classified as erythropoietic and not hepatic.Dark clouds soon began to appear on the horizon, however. In 1963, Porter and Lowe (2) reported a 6-yearold male with clinical and biochemical features of protoporphyria who also had hepatosplenomegaly and abnormal liver biochemistries. At the time of splenectomy, he was found to have a cirrhotic liver. Two years later, Cripps and Scheuer (3) described the histological findings in liver biopsy specimens from five patients with protoporphyria. Four of the biopsies showed focal deposits of pigment containing protoporphyrin, and portal fibrosis was present in two. The rain finally came in 1968, when Barnes and coworkers (4) described a 42-year-old man with a long history of photosensitivity who developed jaundice and hepatomegaly, followed by a rapid downhill course and death (4). Postmortem examination indicated that the patient had protoporphyria. DESCRIPTION OF LIVER DISEASEThe incidence and prevalence of protoporphyria have not been precisely determined for any population group.It appears to be a relatively common type of porphyria, perhaps second only to porphyria cutanea tarda in frequency. It occurs in all racial groups and has no sex predominance.

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Section: Therapeutic Optionsmentioning

confidence: 99%

Section: Therapeutic Optionsmentioning

confidence: 99%

The liver in protoporphyria

Bloomer

1988

Hepatology

106

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“…In theory, blood transfusion should suppress hematopoiesis and thereby reduce the erythropoietic contribution to excess protoporphyrin formation. On this basis, transfusion therapy was recently used in an 8-year-old boy with protoporphyria (25). The patient had less photosensitivity, and the erythrocyte protoporphyrin level decreased from 592 to 263 pg per dl.…”

Section: Discussionmentioning

confidence: 99%

Effect of hematin administration to patients with protoporphyria and liver disease

Bloomer¹,

Pierach²

1982

Hepatology

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Hepatic damage in protoporphyria appears to be caused by a toxic effect of excess protoporphyrin. Therapy which reduces the formation of excess protoporphyrin may, therefore, be helpful. We examined the effects of hematin administered i.v. to two patients with protoporphyria and decompensated cirrhosis. Neither patient had side effects from the compound or manifested signs of toxicity. The vascular disappearance of hematin in one patient was similar to that in patients with porphyria who do not have structural liver disease. In both patients, biochemical changes occurred that were compatible with a reduced rate of protoporphyrin formation. Thus, hematin administration may be useful in treating patients with protoporphyria who develop liver disease.

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“…Hematin infusion (10,16) and hypertransfusion (17) may dimninish protoporphyrin overproduction, but are not suitable for chronic use in the general population of patients with protoporphyria.…”

Section: Introductionmentioning

confidence: 99%

Protoporphyrin hepatopathy. Effects of cholic acid ingestion in murine griseofulvin-induced protoporphyria.

Poh–Fitzpatrick¹,

Sklar²,

Goldsman³

et al. 1983

J. Clin. Invest.

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A B S T R A C T Short-term effects of cholic acid ingestion on hepatic accumulation, fecal excretion, and blood levels of protoporphyrin were studied in vivo in griseofulvin-induced protoporphyric mice. Experimental mice that received feed with 2% griseofulvin and 0.5% cholic acid were compared with control mice that received feed with 2% griseofulvin for 4 wk. Five mice from each group were assessed each week for liver and blood porphyrin levels. Fecal protoporphyrin was compared weekly in the total pooled output of each population. Mean protoporphyrin levels were significantly lower for liver (P < 0.0001), erythrocytes (P < 0.05), and plasma (P < 0.05), and higher for feces (P < 0.001) for the mice that were fed cholic acid. Microscopic protoporphyrin deposits, inflammation, necrosis, and dysplasia were more severe in livers of control mice. A second experimental design compared four regimens in the feed given to all mice after 1-wk induction with 2% griseofulvin: (a) 0.5% cholic acid, (b) no adulterant, (c) 2% griseofulvin and 0.5% cholic acid, and (d) 2% griseofulvin. No difference in protoporphyrin removal from livers of mice in groups 1 and 2 was observed after 1 and 2 wk of these regimens. The apparent reduction in hepatic protoporphyrin content in mice of group 3 as compared with group 4 at weeks 2 and 3 was not significant at P < 0.05.These data suggest that in selected circumstances, Drs. Sklar and Goldsman participated in these studies during student research elective periods at Columbia University College of Physicians and Surgeons. Address all correspondence to Dr. Poh-Fitzpatrick.

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Transfusion therapy in a patient with erythropoietic protoporphyria

Abstract: An 8-year-old boy with erythropoietic protoporphyria had a marked decrease in photosensitivity symptoms after several transfusions with washed, packed RBCs. The reduction in photosensitivity was associated with a decline in free erythrocyte protoporphyrin levels.

Cited by 12 publications

References 0 publications

The liver in protoporphyria

The liver in protoporphyria

Effect of hematin administration to patients with protoporphyria and liver disease

Protoporphyrin hepatopathy. Effects of cholic acid ingestion in murine griseofulvin-induced protoporphyria.

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