Storage Pool Disease: Comparative Fluorescence Microscopical, Cytochemical and Biochemical Studies on Amine-Storing Organelles of Human Blood Platelets

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Abstract
The platelet content of ATP, ADP, serotonin (5-HT), dopamine, noradrenaline [norepinephrine], adrenaline [epinephrine] and the net uptake of radiolabeled 5-HT and mepacrine were subnormal in 6 patients with storage pool disease (SPD). Fewer amine-storing organelles were found by fluorescence microscopy with the fluorescent probe mepacrine and by EM with a cytochemical (uranaffin) reaction specific for 5''-phosphonucleotides. The methods showed that SPD platelets have atypical organelles with a reduced capacity to store amines, 5''-phosphonucleotides and mepacrine. Changes were most marked in platelets of two patients who also had oculocutaneous albinism.