By Dr. Hans Georg Hansen, Elisabeth Graucob (auth.)
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Sample text
Aspartylglucosaminuria. Blood lymphocytes with fine, relatively sparse vacuoles; in a arranged like grapes (Wright staining). 35b 35c 35d Fig. 35a-d. Mucosulfatidosis. Blood. a Heavy, dense granulation in neutrophilic polymorphs. b Eosinophil with very coarse, red-violet granulation. c Less numerous, relatively fine granules in monocytes. d Small lymphocyte with moderately dense, very fine granulation. 49 Similarly, in the bone marrow, the changes in the myeloid and reticulum cells (Fig. 36a--c) are like those observed in MPSosis VI and VII.
Bone marrow. a Reticulum cell with predominantly fine, moderately dense granulation. b-f Plasma cells with partly coarse, lumpy, partly granular, and partly vacuolar storage phenomena; the lumpy inclusions are of different shapes and colors. 56 41 42b 42d r 57 Mucolip ido i GM , anglio ido i type I Peripheral blood arkcr cell •• w 80 ... Marker cell .. : : " (marker cells) in the mucoFig. 43. Schemat ic survey of the morphol ogically characte ristic cells bordered cells marker and from derived line lipidoses.
25a, b. Schematic survey of morphologically characteristic cells (marker cells) in MPSoses. a Blood. Marker cells bordered. 36 II BON E MARROW ad II Me!! Marker cell MP 0 i Gra nul ocyte Type I- H I- HI (Hurler) + Type II (Hunter) Type 1( cheie) Type III ( anlilippo) Type IV (Morquio) ncutroph . eosin. Type I (Marotcaux-Lamy) Type VII (P-glucuronida e deficiency) b . • neutroph . co in. Bone marrow. Cell line derived from and marker cells bordered. 37 Mucolipidoses These diseases either occur more rarely than the MPSoses or are recognized less often, since in some cases at least the dysmorphism is clinically less marked or even absent.