Porphyria cutanea tarda and hematological diseases

American Journal of Hematology 28:209 (1988)

Letter to the Editor: Porphyria Cutanea Tarda and Hematological Diseases We read with interest the article on the occurrence of porphyria cutanea tarda (PCT) in three patients with AIDS [ 11. We would like to point out the possible development of ‘‘sporadic” PCT in hematological disorders other than AIDS. PCT has been reported, rarely, in idiopathic myelofibrosis (IMF) [2]. Two elderly patients of ours with IMF requiring regular transfusions developed PCT (documented by typical skin lesions and high urinary porphyrin levels) 1 and 4 years after diagnosis and following 20 and 31 units, respectively, of packed red blood cell transfusion [ 3 ] . They had no familial history of photosensitivity. We hypothesize that iron overload could play a major role in transfusion-dependent IMF by reducing hepatic uroporphyrinogen decarboxylase (Uro-d) activity. Indeed, it has been shown that ferrous ions inhibit Uro-d activity in vitro [4].However, since polytransfused patients do not usually develop PCT, other factors (genetic?) also could be thought to play a role, as it is postulated in several multifactorial diseases. In AIDS patients, a genetic anomaly (suggested in Wissel’s case 3 ) andlor coexistent viral or alcoholic damage (as in cases 1 and 2) could enhance the possibility of human im-

0 1988 Alan R. Liss, Inc.

munodeficiency virus (HIV) to cause clinically relevant Uro-d damage.

G. De Rosa L. Catalano C. Selleri M. Nigro A. Camera B. Rotoli Cattedra di Ematologia II Facolta di Medicina e Chirurgia Universita di Napoli REFERENCES 1. Wissel PS, Sordillo P, Anderson KE, Sassa S, Savillo RL, Kappas A: Porphyria cutanea tarda associated with the acquired immune deficiency syndrome. Am J Hematol 25:107, 1987. 2. Fivenson DP, King AJ: Porphyria cutanea tarda in a patient with agnogenic myeloid metaplasia. Arch Dermatol 120538, 1984. 3. De Rosa G, Nigro M, Camera A, Selleri C, Catalano L, Rotoli B: Mielofibrosi e porfiria cutanea tarda. Haemotologica Suppl 3:29, 1987. 4. Mukeji SK, Pimstone NR, Bums M: Dual mechanism of inhibition of rat liver uroporphyrinogen decarboxylase activity by ferrous iron: Its potential role in the genesis of porphyria cutanea tarda. Gastroenterology 87:1248, 1984.