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Pyridoxine‐Responsive Anaemias
Author(s) -
Gehrmann G.
Publication year - 1965
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1965.tb00087.x
Subject(s) - pyridoxine , pyridoxine deficiency , medicine , sideroblastic anemia , anemia , reticulocyte , iron deficiency , physiology , endocrinology , chemistry , biochemistry , messenger rna , gene
P atients with sideroblastic anaemia do not respond to iron or other common haematinics. Since the first report by Harris, Whittington, Weisman and Horrigan (1956) on ‘Pyridoxine‐Responsive Anemia in the Human Adult’, 30 additional cases have been reported in the literature (Gehrmann, 1963). The problem presented by these pyridoxine‐responsive anaemias is underlined by the fact that not all patients with sideroblastic anaemia respond to the administration of pyridoxine, and that in some patients with pyridoxine‐responsive anaemia the effect of treatment is incomplete whereas in others full regression of all abnormalities follows. These different responses to the administration of pyridoxine and certain distinguishing clinical and laboratory features suggest that at least two miscellaneous groups of pyridoxine‐responsive anaemias exist. The available data suggest that some of these patients are actually deficient in pyridoxine and this allows us to distinguish between ‘pyridoxine‐deficiency anaemia’ and so‐called pyridoxine‐responsive anaemia. All the reported cases in the literature can be classified into these two groups. Furthermore, it seems justified to distinguish within the group of pyridoxine‐responsive anaemias cases of either hereditary (‘anaemia sideroblastica hereditaria’) or acquired (‘anaemia refractoria sideroblastica’) origin. Features common to both pyridoxine‐deficiency and pyridoxine‐responsive anaemia are: (1) severe hypochromic anaemia with a dimorphic blood picture and a low reticulocyte count; (2) hyperferraemia and haemosiderosis; (3) maturation arrest of the erythroblasts with sideroblastosis (‘ring’ sideroblasts); (4) an increased iron disappearance rate but a decreased red‐cell uptake; (5) a slightly decreased or a normal red‐cell survival time; (6) absence of an abnormal haemoglobin, and (7) at least some correction of anaemia after the administration of pyridoxine. Present knowledge of these two groups will be discussed in the present report and supplemented by personal observations. Patients with pyridoxine‐responsive anaemia which develops during the course of isoniazid treatment will be excluded, since this drug can induce true pyridoxine deficiency by forming a water‐soluble iso ‐nicotinyl hydrazone complex (Williams and Abdulian, 1956).