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Blood, 1972, Vol. 39, No. 2, pp. 197-209.
© 1972 American Society of Hematology, Inc.
Further Evidence for a Deficient Storage Pool
of Adenine Nucleotides
in Platelets From Some Patients With
Thrombocytopathia—"Storage Pool Disease"
Holm Holmsen 1 and
Harvey J. Weiss 1
1 Department of Medicine, Temple University School of Medicine, Philadelphia,
Pa. and the Division of Hematology, Department of Medicine, The Roosevelt Hospital and
the Columbia University College of Physicians & Surgeons, New York, N.Y.
The metalobism of adenine nucleotides
in platelets was studied in one patient
with thrombasthenia and in six patients whose bleeding disorder has
been attributed to a defect in collagen-induced platelet aggregation associated with impaired release of platelet
ADP (thrombocytopathia). In two of
these patients no specific abnormality
was found that might account for the
defect in the release reaction (thrombocytopathia B). In the other four patients (thrombocytopathia A), significantly decreased amounts of platelet
ATP and ADP and an increase in the
ATP/ADP ratio were obtained. The
specific radioactivity of both ATP and,
more strikingly, ADP that was found
after incubating their platelets with 3H-adenine was significantly greater than
normal. This indicated that the patients’ platelets lacked the nonmetabolic pool of adenine nucleotides
present in specialized intracellular
granules and that are specifically extruded from the platelet during the
release reaction. Low platelet serotonin values were found in three of
these patients, indicating that their
platelets may lack the entire content
of substances normally found in these
granules. In all four of the patients
with thrombocytopathia A, for which
the name "storage pool disease" is
proposed, platelet adenine uptake was
normal, but increased hypoxanthine
formation by resting cells was found in
the three patients with low serotonin
values. The breakdown of the ATP to
inosine monophopshate and hypoxanthine during the release reaction was
normal in all patients studied. Platelets
from the patient with thrombasthenia
were normal in all respects studied.
Submitted on April 20, 1971
Revised on June 29, 1971
Accepted on August 20, 1971
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