Congenital Thrombocytopenia With Giant Platelets: An Ultrastructural Morphometric Study

1981 ◽  
Author(s):  
M I Barnhart ◽  
A H Ragab ◽  
V Lui ◽  
B L Evatt ◽  
J M Lusher
Keyword(s):  
Surface Membrane ◽  
Maximum Diameter ◽  
Morphometric Study ◽  
Slight Reduction ◽  
Bleeding Tendency ◽  
Normal Numbers ◽  
Freezing And Thawing ◽  
Giant Platelets ◽  
Dense Bodies ◽  
P Factor

We have studied a family with a previously undescribed familial platelet abnormality. 5 year old twins were evaluated because of excessive bruising and bleeding since infancy. Their mother also had a moderate bleeding tendency. All three had slightly decreased numbers of platelets (P) of variable size, abnormally low prothrombin consumption (which corrected with a P substitute), and abnormal P factor 3 availability (which corrected with disruption of P by repeated freezing and thawing). P aggregation was normal with ADP, epinephrine and ristocetin, but absent upon addition of collagen. SEM revealed abnormal surface activation upon contact, with the multi-podded giant P showing little evidence of spreading capability. In all 3 subjects P morphometry confirmed the impression of giant P. Whereas only 10% of normal control P had a maximum diameter (D max) of >3um, 77-82% of the twinsβ P had a D max of >3um, while 40% of the mother’s P did as well. Whereas only 5% of normal P had a surface area (SA) of>4.5 um2 , 55 and 93% of the twins' P and 50% of their mother’s P had>4.5 um2 SA. Morphologically a number of P profiles had extensively vacuolated interiors (twins had 5-6 X normal). Other P sections contained only a few organelles. We applied quantitative morphometry to P sections. When organelles were counted per um2 of P, all 3 family members had mitochondria and ∝ granules in the normal range, whereas all 3 had a slight reduction in dense bodies. (These findings rule out a diagnosis of gray platelet syndrome). The ∝ granule compartment, although of normal numbers, displayed a higher than normal proportion of mixed type granules (dense component, bulls eye configurations in a lower density homogeneous matrix). Multiple defects (20% or more vacuolated P, reduced dense bodies, abnormal surface membrane) appear to be responsible for ineffective platelet plug formation.

scholarly journals Infra-acetabular screw exited between ischial tuberosity and ischial spine is more suitable for Asian population: A 3D Morphometric study

2020 ◽  
Author(s):  
Fei Liu ◽  
Xiaoreng Feng ◽  
Yang Xiao ◽  
Jie Xiang ◽  
Keyu Chen ◽  
...  
Keyword(s):  
Asian Population ◽  
3D Models ◽  
Ischial Tuberosity ◽  
Fixation Strength ◽  
Maximum Diameter ◽  
Acetabular Fractures ◽  
Morphometric Study ◽  
Exit Point ◽  
Ischial Spine ◽  
Length Direction

Abstract Background Recently, the infra-acetabular screw has been proposed for use in treatment of acetabular fractures as a part of a periacetabular fixation frame. Biomechanical studies have shown that an additional infra-acetabular screw placement can enhance the fixation strength of acetabular fracture internal fixation. Currently, the reported exit point of the infra-acetabular screw has been located at the ischial tuberosity (Screw I). However, our significant experience in placement of the infra-acetabular screw has suggested that when the exit point is located between the ischial tuberosity and the ischial spine (Screw II), the placement of a 3.5 mm infra-acetabular screw may be easier for some patients. We conducted this study in order to determine the anatomical differences between the two different IACs. Methods The raw datasets were reconstructed into 3D models using the software MIMICS. Then, the models, in the STL format model, were imported into the software Geomagic Studio to delete the inner triangular patches. Additionally, the STL format image processed by Geomagic Studio was imported again into MIMICS. Finally, we used an axial perspective based on 3D models in order to study the anatomical parameters of the two infra-acetabular screw corridors with different exit points. Hence, we placed the largest diameter virtual screw in the two different screw corridors. The data obtained from this study presents the maximum diameter, length, direction, and distances between the entry point and center of IPE. Results In 65.31% males and 40.54% females, we found a screw I corridor with a diameter of at least 5 mm, while a screw II corridor was present in 77.55% in males and 62.16% in females. Compared to screw I, the length of screw II is reduced, the angle with the coronal plane is significantly reduced, and the angle with the transverse plane is significantly increased.Conclusions For East Asians, changing the exit point of the infra-acetabular screw can increase the scope of infra-acetabular screw use, especially for females.

scholarly journals Double heterozygosity for mutations in the platelet glycoprotein IX gene in three siblings with Bernard-Soulier syndrome

Blood ◽  
1993 ◽  
Vol 81 (9) ◽  
pp. 2339-2347 ◽  
Author(s):  
SD Wright ◽  
K Michaelides ◽  
DJ Johnson ◽  
NC West ◽  
EG Tuddenham
Keyword(s):  
Normal Subjects ◽  
Immunoblot Analysis ◽  
Platelet Glycoprotein ◽  
Bleeding Tendency ◽  
Missense Mutations ◽  
Coding Region ◽  
Giant Platelets ◽  
Double Heterozygosity ◽  
Induced Aggregation ◽  
Bernard Soulier Syndrome

Abstract Bernard-Soulier syndrome (BSS) giant platelets have defective and/or deficient glycoprotein (GP) Ib/IX complexes, causing absent ristocetin- induced aggregation, defective interaction with von Willebrand factor, morphologic abnormality, and a clinical bleeding tendency. Recently several mutations have been described in the platelet GPIb alpha gene in individuals exhibiting the BSS phenotype. We have studied a family with classical BSS, and have excluded lesions at the GPIb alpha locus by restriction fragment length polymorphism linkage analysis. Analysis of the genes for two other components of the platelet GPIb:IX complex, namely GPIb beta and GPIX, showed two different missense mutations in the coding region of the GPIX gene: an A-->G transition in codon 21 results in conversion of an aspartic acid to glycine and an A-->G change in codon 45 converts an asparagine residue to serine. Three affected individuals are doubly heterozygous for these mutations, which alter conserved residues in or flanking the GPIX leucine-rich glycoprotein motif. Both mutations create new recognition sites for the enzyme Fnu 4H1; therefore, this enzyme was used to screen 60 normal subjects (120 alleles). Neither mutation was detected in any subject other than direct relatives of the affected individuals. Although low levels of GPIb were demonstrable by both flow cytometry and immunoblot analysis in an affected individual's platelets, there was no evidence of GPIX immunoreactivity. We propose that expression of abnormal GPIX prevents stable assembly of the GPIb/IX complex, causing BSS in the doubly heterozygous individuals in this family.

ELECTRONMICROSCOPIC STUDIES ON PLATELETS AND MEGAKARYOCYTES IN GIANT PLATELET SYNDROME

1987 ◽  
Author(s):  
Sozo Suzuki ◽  
Kazuo Mori ◽  
Koji Sugai ◽  
Yasuyuki Akutsu ◽  
Masaaki Ishikawa ◽  
...  
Keyword(s):  
Peripheral Blood ◽  
Uranyl Acetate ◽  
Ruthenium Red ◽  
Membrane Systems ◽  
Platelet Factor ◽  
Giant Platelets ◽  
Thin Sections ◽  
Giant Platelet ◽  
Dense Bodies ◽  
Large Platelet

Giant platelet syndrome are characterized morphologically by many giant platelets associated with several functional abnormalities in the peripheral blood. However, the mechanism of large platelet production has not yet been clarified. In 1981, we reported acase with Bernard-Soulier syndrome(BSS) in whom giant platelets were considered to be formed by fusion of two or three platelets in the circulating blood. We examined the ultrastructure of platelets and megakaryocytes in another case with BSS (29 year-old female) and a case with May-Hegglin anomaly (31 year-old male). Whole blood and bone marrow specimens were fixed with glutaraldehyde-osmium solution. Thin sections were prepared and stained with uranyl acetate and lead cytrate. Membrane systems of platelets and megakaryocytes in a case with BSS was investigated by staining of surface coating with ruthenium red.In a case with BSS, most platelets were very large and similar in morphology to those in formerly reported case. Giant platelets contained several-fold increased number of α-granules and mitochondria. Typical dense bodies were also observed. Contents of ATP/ADP, platelet factor-4(PF-4), B-thromboglobulin(B-TG) and platelet factor-3 availability(PF-3) were increased. Disorganization of microtubules was recognized. Some giant platelet contained membrane systems similar to demarcation membranes(DM) in megakaryocytes, characteristically. In mature megakaryocytes, areas divided by DM similar in size to those in normal megakaryocytes were observed. Several of these areas appeared to fuse together to form the giant platelets containing many granules and remnants of DM. In a case with May-Hegglin anomaly, typical Dohle’s bodies were shown in neutrophilic granulocytes. Giant platelets in this case also contained large number of α-granules and some of them contained membrane systems similar to DM. Areas similar in morphology to these giant platelets were clearly noted in the cytoplasm of mature megakaryocytes.In these cases, most giant platelets in the peripheral blood may be formed in the cytoplasm of megakaryocytes by fusion of several areas divided by DM, each of which may become normal sized platelets in normal megakaryocytes.

Compound Heterozygosity (554-589 del, C515-T Transition) in the Platelet Glycoprotein Ibα Gene in a Patient with a Severe Bleeding Tendency

1999 ◽  
Vol 81 (04) ◽  
pp. 486-492 ◽  
Author(s):  
Maurizio Margaglione ◽  
Giovanna D’Andrea ◽  
Elvira Grandone ◽  
Vincenzo Brancaccio ◽  
Aldo Amoriello ◽  
...  
Keyword(s):  
Flow Cytometric Analysis ◽  
Normal Function ◽  
Platelet Glycoprotein ◽  
Severe Bleeding ◽  
Bleeding Tendency ◽  
Giant Platelets ◽  
Novel Variant ◽  
Leucine Rich Repeats ◽  
Von Willebrand

SummaryGiant platelets in the blood smear, absent in vitro platelet agglutination in response to ristocetin, and normal aggregation, ATP secretion and thromboxane B2 formation were found in a young patient with a life-long bleeding tendency. Ristocetin-induced von Willebrand factor binding to her platelets was less than 10% of normal. Flow cytometric analysis with monoclonal antibodies LJ-Ib-1, LJ-Ib-10, and LJ-P3 was consistent with the latter finding. SDS-PAGE analysis of solubilized platelets showed a marked reduction of the platelet glycoprotein (GP) Ibα. Genetic characterisation demonstrated that the patient and her father were heterozygous for a deletion of 36 nucleotides (positions 554-589) leading to a mutant GPIbμ (deletion of aminoacids from residue 169 to 180 and a Glu → Lys substitution at residue 181). In addition, a C → T transition at nucleotide 515 in the other allele of the GPIbα gene was found in the patient and in her mother that results in the substitution of alanine for valine in codon 156 (Bernard-Soulier type Bolzano). These variations occurred within the VI and VII leucine-rich repeats. The novel variant of Bernard-Soulier syndrome identified further suggests that the integrity of leucine-rich repeats is important for normal function of the GP Ib-IX-V receptor complex.

scholarly journals Infra-acetabular screw exited between ischial tuberosity and ischial spine is more suitable for Asian population: a 3D morphometric study

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Fei Liu ◽  
Xiaoreng Feng ◽  
Yang Xiao ◽  
Jie Xiang ◽  
Keyu Chen ◽  
...  
Keyword(s):  
Asian Population ◽  
3D Models ◽  
Ischial Tuberosity ◽  
Fixation Strength ◽  
Maximum Diameter ◽  
Acetabular Fractures ◽  
Morphometric Study ◽  
Exit Point ◽  
Ischial Spine ◽  
Length Direction

Abstract Background Recently, the infra-acetabular screw has been proposed for use in treatment of acetabular fractures as a part of a periacetabular fixation frame. Biomechanical studies have shown that an additional infra-acetabular screw placement can enhance the fixation strength of acetabular fracture internal fixation. Currently, the reported exit point of the infra-acetabular screw has been located at the ischial tuberosity (Screw I). However, our significant experience in placement of the infra-acetabular screw has suggested that when the exit point is located between the ischial tuberosity and the ischial spine (Screw II), the placement of a 3.5 mm infra-acetabular screw may be easier for some patients. We conducted this study in order to determine the anatomical differences between the two different IACs. Methods The raw datasets were reconstructed into 3D models using the software MIMICS. Then, the models, in the STL format model, were imported into the software Geomagic Studio to delete the inner triangular patches. Additionally, the STL format image processed by Geomagic Studio was imported again into MIMICS. Finally, we used an axial perspective based on 3D models in order to study the anatomical parameters of the two infra-acetabular screw corridors with different exit points. Hence, we placed the largest diameter virtual screw in the two different screw corridors. The data obtained from this study presents the maximum diameter, length, direction, and distances between the entry point and center of IPE. Results In 65.31% males and 40.54% females, we found a screw I corridor with a diameter of at least 5 mm, while a screw II corridor was present in 77.55% in males and 62.16% in females. Compared to screw I, the length of screw II is reduced, the angle with the coronal plane is significantly reduced, and the angle with the transverse plane is significantly increased. Conclusions For East Asians, changing the exit point of the infra-acetabular screw can increase the scope of infra-acetabular screw use, especially for females.

scholarly journals Spontaneous Platelet Aggregation With Congenital Giant Platelet Containing Large Granules and Thick Membrane

2001 ◽  
Vol 7 (4) ◽  
pp. 305-310 ◽  
Author(s):  
Zhaoyue Wang ◽  
Jumei Shi ◽  
Yue Han ◽  
Yingchun Wang ◽  
Changgeng Ruan
Keyword(s):  
Electron Microscopy ◽  
Plasma Membrane ◽  
Platelet Aggregation ◽  
Bleeding Tendency ◽  
Giant Platelets ◽  
Platelet Surface ◽  
Giant Platelet ◽  
Platelet Disorder ◽  
Platelet Disorders ◽  
Spontaneous Platelet Aggregation

Inherited giant platelet disorders are a heterogeneous group of disorders. In the current study, a patient was reported with moderate bleeding tendency, giant platelets, and spontaneous platelet aggregation, which were not affected by the administration of aspirin or ticlopidine. The electron microscopy of platelets showed a black and thick plasma membrane with crystal-like fine hairs in the exterior coat and more large and variously shaped granules in the cytoplasm. The expression of glycoprotein (GP) Ib, GP IIb, and GP IIIa on platelet surface was normal, and no mutations in genes for GP Ibα, GP Ibβ, and GP IX were detected. These phenomena are so distinguishable from those of Mondreal platelet syndrome and other hereditary giant platelet disorders, that we propose that this patient probably has a novel platelet disorder, which has not yet been reported.

scholarly journals Influence of Imidazole Carboxamide on Platelet Function

1977 ◽  
Author(s):  
P. Kubisz ◽  
P. Klener ◽  
S. Cronberg
Keyword(s):  
Platelet Function ◽  
Light Transmission ◽  
Platelet Rich Plasma ◽  
Cytostatic Drug ◽  
Bleeding Tendency ◽  
Platelet Dysfunction ◽  
Freezing And Thawing ◽  
Trade Name ◽  
Coagulation And Fibrinolysis

Imidazol carboxamide (DTIC, NSC-45388) is a cytostatic drug used in the treatment of malignant melanoma under the trade name of DacarbazinR, MSD. Its influence on platelet function, blood coagulation and fibrinolysis was investigated in vitro.At a concentration of 160 µg/ml it inhibited the increase in light transmission induced in platelet-rich plasma by standardized freezing and thawing. It also retarded the retraction of reptilase clots. This therefore indicated a stabilizing effect on the platelets at this dosage.At a concentration of 40 μg/ml the drug did not significantly influence the platelet function in vitro.This concentration corresponds to therapeutic plasma levels. At current dosage of the drug any bleeding tendency due to platelet dysfunction therefore seems unlikely.

Protein composition and protein synthesis in the surface membranes of Schistosoma mansoni

Parasitology ◽  
1972 ◽  
Vol 65 (1) ◽  
pp. 55-69 ◽  
Author(s):  
J. R. Kusel
Keyword(s):  
Protein Synthesis ◽  
Calcium Chloride ◽  
Unit Area ◽  
Surface Membrane ◽  
Biological Significance ◽  
Protein Composition ◽  
Major Protein ◽  
Freezing And Thawing ◽  
Protein Components ◽  
Protein Incorporation

Saponin treatment of cercariae and schistosomula alters the surface membrane so that it may be sheared from the organism and isolated as fragments by centrifugation. Saponin-calcium chloride treatment or freezing and thawing of adult worms removes the surface membrane, which can be washed from the bodies and collected by centrifugation. The small quantities of material available necessitated the development of a sensitive technique for detecting the proteins in the membranes. The surface membranes were radioiodinated after butan-1-ol extraction and electrophoresed in polyacrylamide gel. Adult surfaces prepared by the saponin-calcium chloride and by the freezing and thawing technique had identical protein components, detected as gel-cut profiles or in autoradiographs. The quantity of a rapidly migrating PAS-positive amido black negative component was greater in the surfaces prepared by saponincalcium chloride than in the frozen and thawed surfaces. This component contains lipid, some of which may be glycolipid. It was largely absent from the surface membranes of cercariae and schistosomula. Cercarial surface membranes contained a major protein component which was absent from the surface membranes of schistosomula. Otherwise the surface membranes of the cercariae were identical to those of the schistosomula in their protein components. The rate of incorporation of freshly synthesized protein per unit area of surface membrane of schistosomula was very low in the first 5 days in culture, after which there was a very rapid increase to a maximum rate on the 15th day. After this time, the rate of protein synthesis decreased to a low level at 26 days. In these studies the activity per unit area was measured and this would not be expected to vary greatly during growth. The biological significance of the observed variation in protein incorporation into the membrane is unclear.

scholarly journals Nuclear morphometric study of prostatic atrophy

2015 ◽  
Vol 21 (2) ◽  
pp. 101-104
Author(s):  
Manuela Enciu ◽  
Mariana Aschie ◽  
Anca Chisoi ◽  
I. Poinareanu ◽  
Liliana Ana Tuta
Keyword(s):  
Elongation Factor ◽  
Average Diameter ◽  
The Elderly ◽  
Maximum Diameter ◽  
Morphometric Study ◽  
Control Group ◽  
Nuclear Area ◽  
Adenomatous Hyperplasia ◽  
Prostatic Atrophy ◽  
Over Time

Abstract The prostatic atrophy is a lesion with small glands that can mimic adenocarcinoma, commonly diagnosed in the elderly and whose preneoplastic role is controversial. Over time several classifications have been developed for this lesions according to its architectural and cellular aspects. Materials and Methods: Aim of the study is morphometrically assessment of epithelial component nuclei in prostatic atrophy. We evaluated parameters represented by nuclear area (NA), nuclear perimeter P (μm) Elongation factor (E), average diameter (DEQ), maximum diameter (Dmax), Minim diameter (Dmin). The control group was represented by adenomatous hyperplasia component of benign hyperplasia of the prostate. Results: Morphometric analysis revealed significantly higher values of nuclear parameters in atrophy compared with adenomatous component in controls (p <0.05) which can signify nucleomegaly and a potential preneoplastic role.

scholarly journals Absence of the 145000 Molecular Weight, Soluble Platelet Glycoprotein - Lack of Platelet Agglutination

1977 ◽  
Author(s):  
N.O. Solum ◽  
I. Hagen ◽  
M. Peterka ◽  
T. Gjemdal
Keyword(s):  
Molecular Weight ◽  
Soluble Fraction ◽  
Periodic Acid ◽  
Subcellular Fractionation ◽  
Positive Control ◽  
Human Platelets ◽  
Platelet Glycoprotein ◽  
Freezing And Thawing ◽  
Periodic Acid Schiff ◽  
Giant Platelets

The agglutination of human platelets by bovine factor VIII related protein has been studied in relation to the presence or absence of the 145ooo molecular weight, soluble platelet glycoprotein (GPS) using the presence of a granule-located glycoprotein as a positive control. GPS is found in the soluble fraction after subcellular fractionation of platelet homogenates on sucrose density gradients, and is probably identical to the soluble membrane protein glycocalicin The granule and the soluble glycoproteins separate into two bands on SDS-polyacrylamide gels both in reduced and unreduced samples when the gels contain urea and EDTA and are stained with periodic acid-Schiff reagent (PAS). A concomitant loss, or specific elution, of GPS and loss of agglutination was observed when platelets were stored in Tris-buffered saline, pH 7.4 (TS)at 4°C for 7 days, or when freshly prepared platelets were frozen and thawed when suspended in TS. When suspended in an EDTA-containing buffer during freezing and thawing, the resulting platelet ghosts still contained GPS and still agglutinated. The soluble fraction obtained by subcellular fractionation of giant platelets(Bernard-Soulier)did not show any PAS bands, whereas soluble fraction of these platelets prepared by freezing and thawing in TS clearly showed the presence of the granule protein but not the GPS band. The data indicate an association between GPS and the agglutination phenomenon.

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