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Myelosclerosis and Paroxysmal Nocturnal Haemoglobinuria
Author(s) -
Lewis S. M.,
Pettit J. E.,
Tattersall M. H. N.,
Pepys M. B.
Publication year - 1971
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1971.tb00898.x
Subject(s) - lysis , haemolysis , medicine , erythropoiesis , hemoglobinuria , immunology , paroxysmal nocturnal hemoglobinuria , clone (java method) , hemolysis , biology , anemia , biochemistry , dna
3 patients with myelosclerosis have subsequently developed paroxysmal nocturnal haemoglobinuria, with positive in‐vitro lysis tests (acidified‐serum lysis, cold‐antibody lysis and sucrose lysis), characteristic of PNH. These cases provide further evidence of a relationship between PNH and myeloproliferative disorders; they support the proposition that PNH is an acquired defect of erythropoiesis due to an abnormal clone. In two of the patients PNH has been severe with marked intravascular haemolysis, so that in these cases the clinical course and management of the original disease have been influenced by the PNH. In the third patient the PNH defect has, so far, been essentially only a phenomenon of in‐vitro tests and apparently without clinical effect.