Porphyrinämie Ohne Porphyrinurie

Erythropoietic protoporphyria and lead intoxication: the molecular basis for difference in cutaneous photosensitivity. I. Different rates of disappearance of protoporphyrin from the erythrocytes, both in vivo and in vitro.

“…An elevated concentration of protoporphyrin (PP)1 may be extracted with acid organic solvents from the erythrocytes of individuals with the genetic disorder erythropoietic protoporphyria (EPP) (1) and of individuals with chronic lead intoxication (PbI) (2). In EPP the values observed may be up to 100 times greater than normal; in PbI PP concentration in the erythrocytes increases exponentially as the blood Pb level increases and in severe cases it reaches values even greater than in EPP (3).…”

Free Erythrocyte Protoporphyrin 1

“…In Table IV the normal values for free erythrocyte protoporphyrin given by various investigators are compared (3)(4)(5)(19)(20)(21)(22)(23). In these normal subjects no correlation could be found between free erythrocyte protoporphyrin and such factors as the amount of hemoglobin, percentage of reticulocytes, volume of packed erythrocytes, appearance of the blood smear, and age or sex of the subjects.…”

Variegate Porphyria

“…2 Initially, the features of VP were confused with those of porphyria cutanea tarda (PCT), acute intermittent porphyria (AIP), and hereditary coproporphyria (HCP), and a number of terms were subsequently used to describe the disease. These included mixed porphyria, protocoproporphyria, and PCT hereditaria.…”