Platelet Number and Function in Diamond-Blackfan Anemia

PEDIATRICS ◽  
1981 ◽  
Vol 68 (2) ◽  
pp. 238-241
Author(s):  
George R. Buchanan ◽  
Blanche P. Alter ◽  
Christine A. Holtkamp ◽  
Elaine G. Walsh

Congenital red cell aplasia (Diamond-Blackfan anemia) is occasionally associated with hematologic defects other than a deficiency of red blood cell progenitors, but such alterations have not been well studied. The frequency and magnitude of abnormalities in platelet count and platelet function were therefore examined in 38 patients. Thrombocytosis was seen in 21 patients, and 12 had mild thrombocytopenia on at least one occasion. Elevated platelet counts were demonstrated repeatedly in nine children. The three patients with the lowest platelet counts also had leukopenia. Platelet aggregation was normal in all 16 patients in whom it was studied, and bleeding times were within the normal range in 14 of them. Bleeding signs and symptoms were not observed. It is concluded that thrombocytosis or thrombocytopenia often occurs but that platelet function is normal in patients with Diamond-Blackfan anemia.

2008 ◽  
Vol 100 (07) ◽  
pp. 01-12 ◽  
Author(s):  
Karen A. Moffat ◽  
Menaka Pai ◽  
Yang Liu ◽  
Jodi Seecharan ◽  
Heather McKay ◽  
...  

SummaryLight transmission platelet aggregation tests are important for diagnosing platelet function defects. However, uncertainties exist about the best procedures to determine aggregation reference intervals. We investigated methods for determining reference intervals for light transmission aggregation tests, using the % maximal aggregation values for prospectively collected data on healthy control samples. Reference intervals for samples tested at 250 x 109 platelets/l were determined by mean ± 2 standard deviations and non-parametric analyses. To establish reference intervals for tests on thrombocytopenic subjects, regression analyses were used to estimate 95% confidence limits for % maximal aggregation, according to sample platelet counts, using data for control samples diluted to match the platelet count of undiluted thrombocytopenic patient platelet-rich plasma samples. For samples tested at 250 x 109 platelets/l, non-parametric analyses described 95% of data for healthy control samples better than mean ± 2 standard deviations. For samples tested at lower counts, to match thrombocytopenic samples, the % maximal aggregation was influenced by platelet count and derived limits were wider at very low platelet counts for almost all agonists. With ristocetin, it proved feasible to test samples with very low platelet counts to exclude Bernard-Soulier syndrome and type 2B von Willebrand disease. Non-parametric analyses should be the preferred method to establish light transmission aggregation reference intervals for samples tested at normal platelet counts. The derived limits for thrombocytopenic samples provide guidance for evaluating thrombocytopenic platelet function disorders, including which agonists to test, based on the sample platelet count.


Blood ◽  
1946 ◽  
Vol 1 (6) ◽  
pp. 472-496 ◽  
Author(s):  
PAUL M. AGGELER ◽  
JOAN HOWARD ◽  
S. P. LUCIA ◽  
EDITH MILLS

Abstract Determinations of the platelet count, clot retraction, bleeding time, capillary fragility, and coagulation time were made in 64 normal subjects and in 404 patients suffering from various diseases. The normal values for the platelet count done by an experienced technician using the Rees and Ecker method were: mean, 409,000 per cu. mm.; standard deviation, 68,000 per cu. mm.; normal range (M ± 2.σ), 273,000 per cu. mm. to 545,000 per cu. mm. There was a statistically significant relationship between the platelet count and the results of tests of clot retraction, bleeding time, and capillary fragility, but there was no significant relationship between the platelet count and the coagulation time. Factors other than platelet count or platelet function which may influence the results of these tests are discussed. The critical level of the platelet count, below which abnormal bleeding is likely to occur, was found to be approximately 190,000 per cu. mm. in primary thrombocytopenic purpura and 230,000 per cu. mm. in secondary thrombocytopenic purpura. However, platelet counts as low as 100,000 per cu. mm. were found in one patient without abnormal bleeding, and counts as high as 280,000 per cu. mm. were found in another patient with classical primary thrombocytopenic purpura. In all patients in the active phase of bleeding in primary thrombocytopenic purpura and in most with secondary thrombocytopenic purpura, the bleeding time was markedly prolonged and clot retraction was definitely diminished. In approximately one half of the patients suffering from thrombocytopenia without bleeding or from thrombocytopenia complicating other hemorrhagic states, the results of these tests were abnormal. Capillary fragility was increased in approximately three fourths of the patients with primary thrombocytopenic purpura, one half with secondary thrombocytopenic purpura, and less than one half with thrombocytopenia without bleeding or with thrombocytopenia complicating other hemorrhagic states. In the stage of recovery from thrombocytopenic purpura, dissociation of the results of the various tests was sometimes found. In some patients the platelet counts returned to normal hut abnormalities persisted in the tests of the bleeding time, clot retraction, or capillary fragility. In other patients the results of one or all of these tests returned to normal before the platelet count had reached the normal range. These results have been interpreted as evidence of variability in the functional capacity of the platelets.


1972 ◽  
Vol 28 (02) ◽  
pp. 213-220 ◽  
Author(s):  
L Mettler ◽  
Meseck B. Selchow

SummaryPlatelet function was studied in women receiving two oral oestrogen-progestagen compounds (Neogynon and Noracyclin-22). Platelet count, adhesiveness, spreading, aggregation (PAT) and thromboelastography (TEG) were studied twice monthly for six months in 186 cycles. Platelet counts in rotated plasma were decreased in both groups. A significant increase in non-spread platelets was found in the Neogynon group only. PAT-degrees were significantly increased in both groups. Platelet adhesiveness and thromboelastographic measurements were not affected. Increased platelet aggregation during oral contraceptive therapy is regarded as a predisposing factor to thrombosis.


Author(s):  
Z. Jerushalmy ◽  
J. Pinkhas ◽  
M. Krinsky ◽  
A. de Vries

Methotrexate administered intraperitoneally to rats caused a transient disturbance in platelet function manifested by absence of ADP-induced aggregation and serotonin incorporation, and by decreased factor 3 (PF3) availability. These phenomena were noticed when the megakaryocyte count decreased while the platelet count was still within the normal range.Vincristine injection was not followed by an immediate drop in megakaryocyte and platelet count or PF3 availability, however a transient decrease of ADP-induced platelet aggregation and serotonin incorporation occurred.


Perfusion ◽  
1994 ◽  
Vol 9 (4) ◽  
pp. 265-269 ◽  
Author(s):  
Anthony Stallion ◽  
Barry R Cofer ◽  
Janice A Rafferty ◽  
Moritz M Ziegler ◽  
Frederick C Ryckman

Haemorrhagic complications, which occur in up to 35% of infants during extracorporeal membrane oxygenation (ECMO), often produce devastating sequelae. Although many complex factors interact to control haemostasis, platelet number and function has significant impact on the development of primary haemostasis. The optimum platelet count on ECMO, however, has not been defined. At our institution prior to August 1987, platelet counts were maintained at greater than 100 000/mm3. After August 1987, however, platelet counts of greater than 200 000/mm 3 were maintained. In a retrospective study, patients were randomly chosen from these two treatment periods: group 1 - March 1986 to July 1987; and group 2 - June 1988 to June 1989. The average platelet count, platelets administered, hours on ECMO, and bleeding complications were compared to each other and to the July 1992 ELSO Registry. There was a significant difference in average platelet counts between group 1 and group 2. However, the amount of platelets administered per kg per day was similar. There was a significant difference in overall bleeding complications between Group 2 (12%) and the ELSO Registry (35%) (p < 0.01). There was a trend towards decreased complications in all subgroups, although sample size precluded significance. We conclude that increasing platelet counts to greater than 200 000/mm3 decreases the overall bleeding complication rate. This advantage is achieved without a continuous need for increased platelet administration once the desired level is reached and without an increase in perfusion time, mechanical complications, or mortality.


1976 ◽  
Vol 36 (01) ◽  
pp. 221-229 ◽  
Author(s):  
Charles A. Schiffer ◽  
Caroline L. Whitaker ◽  
Morton Schmukler ◽  
Joseph Aisner ◽  
Steven L. Hilbert

SummaryAlthough dimethyl sulfoxide (DMSO) has been used extensively as a cryopreservative for platelets there are few studies dealing with the effect of DMSO on platelet function. Using techniques similar to those employed in platelet cryopreservation platelets were incubated with final concentrations of 2-10% DMSO at 25° C. After exposure to 5 and 10% DMSO platelets remained discoid and electron micrographs revealed no structural abnormalities. There was no significant change in platelet count. In terms of injury to platelet membranes, there was no increased availability of platelet factor-3 or leakage of nucleotides, 5 hydroxytryptamine (5HT) or glycosidases with final DMSO concentrations of 2.5, 5 and 10% DMSO. Thrombin stimulated nucleotide and 5HT release was reduced by 10% DMSO. Impairment of thrombin induced glycosidase release was noted at lower DMSO concentrations and was dose related. Similarly, aggregation to ADP was progressively impaired at DMSO concentrations from 1-5% and was dose related. After the platelets exposed to DMSO were washed, however, aggregation and release returned to control values. Platelet aggregation by epinephrine was also inhibited by DMSO and this could not be corrected by washing the platelets. DMSO-plasma solutions are hypertonic but only minimal increases in platelet volume (at 10% DMSO) could be detected. Shrinkage of platelets was seen with hypertonic solutions of sodium chloride or sucrose suggesting that the rapid transmembrane passage of DMSO prevented significant shifts of water. These studies demonstrate that there are minimal irreversible alterations in in vitro platelet function after short-term exposure to DMSO.


1982 ◽  
Vol 48 (01) ◽  
pp. 108-111 ◽  
Author(s):  
Elisabetta Dejana ◽  
Silvia Villa ◽  
Giovanni de Gaetano

SummaryThe tail bleeding time (BT) in rats definitely varies according to the method applied. Of the various variables that may influence BT, we have evaluated the position (horizontal or vertical) of the tail, the environment (air or saline), the temperature (4°, 23° or 37° C) and the type of anaesthesia. Transection of the tail tip cannot be used to screen drugs active on platelet function since it is sensitive to coagulation defects. Template BT in contrast is not modified by heparin and is sensitive to defects of platelet number and function (“storage pool disease”, dipyridamole-like drugs, exogenous prostacyclin). In contrast the test fails to detect aspirin-induced platelet dysfunction. The evidence reported indicates that thromboxane A2-prostacyclin balance is not a factor regulating BT. Aspirin treatment however may be a precipitating factor when associated with other abnormalities of platelet function.Template BT is a valid screening test for platelet disorders and for antiplatelet drugs.


1981 ◽  
Author(s):  
E Walter ◽  
D Deppermann ◽  
K Andrassy ◽  
E Weber

Thromboembolic phenomena often (30 %) complicate the nephrotic syndrome. It was therefor investigated, wether disturbed platelet functions play a role in this disease.28 normals, 34 patients with nephrotic syndrome and 18 of them with impaired kidney function were tested. In 20 patients the measurements were repeated after administration of aspirin plus dipyridamo1e.Patients with nephrotic syndrome showed in comparison to normals the following changes: 1. increased platelet count (p < 0.01), 2. enhanced platelet adhesiveness (Wright-test: p < 0.001), 3. increased spontaneous aggregation (PAT I: p < 0.001; PAT III: p < 0.01), 4. enhanced PF 4-activity (heparin neutralisation: p < 0.001), 5. elevated β TG-levels only in impaired kidney function. There was no difference in the reaction of platelets against ADP as well as collagen. The changes in platelet function correlated with the severity of the nephrotic syndrome (proteinurea, hypalbuminaemia, hyperlipo- proteinaemia). After aspirin plus dipyridamole administration spontaneous platelet aggregation and adhesiveness were normalized.There is a disturbance of platelet function in patients with nephrotic syndrome, which can be reversed with antiaggregating agents.


2000 ◽  
Vol 83 (03) ◽  
pp. 480-484 ◽  
Author(s):  
John James ◽  
Dianne Brown ◽  
Gordon Whyte ◽  
Mark Dean ◽  
Colin Chesterman ◽  
...  

SummaryThis is the first report of a method to assess the significance of numerical changes in the platelet count based upon a result exceeding the normal intra-individual variation in platelet numbers. Serial platelet counts from 3,789 subjects were analysed to determine the intra-individual variation in platelet numbers. A platelet count difference of 98 × 109/L in males was found to represent a change that would occur by chance in less than 1 in 1,000 platelet count determinations. Tables to determine the significance of platelet number variations, given N previous observations, are provided at two probability levels. The repeatability of the platelet count was calculated as 0.871 (males) and 0.849 (females) indicating that the heritability of platelet count is high and that the platelet count is predominantly genetically determined. A seasonal variation in platelet count was found with a ‘winter’ versus ‘summer’ difference of 5.10 × 109/L (males) and 5.82 × 109/L (females).


Cancers ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 1150
Author(s):  
Alberto Zanetto ◽  
Marco Senzolo ◽  
Elena Campello ◽  
Cristiana Bulato ◽  
Sabrina Gavasso ◽  
...  

Hyper-functional platelets are being proposed as a potential therapeutic target in multiple cancers. Whether this can be considered in patients with cirrhosis and hepatocellular carcinoma (HCC) is unknown as their platelet function has not yet been investigated. We evaluated platelet function in cirrhosis patients with HCC. Patients with cirrhosis with and without HCC were prospectively recruited. Platelet aggregation, a marker of platelet function, was assessed by impedance aggregometry with adenosine diphosphate (ADP), arachidonic acid (ASPI), and thrombin (TRAP) stimulation. Plasmatic levels of Von Willebrand factor antigen (VWF) were also determined. One-hundred patients were recruited (50 cirrhotics with and 50 without HCC). Cirrhosis severity by Child class and platelet count were comparable between cirrhotics with and without HCC. Cirrhotics with HCC had higher ADP- (45 vs. 28; p < 0.001), ASPI- (47 vs. 28; p < 0.001), and TRAP- (85 vs. 75; p = 0.01) induced platelet aggregation than cirrhotics without HCC, all indicative of platelet hyper-function. The relatively increased platelet aggregation in patients with HCC was confirmed after adjusting the analysis for platelet count/severity of thrombocytopenia. Levels of VWF were higher in patients with vs. without HCC (348 vs. 267; p = 0.006), particularly in compensated cirrhosis. In patients with cirrhosis, HCC is associated with increased platelet aggregation and higher VWF. The clinical implications of these findings deserve further investigation.


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