scholarly journals Isolated delta-storage pool disease

2020 ◽  
Author(s):  
Keyword(s):  
Storage Pool ◽  
Storage Pool Disease

Detection of an Acquired Platelet Storage Pool Disease in Three Patients with a Myeloproliferative Disorder

1979 ◽  
Vol 42 (02) ◽  
pp. 794-796 ◽  
Author(s):  
Francine Rendu ◽  
Marilyne Lebret ◽  
Alan Nurden ◽  
Jacques P Caen
Keyword(s):  
Myeloproliferative Disorder ◽  
Storage Pool ◽  
Platelet Storage ◽  
Storage Pool Disease

scholarly journals Immature dense granules in platelets from mice with platelet storage pool disease

Blood ◽  
1987 ◽  
Vol 69 (5) ◽  
pp. 1300-1306 ◽  
Author(s):  
M Reddington ◽  
EK Novak ◽  
E Hurley ◽  
C Medda ◽  
MP McGarry ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Dense Granule ◽  
Mutant Mice ◽  
Normal Numbers ◽  
Group Of Seven ◽  
Dense Granules ◽  
Storage Pool ◽  
Platelet Storage ◽  
Transmission Electron ◽  
Storage Pool Disease

Mepacrine uptake into platelets and bone marrow megakaryocytes was analyzed to further characterize the dense granule defects in a group of seven mouse pigment mutants that have characteristics of platelet storage pool disease (SPD). In contrast to our previous studies using electron microscopy, this method revealed that all mutants had normal numbers of dense granules. However, total mepacrine uptake in all mutant platelets was significantly diminished to less than 50% of normal uptake. Also, the flashing phenomenon observed when normal dense granules are irradiated with ultraviolet light was either greatly diminished or absent when platelets of individual mutants were similarly irradiated. Therefore the principal defect in the mutant platelets is an inability to accumulate dense granule contents rather than an absence of the granules. Mepacrine uptake into megakaryocytes was indistinguishable in normal and mutant mice. This indicates the mutant dense granule defects appear either very late in megakaryocyte development or early in platelet formation in correlation with development of the mature dense granule. By standard transmission electron microscopy we have not been able to detect gross structural or subcellular abnormalities in either platelets or megakaryocytes of mutant mice. It appears all seven mutants produce immature or functionally abnormal dense granules.

scholarly journals Acquired storage pool disease in platelets during disseminated intravascular coagulation

Blood ◽  
1976 ◽  
Vol 48 (4) ◽  
pp. 511-515
Author(s):  
FI Pareti ◽  
A Capitanio ◽  
PM Mannucci
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Adenine Nucleotides ◽  
Second Phase ◽  
Intravascular Coagulation ◽  
Vein Thrombosis ◽  
Storage Pool ◽  
Storage Pool Disease ◽  
Deep Vein ◽  
Induced Aggregation ◽  
Uptake And Metabolism

A patient with clinical and laboratory evidence of disseminated intravascular coagulation associated with deep-vein thrombosis and pulmonary embolism developed a qualitative platelet abnormality characterized by a defective release reaction. Second-phase aggregation induced by ADP and adrenaline was impaired, and reduced collagen- induced aggregation was accompanied by defective release of ADP and ATP. The decrease in total platelet ATP and ADP, the high ATP:ADP ratio in the presence of normal amounts of metabolic adenine nucleotides, and the low content of serotonin associated with abnormal uptake and metabolism of the exogenous amine suggested that the defective platelet function was due to lack of the platelet organelles in which serotonin and nonmetabolic adenine nucleotides are normally stored. Acquired storage pool disease is likely to be related to exposure of circulating platelets to aggregating agents, with their degranulation occurring during disseminated intravascular coagulation.

scholarly journals Low Neonatal Circumcision Bleeding Rate in Patients Diagnosed with Delta-Storage Pool Deficiency Later in Life

2021 ◽  
Author(s):  
Hebah Al Absi ◽  
Stein Dagmar
Keyword(s):  
Medical Records ◽  
Postoperative Bleeding ◽  
Newborn Period ◽  
Common Procedure ◽  
Intraoperative Bleeding ◽  
Bleeding Rate ◽  
Dense Granules ◽  
Storage Pool ◽  
Storage Pool Disease ◽  
Male Subjects

Introduction male circumcision is a common procedure, generally performed during the newborn period. Few reports have described circumcision in patients with bleeding disorders. Aim to determine bleeding rate after circumcision in neonatal male subjects who were diagnosed later in life with delta-storage pool disease (SPD). Methods we retrospectively reviewed the medical records of male subjects (<18 years of age) who were diagnosed with SPD later in life and were circumcised at birth without hemostatic prophylaxis due to lack of family history at that time from 2000-2020. Intraoperative/postoperative bleeding and bleeding severity were the main outcomes evaluated. Results 153 male subjects were included. Circumcision was performed at a median age of 2 days (range, 1 day-4 months). The main indication for circumcision was parental request. Median severity of granule deficiency was 2.76 dense granules/platelet (range, 1.12-3.82 DG/Plt). None of the subjects had intraoperative bleeding. Three subjects (2%) had postoperative bleeding and only one (0.65%) required ER intervention to stop bleeding. Conclusion the overall incidence of bleeding in our subjects with SPD who were undiagnosed and untreated at circumcision, is comparable to that reported for patients without a bleeding disorder.

scholarly journals EDTA-related degranulation mimicking Storage Pool Disease

2018 ◽  
Vol 93 (9) ◽  
pp. 1192-1193 ◽  
Author(s):  
Marco Amoruso ◽  
Lorenzo Alberio ◽  
Monika Nagy
Keyword(s):  
Storage Pool ◽  
Storage Pool Disease

δ-Storage Pool Disease

2009 ◽  
pp. 1999-1999
Author(s):  
Alexander K. C. Leung ◽  
Cham Pion Kao ◽  
Andrew L. Wong ◽  
Alexander K. C. Leung ◽  
Thomas Kolter ◽  
...  
Keyword(s):  
Storage Pool ◽  
Storage Pool Disease

scholarly journals Content and thrombin-induced release of acid hydrolases in gel-filtered platelets from patients with storage pool disease

Blood ◽  
1975 ◽  
Vol 46 (1) ◽  
pp. 131-142 ◽  
Author(s):  
H Holmsen ◽  
CA Setkowsky ◽  
B Lages ◽  
HJ Day ◽  
HJ Weiss ◽  
...  
Keyword(s):  
Acid Phosphatase ◽  
Normal Subjects ◽  
Acid Hydrolases ◽  
Release Reaction ◽  
Dense Granules ◽  
Storage Pool ◽  
Low Concentrations ◽  
Storage Pool Disease ◽  
Beta Galactosidase

Abstract The levels of four acid hydrolases, beta-N-acetyl glucosaminidase, beta- glucuronidase, beta-galactosidase, and acid phosphatase, and the extent of their release (release II) by thrombin was determined in platelets from nine normal subjects, nine patients with storage pool disease, and in normal platelets which had been exposed to aspirin. The levels of all four hydrolases were normal in patients with SPD. However, release of three of these hydrolases (acid phosphatase was an exception) by low concentrations of thrombin (0.015 and 0.04 U/ml) was decreased in the patients as a group, although considerable variation in the extent of release of each enzyme was noted. In contrast, aspirin failed to inhibit release II in normal platelets (except for a slight impairment in the release of beta-N-acetyl glucosaminidase), although release I (serotonin, ATP and ADP) was inhibited. All release defects could be overcome by using higher concentrations of thrombin (0.2 U/ml). The normal levels of acid hydrolases in the platelets of patients with SPD (who are deficient in the platelet dense granules) suggest that these enzymes are not normally stored in the dense granules, but rather in alpha-granules. The findings also support the conclusions of previous studies that the release reaction is impaired in SPD. This release defect appears to be different from that seen in normal platelets after exposure to aspirin.

Paradoxical constriction to platelets by arteries from rats with pulmonary hypertension

1991 ◽  
Vol 260 (6) ◽  
pp. H1929-H1934 ◽  
Author(s):  
R. C. Ashmore ◽  
D. M. Rodman ◽  
K. Sato ◽  
S. A. Webb ◽  
R. F. O'Brien ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Tone ◽  
Pulmonary Arteries ◽  
Systemic Hypertension ◽  
Sprague Dawley ◽  
Storage Pool ◽  
Platelet Storage ◽  
Early Appearance ◽  
Storage Pool Disease ◽  
And Storage

We recently described the early appearance of pulmonary hypertension in the fawn-hooded rat (FHR), an animal with platelet storage pool disease also known to develop systemic hypertension at later ages. Since mediators released from aggregating platelets influence vascular tone, we hypothesized that platelet-mediated pulmonary vascular responses in FHR may be abnormal and potentially linked to the mechanism of pulmonary hypertension. To test this we examined reactivity of isolated pulmonary arteries (PA) and thoracic aortas (Ao) from young FHR with moderately severe pulmonary hypertension but normal systemic pressures. These vessels were compared with PA and Ao from control Sprague-Dawley rat (SDR). Aggregating platelets (1,000-40,000 platelets/mm3) from FHR caused dilation of SDR PA and Ao but constriction of FHR PA and Ao. Qualitatively similar responses were also observed with platelets isolated from SDR implying that abnormal responses were not simply due to the storage pool deficiency in FHR. Response to the platelet-derived endothelium-dependent vasodilator ADP was markedly impaired in FHR PA and mildly impaired in FHR Ao. Endothelium-dependent dilation to acetylcholine, but not to A23187, was mildly impaired in FHR PA while responses to both dilators were normal in FHR Ao. Endothelium-independent dilation to sodium nitroprusside was normal in both FHR PA and Ao. Constrictor sensitivity to serotonin, but not to the thromboxane A2 mimetic U-46619, was increased in FHR PA while responses to both constrictors were normal in FHR Ao. In summary, PAs from FHR with spontaneous pulmonary hypertension exhibit paradoxical constriction to both normal and storage pool deficient platelets.(ABSTRACT TRUNCATED AT 250 WORDS)

scholarly journals Acquired storage pool disease in platelets during disseminated intravascular coagulation

Blood ◽  
1976 ◽  
Vol 48 (4) ◽  
pp. 511-515 ◽  
Author(s):  
FI Pareti ◽  
A Capitanio ◽  
PM Mannucci
Keyword(s):  
Disseminated Intravascular Coagulation ◽  
Adenine Nucleotides ◽  
Second Phase ◽  
Intravascular Coagulation ◽  
Vein Thrombosis ◽  
Storage Pool ◽  
Storage Pool Disease ◽  
Deep Vein ◽  
Induced Aggregation ◽  
Uptake And Metabolism

Abstract A patient with clinical and laboratory evidence of disseminated intravascular coagulation associated with deep-vein thrombosis and pulmonary embolism developed a qualitative platelet abnormality characterized by a defective release reaction. Second-phase aggregation induced by ADP and adrenaline was impaired, and reduced collagen- induced aggregation was accompanied by defective release of ADP and ATP. The decrease in total platelet ATP and ADP, the high ATP:ADP ratio in the presence of normal amounts of metabolic adenine nucleotides, and the low content of serotonin associated with abnormal uptake and metabolism of the exogenous amine suggested that the defective platelet function was due to lack of the platelet organelles in which serotonin and nonmetabolic adenine nucleotides are normally stored. Acquired storage pool disease is likely to be related to exposure of circulating platelets to aggregating agents, with their degranulation occurring during disseminated intravascular coagulation.

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