1954 Volume 59 Issue 3 Pages 315-332
1. In this paper the first case ever reported of qualitative abnormity of peroxidase of blood leucocytes was outlined and the name of “Congenital Gigantism of Peroxidase Granules” was suggested for this abnormity.
2. The Congenital Gigantism of Peroxidase Granules represents a monstrous malformation of the peroxidase granules-obvious aggregation of fine peroxidase blue granules producing gigantic clumpy effects in each cell-of all the myeloic leucocytes in the blood or/and bone marrow (Fig. 3, Fig. 4 and Fig. 5).
3. The Giemsa stained picture of the blood or/and marrow smears of this case was also in no way normal, while the morphological change was not always corresponding to the abnormal peroxidase granules (Cf. Fig. 6 and Fig. 7).
4. The oxidase granules of blood and marrow cells of this case showed a monstrous malformation similar to those of peroxidase granules. So the name of “Congenital Gigantism of Oxidase Granules” may be used instead.
5. The supravital method revealed a peculiar behavior of neutral red vacuoles in neutrophils, monocytes, lymphocytes and red blood corpusices (Cf. Fig. 8).
6. This patient had photophobia, xerodermia pigmentosum, albinismus fundi oculi utrisque and albinism of hair. He died at 11 months of age developing, besides bronchopneumonia, hepatosplenomegaly, jaundice, melena and pancytopenia.
7. The family pedigree of this patient is characteristic. The parents were of consanguinous marriage. Three sibs had the same congenital stigmata i.e. photophobia, pale color of hair, pigmentation of uncovered parts of the skin and died very early in life during some infection in the clinical conditions very similar to the present case. It is not difficult to suppose that these sibs had “Congenital Gigantism of Peroxidase Granules.”
8. The Congenital Gigantism of Peroxidase Granules might have a close connection with a short span of life of the patient.