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ARTICLE |

'Storage Pool Disease' of Platelets: Title and subTitle BreakAssociation With Multiple Congenital Cavernous Hemangiomas FREE

Mohan Singh Khurana, MD; Eric Chun-Yet Lian, MD; Donald R. Harkness, MD
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JAMA. 1980;244(2):169-171. doi:10.1001/jama.1980.03310020045027
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A woman with congenital cavernous hemangiomas and a bleeding diathesis since childhood was found to have a qualitative platelet defect characterized by the absence of a second-phase aggregation induced by epinephrine, adenosine diphosphate (ADP), and collagen, accompanied by decreased levels of adenosine triphosphate (ATP) and ADP, with a high ATP-ADP ratio consistent with the diagnosis of "storage pool disease" of the platelets. There was no evidence of disseminated intravascular coagulation or circulating antiplatelet antibodies. The bleeding tendency responded to platelet transfusion.

(JAMA 244:169-171, 1980)

REFERENCES

Weiss HJ, Chervenick PA, Zalusky R, et al:  A familial defect in platelet function associated with impaired release of adenosine diphosphate . N Engl J Med 281:1264-1270, 1969;.
Holmsen H, Weiss HJ:  Further evidence for a deficient storage pool of adenine nucleotides in platelets from some patients with thrombocytopathia—"storage pool disease." Blood 39:197-209, 1972;.
Pareti FI, Capitanio A, Mannucci PM:  Acquired storage pool disease in platelets during disseminated intravascular coagulation . Blood 48:511-515, 1976;.
Zahavi J, Marder VJ:  Acquired "storage pool disease" of platelets associated with circulating antiplatelet antibodies . Am J Med 56:883-890, 1974;.
Russell NH, Keenan JP, Frais MA:  Thrombocytopathy associated with autoimmune haemolytic anaemia . Br Med J 2:604, 1978;.
Pareti FI, Maggi CA, Mannucci PM: Abstracts of the International Society of Haematology (Istanbul), abstract 201, 1977.
Lian ECY, Deykin D:  Diagnosis of von Willebrand's disease . Am J Med 60:344-356, 1976;.
Holmsen H, Storm E, Day HJ:  Determination of ATP and ADP in blood platelets (a modification of the firefly luciferase assay for plasma) . Anal Biochem 46:489-501, 1972;.
Weiss HJ:  Platelet aggregation, adhesion and adenosine diphosphate release in thrombopathia (platelet factor 3 deficiency): A comparison with Glanzmann's thrombasthenia and von Willebrand's disease . Am J Med 43:570-578, 1967;.
Lages B, Scrutton M, Holmsen H, et al:  Metal ion content of gel filtered platelets from patients with storage pool disease . Blood 46:119-130, 1975;.
Holmsen H, Setkowsky CA, Lages B, et al:  Content and thrombin-induced release of acid hydrolases in gel-filtered platelets from patients with storage pool disease . Blood 46:131-142, 1975;.
Hardisty RM, Mills DCB, Ketsa-ard K:  The platelet defect associated with albinism . Br J Haematol 23:679-692, 1972;.

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Weiss HJ, Chervenick PA, Zalusky R, et al:  A familial defect in platelet function associated with impaired release of adenosine diphosphate . N Engl J Med 281:1264-1270, 1969;.
Holmsen H, Weiss HJ:  Further evidence for a deficient storage pool of adenine nucleotides in platelets from some patients with thrombocytopathia—"storage pool disease." Blood 39:197-209, 1972;.
Pareti FI, Capitanio A, Mannucci PM:  Acquired storage pool disease in platelets during disseminated intravascular coagulation . Blood 48:511-515, 1976;.
Zahavi J, Marder VJ:  Acquired "storage pool disease" of platelets associated with circulating antiplatelet antibodies . Am J Med 56:883-890, 1974;.
Russell NH, Keenan JP, Frais MA:  Thrombocytopathy associated with autoimmune haemolytic anaemia . Br Med J 2:604, 1978;.
Pareti FI, Maggi CA, Mannucci PM: Abstracts of the International Society of Haematology (Istanbul), abstract 201, 1977.
Lian ECY, Deykin D:  Diagnosis of von Willebrand's disease . Am J Med 60:344-356, 1976;.
Holmsen H, Storm E, Day HJ:  Determination of ATP and ADP in blood platelets (a modification of the firefly luciferase assay for plasma) . Anal Biochem 46:489-501, 1972;.
Weiss HJ:  Platelet aggregation, adhesion and adenosine diphosphate release in thrombopathia (platelet factor 3 deficiency): A comparison with Glanzmann's thrombasthenia and von Willebrand's disease . Am J Med 43:570-578, 1967;.
Lages B, Scrutton M, Holmsen H, et al:  Metal ion content of gel filtered platelets from patients with storage pool disease . Blood 46:119-130, 1975;.
Holmsen H, Setkowsky CA, Lages B, et al:  Content and thrombin-induced release of acid hydrolases in gel-filtered platelets from patients with storage pool disease . Blood 46:131-142, 1975;.
Hardisty RM, Mills DCB, Ketsa-ard K:  The platelet defect associated with albinism . Br J Haematol 23:679-692, 1972;.
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