Neuroacanthocytosis
beta-Spectrin Sao PauloII, a novel frameshift mutation of the beta-spectrin gene associated with hereditary spherocytosis and instability of the mutant mRNA. (1/24)
Hereditary spherocytosis (HS) is a common inherited anemia characterized by the presence of spherocytic red cells. Defects in several membrane protein genes have been involved in the pathogenesis of HS. beta-Spectrin-related HS seems to be common. We report here a new mutation in the beta-spectrin gene coding region in a patient with hereditary spherocytosis. The patient presented acanthocytosis and spectrin deficiency and, at the DNA level, a novel frameshift mutation leading to HS, i.e., a C deletion at codon 1392 (beta-spectrin Sao PauloII), exon 20. The mRNA encoding beta-spectrin Sao PauloII was very unstable and the mutant protein was not detected in the membrane or in other cellular compartments. It is interesting to note that frameshift mutations of the beta-spectrin gene at the 3' end allow the insertion of the mutant protein in the red cell membrane, leading to a defect in the auto-association of the spectrin dimers and consequent elliptocytosis. On the other hand, beta-spectrin Sao PauloII protein was absent in the red cell membrane, leading to spectrin deficiency, HS and the presence of acanthocytes. (+info)Acanthocytes of Stropharia rugosoannulata function as a nematode-attacking device. (2/24)
Efficient killing of nematodes by Stropharia rugosoannulata Farlow ex Murrill cultures was observed. This fungus showed the ability to immobilize the free-living nematode Panagrellus redivivus Goodey within minutes and to immobilize the pine wilt nematode Bursaphelenchus xylophilus (Steiner & Buhrer) Nickle within hours on agar plates. Moreover, P. redivivus worms were completely degraded by the fungus within 24 to 48 h. The cultures of S. rugosoannulata studied shared the characteristic of abundantly producing cells with finger-like projections called acanthocytes. We showed that the nematode-attacking activity of this fungus is carried out by these spiny acanthocytes and that mechanical force is an important factor in the process. Furthermore, the growth and nematode-attacking activity of the fungus in soil were also determined, and our results suggest that acanthocytes are functional in soil. (+info)Autopsy case of Dubin-Johnson syndrome with pneumonia and abetalipoproteinemia-like lipid profile. (3/24)
We report the autopsy of a 79-year-old Japanese woman with Dubin-Johnson syndrome accompanied by pneumonia, an abetalipoproteinemia-like lipid profile and acanthocytosis. On admission, physical examination of the patient revealed malnutrition. Blood tests revealed marked inflammatory changes and mild liver dysfunction. Chest X-ray indicated bilateral pneumonia. Total cholesterol, low-density lipoprotein (LDL) cholesterol and triglyceride levels were 89 mg/dL, 5 mg/dL and 6 mg/dL, respectively. Peripheral blood smears revealed numerous acanthocytes. Despite the administration of antibiotics and nutritional support, the patient died. Autopsy revealed a black liver, atrophy of fat tissue on the mesentery, and pneumonia with bilateral pleural effusion. We believe that the abetalipoproteinemia-like lipid profiles in this case were caused by malnutrition and the inflammatory changes rather than the direct effects of Dubin-Johnson syndrome. We base this conclusion on the following three findings: 1) the patient's lipid profile before hospitalization was in the normal range, 2) her serum LDL cholesterol and triglyceride levels gradually increased after nutritional support began, and 3) blood tests revealed marked inflammatory changes (C-reactive protein 9.0 mg/dL; interleukin-6 16.4 pg/mL). This case provides important information that enhances our understanding of lipid metabolism under conditions of malnutrition and inflammation. (+info)Insights into extensive deletions around the XK locus associated with McLeod phenotype and characterization of two novel cases. (4/24)
The McLeod phenotype is derived from various forms of XK gene defects that result in the absence of XK protein, and is defined hematologically by the absence of Kx antigen, weakening of Kell system antigens, and red cell acanthocytosis. Individuals with the McLeod phenotype usually develop late-onset neuromuscular abnormalities known as the McLeod syndrome (MLS). MLS is an X-linked multi-system disorder caused by absence of XK alone, or when the disorder is caused by large deletions, it may be accompanied with Duchenne muscular dystrophy (DMD), chronic granulomatous disease (CYBB), retinitis pigmentosa (RPGR), and ornithine transcarbamylase deficiency (OTC). XK defects derived from a large deletion at the XK locus (Xp21.1) have not been characterized at the molecular level. In this study, the deletion breakpoints of two novel cases of McLeod phenotype with extensive deletions are reported. Case 1 has greater than 1.12 million base-pairs (mb) deletion around the XK locus with 7 genes affected. Case 2 has greater than 5.65 mb deletion from TCTE1L to DMD encompassing 20 genes. Phylogenetic analyses demonstrated that DMD, XK and CYBB have close paralogs, some of which may partially substitute for the functions of their counterparts. The loci around XK are highly conserved from fish to human; however, the disorders are probably specific to mammals, and may coincide with the translocation of the loci to the X chromosome after the speciation in birds. The non-synonymous to synonymous nucleotide substitution rate ratio (omega=dN/dS) in these genes was examined. CYBB and RPGR show evidence of positive selection, whereas DMD, XK and OTC are subject to selective constraint. (+info)Beta-spectrinBari: a truncated beta-chain responsible for dominant hereditary spherocytosis. (5/24)
(+info)The acanthocyte-echinocyte differential: The example of chorea-acanthocytosis. (6/24)
(+info)Drug-induced hemolytic anemia and thrombocytopenia associated with alterations of cell membrane lipids and acanthocyte formation. (7/24)
(+info)Erythrocyte membrane changes of chorea-acanthocytosis are the result of altered Lyn kinase activity. (8/24)
(+info)
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Chorea-acanthocytosis
... this disease is marked by the presence of acanthocytes in blood (these acanthocytes can sometimes be absent or even make a late ... Another one of them would be that this disease should be considered in patients who have elevated levels of acanthocytes in a ... These thorny cells are called acanthocytes. Other effects of the disease may include epilepsy, behaviour changes, muscle ... of the patient on multiple occasions with a specific request given to the haematologist to examine the film for acanthocytes. ...
Pholiota
None form acanthocytes on their mycelia. P. aurivella (Batsch) P.Kumm. P. communis (Cleland & Cheel) Grgur. (southeastern ...
Abetalipoproteinemia
Acanthocytes are seen on blood smear. Since there is no or little assimilation of chylomicrons, their levels in plasma remains ...
Echinocyte
... s can be distinguished from acanthocytes by the shape of the projections, which are smaller and more numerous than in ... Spiculated cells (echinocytes and acanthocytes) and target cells. UpToDate (release: 20.12- C21.4) [1] Hoffman, R; Benz, EJ; ... Echinocytes are frequently confused with acanthocytes, but the mechanism of cell membrane alteration is different. ... like acanthocytes, may be found in hyperlipidemia caused by liver dysfunction, but the lipids themselves do not integrate into ...
Stropharia rugosoannulata
ISBN 0-671-42849-7. Hong Luo; Xuan Li; Guohong Li; Yanbo Pan & Keqin Zhang (2006). "Acanthocytes of Stropharia rugosoannulata ... the fungus produces unique spiny cells called acanthocytes which are able to immobilise and digest the nematodes. See ...
Hemangiosarcoma
... and acanthocytes in the blood smear; and leukocytosis with neutrophilia, left shift, and monocytosis. A definitive diagnosis ...
McLeod syndrome
... is one of only a few disorders in which acanthocytes may be found on the peripheral blood smear. Blood ... McLeod females have only occasional acanthocytes and very mild hemolysis; the lesser severity is thought to be due to X ...
Stropharia caerulea
The fungus produces acanthocytes, spiny cells produced on short branches on the mycelium. There are a few greenish Stropharia ... ISBN 978-0-19-957112-3. Farr DA (1980). "The acanthocyte, a unique cell type in Stropharia (Agaricales)". Mycotaxon. 11 (1): ...
Spur cell hemolytic anemia
This condition also creates rough or thorny projections on the erythrocyte named acanthocytes. List of hematologic conditions ...
Neuroacanthocytosis
... spiculated red blood cells called acanthocytes. The 'core' neuroacanthocytosis syndromes, in which acanthocytes are a typical ... Acanthocytes are spiculated red blood cells and can be caused by altered distribution of membrane lipids or membrane protein/ ... Acanthocytes are nearly always present in these conditions and they share common clinical features. Some of these features are ... Acanthocytes are seen less frequently in other conditions including Huntington's disease-like syndrome 2 (HDL2) and ...
Stropharia
Members of the core clade of Stropharia are characterized by crystalline acanthocytes among the hyphae of the mycelium and that ... have now been classified as separate genera by some authors for species lacking acanthocytes. Two examples of the ...
Crenation
Acanthocytes and echinocytes may arise from abnormalities of the cell membrane lipids or proteins, or from other disease ... The crenations may be either large, irregular spicules of acanthocytes, or smaller, more numerous, regularly irregular ...
Protostropharia
Stercophila). It is differentiated from Stropharia by production of astrocystidia on its mycelium rather than by acanthocytes ... The name Protostropharia refers to the less anatomically complex astrocystidia (Greek proto-) as compared to the acanthocytes ...
Protostropharia alcis
... a genus circumscribed to contain Stropharia species characterized by the formation of astrocystidia rather than acanthocytes on ...
Zieve's syndrome
It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinaemia (excessive blood lipoprotein), ...
Strophariaceae
Members of the core clade of Stropharia are characterized by crystalline acanthocytes among the hyphae that make up the ...
Epidermis
... and not to be confused with acanthocytes). It implies increased thickness of the Malpighian layer (stratum basale and stratum ...
Frank Bassen
Treatment includes vitamin E. Characteristics of the syndrome include the presence of acanthocytes (burr-cell malformation of ...
Abraham Kornzweig
Treatment includes vitamin E. Characteristics of the syndrome include the presence of acanthocytes (burr-cell malformation of ...
Normocytic anemia
... acanthocytes for pyruvate kinase deficiency or McLeod phenotype, sickle cells for sickle cell anemia, spherocytes for immune- ...
List of MeSH codes (A11)
... acanthocytes MeSH A11.118.290.330.315 - erythrocyte inclusions MeSH A11.118.290.330.315.335 - heinz bodies MeSH A11.118.290.330 ... acanthocytes MeSH A11.443.240.330.531 - megaloblasts MeSH A11.443.240.330.802 - spherocytes MeSH A11.443.250.200 - ...
List of MeSH codes (A15)
... acanthocytes MeSH A15.145.229.334.330.340 - erythrocyte inclusions MeSH A15.145.229.334.330.340.335 - heinz bodies MeSH A15.145 ...
Protostropharia semiglobata
... new genus circumscribed to contain Stropharia species characterized by the formation of astrocystidia rather than acanthocytes ...
Poikilocytosis
Acanthocytes or Spur/Spike cells Codocytes or Target cells Echinocytes and Burr cells Elliptocytes and Ovalocytes Spherocytes ...
Acanthocyte
... : Presented by the University of Virginia Acanthocytes at the US National Library of Medicine Medical Subject ... Acanthocyte-like cells may be found in hypothyroidism, after splenectomy, and in myelodysplasia. Acanthocytes should be ... Spur cells may refer synonymously to acanthocytes, or may refer in some sources to a specific subset of 'extreme acanthocytes' ... Acanthocyte (from the Greek word ἄκανθα acantha, meaning 'thorn'), in biology and medicine, refers to an abnormal form of red ...
Acanthocyte (mycology)
In mycology, acanthocyte refers to stellate cells found on the hyphae of fungi of the genus Stropharia. Recent work, published ... Hong Luo; Xuan Li; Guohong Li; Yanbo Pan; Keqin Zhang (2006). "Acanthocytes of Stropharia rugosoannulata Function as a Nematode ...
Acanthocyte (disambiguation)
Look up acanthocyte in Wiktionary, the free dictionary. Acanthocyte is a form of red blood cell that has a spiked cell membrane ... Acanthocyte may also refer to: Acanthocyte (mycology), stellate cells found on the hyphae of fungi of the genus Stropharia ... a genus of spiders This disambiguation page lists articles associated with the title Acanthocyte. If an internal link led you ... thickening of the skin not connected to acanthocytes Acanthoctenus, ...
Acanthocyte - Wikipedia
Acanthocyte: Presented by the University of Virginia Acanthocytes at the US National Library of Medicine Medical Subject ... Acanthocyte-like cells may be found in hypothyroidism, after splenectomy, and in myelodysplasia. Acanthocytes should be ... Spur cells may refer synonymously to acanthocytes, or may refer in some sources to a specific subset of extreme acanthocytes ... Acanthocyte (from the Greek word ἄκανθα acantha, meaning thorn), in biology and medicine, refers to an abnormal form of red ...
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DIFFERENTIAL DIAGNOSIS OF ACANTHOCYTES - ABIBLO
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Erythrocyte Morphology on Peripheral Smear
Acanthocyte, Spur Cell, Dacryocyte, Howell-Jolly, Target Cell, Basophilic Stippling, Sideroblast, Cabots Ring Bodies. ... Acanthocytes, cells spur, acanthocyte, acanthocytes, acanthrocyte, spur cell, Acanthocyte (morphologic abnormality), Spur cell ... finding), Acanthocyte, Acanthrocyte, Crenated cell, Spur cell, Acanthocyte (cell), Spiny prickle cell, Acanthocyte [dup] ( ... Ontology: Acanthocytes. (C0000886) Definition (NCI) Erythrocytes with protoplasmic projections giving the cell a thorny ...
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Acanthocytes, or spur cells, are spiculated red cells with a few projections of varying size and surface distribution that can ... Acanthocytes should be distinguished from echinocytes, or burr cells, that also demonstrate multiple small projections but ... Shape determinants of McLeod acanthocytes. Journal of Membrane Biology. 1989 Mar 1;107(3):213-8. ...
Formation of acanthocytes2
- This causes abnormalities of membrane of RBC causing remodeling in spleen and formation of acanthocytes. (wikipedia.org)
- The formation of acanthocytes depends on the alteration of the lipid composition and fluidity of the red cell membrane. (askhematologist.com)
Spur cell1
- The erythrocyte in the center, had undergone the process of crenation, whereupon, it developed a number of cell wall projections, thereby, transforming it into what is termed an acanthocyte, or spur cell. (cdc.gov)
Schistocytes1
- Acanthocytes or schistocytes may be seen on the blood film. (mhmedical.com)
Poikilocytosis1
- The peripheral blood smear showed numerous acanthocytes and poikilocytosis shown below. (labmedicineblog.com)
Echinocytes3
- Acanthocytes should be distinguished from echinocytes, which are also called 'burr cells', which although crenated are dissimilar in that they have multiple, small, projecting spiculations at regular intervals on the cell membrane. (wikipedia.org)
- Spiculated cells (echinocytes and acanthocytes) and target cells. (wikipedia.org)
- Acanthocytes should be distinguished from echinocytes, or burr cells, that also demonstrate multiple small projections but these are uniformly distributed on the red cell surface. (labmedicineblog.com)
Dysmorphic1
- Besides doughnut-shaped ring forms with smooth or wavy structures, acanthocytes are a special form of dysmorphic RBC. (sysmex.no)
Cells9
- Spur cells may refer synonymously to acanthocytes, or may refer in some sources to a specific subset of 'extreme acanthocytes' that have undergone splenic modification whereby additional cell membrane loss has blunted the spicules and the cells have become spherocytic ('spheroacanthocyte'), as seen in some patients with severe liver disease. (wikipedia.org)
- Acanthocyte-like cells may be found in hypothyroidism, after splenectomy, and in myelodysplasia. (wikipedia.org)
- Acanthocytes should also be distinguished from keratocytes, also called 'horn cells' which have a few very large protuberances. (wikipedia.org)
- Acanthocytes (also called spur cells) are spiculated cells with irregular, pointed or clublike projections that are unevenly distributed on the cell surface. (askhematologist.com)
- Acanthocytes, or spur cells, are spiculated red cells with a few projections of varying size and surface distribution that can be seen in a variety of clinical conditions including CGD with McLeod red cell phenotype. (labmedicineblog.com)
- and thorny-looking red cells (ACANTHOCYTES). (wakehealth.edu)
- Ultrastructure and development of acanthocytes, specialized cells in Stropharia rugosoannulata , revealed by scanning electron microscopy (SEM) and cryo-SEM. (im.ac.cn)
- Acanthocytes (spur cells) are spiculated red cells with a few projections of varying size and surface distribution. (askhematologist.com)
- Occasionally increased numbers of small red blood cells ( microcytes ) or spiculated (spike-shaped) red blood cells ( acanthocytes ) may be found in association with liver disease. (santaclarapethospital.com)
Liver disease1
- Acanthocytes in two patients with liver disease. (askhematologist.com)
Cell1
- Acanthocyte (from the Greek word ἄκανθα acantha, meaning 'thorn'), in biology and medicine, refers to an abnormal form of red blood cell that has a spiked cell membrane, due to thorny projections. (wikipedia.org)
RBCs3
- Poikilocytes refer to RBCs with specific morphologies (e.g., acanthocytes, spherocytes) as well as abnormally shaped RBCs that do not fit into a specific category. (vetbloom.com)
- BACKGROUND: Chorea-acanthocytosis (ChAc) is a rare hereditary neurodegenerative disease with deformed red blood cells (RBCs), so-called acanthocytes, as a typical marker of the disease. (uni.lu)
- RESULTS: The mechano-morphological characterization of RBCs from two ChAc patients in an off-label treatment with dasatinib revealed differences in the ESR and the acanthocyte count during and after the treatment period, which could not directly be related to each other. (uni.lu)
Abnormal2
- Acanthocytes are abnormal red blood cells that look like they have little spikes sticking out of them. (zerocarbzen.com)
- What are their coping habits and that if antibiotics are required to monitor the e ectiveness o cognitive or unctional dystonia include an abnormal eeg, particularly i acanthocytes are identi ed in these populations may be affected and unaffected breasts ii frequent pumping hand expression of most procoagulant proteins are rarely effective as prophylaxis in setting up treatment goals for this patient have. (umaine.edu)
Erythrocyte1
- The erythrocyte in the center, had undergone the process of crenation, whereupon, it developed a number of cell wall projections, thereby, transforming it into what is termed an acanthocyte, or spur cell. (cdc.gov)
Pemphigus2
- Pemphigus foliaceus pustules have acanthocytes , which are rounded, darkly stained cells with prominent nuclei, and neutrophils . (wikivet.net)
- Acanthocytes are rare in pemphigus vulgaris cases as the clefting is deep. (wikivet.net)
Casts2
- Lipid casts and free fat, numerous acanthocytes, no cellular casts. (glomcon.org)
- This can help the detection of: Hyaline casts Ghost red blood cells, Squamous epithelial cells, Acanthocyte and yeast differentiation, Separation of cocci and rod bacteria, More clearly defined crystals. (menarinidiag.co.uk)
Erythrocytes1
- A relative measurement (ratio or percentage) of acanthocytes to all erythrocytes in a biological specimen. (immport.org)
Separation1
- Autoantibodies are directed at the interepidermal cell antigens and cement substances and cause epidermal cell separation and cell rounding (acanthocyte formation). (wikivet.net)
Liver2
- Acanthocytes may also be seen in dogs with liver disease or lipid metabolopathy. (vetbloom.com)
- central versus Heavy article lifelong liver for mellitus in acanthocytes with HIV level in Botswana: a been, likely, left photograph. (lakesinclair.org)
Blood1
- A rare condition in which the majority of the red blood cells are acanthocytes. (definithing.com)
Found1
- Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome) and hypobetalipoproteinemia leading to vitamin E malabsorption. (nih.gov)
Treatment1
- CONCLUSIONS: The results increase our understanding of the role of acanthocytes and their associated properties in the ESR, but the data are too sparse to answer the question of whether the ESR is a suitable biomarker for treatment success, whereas a correlation between hematological and neuronal phenotype is still subject to verification. (uni.lu)