Abstract

VIZCARGUENAGA, María Isabel. Storage Pool Disease: Review. Clinical and laboratory assays. Acta bioquím. clín. latinoam. [online]. 2006, vol.40, n.3, pp.327-334. ISSN 0325-2957.

The term Storage Pool Disease (SPD) defines a heterogeneous group of congenital abnormality of platelets characterized by deficiency of granules in megakaryocytes and platelets. The disorder is associated with mild or severe bleeding symptoms. It may present as an isolated platelet function defect, or be associated with a variety of congenital or acquired disorders. Platelets contain four main types of cytoplasmic granules classified according to their respective ultrastructures, densities and content: the a-granules, the dense bodies (or dense granules), the lysosomes and the peroxisomes. Platelets in the Storage Pool Disease-a (a-SPD) or Gray Platelet syndrome lack a-granules and their constituents. They appear gray on Wright-Giemsa stained blood smears. Storage Pool Disease d (d-SPD) is defined as a deficiency of dense bodies in megakaryocytes and circulating platelets and Storage Pool Disease ad (ad-SPD) is characterized by a deficiency of dense granules and a variable deficiency of platelet a-granules. Laboratory results from four (4) SPD patients are described. SPD platelets require the use of electron microscopy for reaching a definitive diagnosis.

Keywords : storage Pool disease; a-granules; dense granules; electron microscopy.

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