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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. A similar term is spur cells. Often they may be confused with echinocytes or
schistocytes A schistocyte or schizocyte (from Greek language, Greek for "divided" and for "hollow" or "cell") is a cell fragmentation, fragmented part of a red blood cell. Schistocytes are typically irregularly shaped, jagged, and have two pointed ends. S ...
. Acanthocytes have coarse, irregularly spaced, variably sized
crenation Crenation (from modern Latin ''crenatus'' meaning "scalloped or notched", from popular Latin ''crena'' meaning "notch") in botany and zoology, describes an object's shape, especially a leaf or shell, as being round-toothed or having a scalloped ed ...
s, resembling many-pointed stars. They are seen on blood films in abetalipoproteinemia, liver disease, chorea acanthocytosis, McLeod syndrome, and several inherited neurological and other disorders such as neuroacanthocytosis, anorexia nervosa, infantile
pyknocytosis Pyknocytosis is a hematologic state characterized by the presence of pyknocytes in the blood. Pyknocytes are red blood cells that appear distorted, irregular and small with abnormal projections and would typically be identified by a medical scientis ...
,
hypothyroidism Hypothyroidism (also called ''underactive thyroid'', ''low thyroid'' or ''hypothyreosis'') is a disorder of the endocrine system in which the thyroid gland does not produce enough thyroid hormone. It can cause a number of symptoms, such as po ...
, idiopathic neonatal hepatitis, alcoholism, congestive splenomegaly,
Zieve syndrome Zieve's syndrome is an acute metabolic condition that can occur during withdrawal from prolonged heavy alcohol use. It is defined by hemolytic anemia (with spur cells and acanthocytes), hyperlipoproteinaemia (excessive blood lipoprotein), jaundice ...
, and chronic granulomatous disease.


Usage

Spur cells may refer
synonym A synonym is a word, morpheme, or phrase that means exactly or nearly the same as another word, morpheme, or phrase in a given language. For example, in the English language, the words ''begin'', ''start'', ''commence'', and ''initiate'' are all ...
ously 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.Mentzer WC. Spiculated cells (echinocytes and acanthocytes) and target cells. UpToDate (release: 20.12- C21.4

/ref> Acanthocytosis can refer generally to the presence of this type of crenated red blood cell, such as may be found in severe cirrhosis or pancreatitis, but can refer specifically to abetalipoproteinemia, a clinical condition with acanthocytic red blood cells, neurologic problems and steatorrhea. This particular cause of acanthocytosis (also known as abetalipoproteinemia, apolipoprotein B deficiency, and Bassen-Kornzweig syndrome) is a rare, genetically inherited, autosomal recessive condition due to the inability to fully digest dietary fats in the intestines as a result of various mutations of the
microsomal triglyceride transfer protein Microsomal triglyceride transfer protein large subunit is a protein that in humans is encoded by the ''MTTP'' gene. MTP encodes the large subunit of the heterodimeric microsomal triglyceride transfer protein. Protein disulfide isomerase (PDI) c ...
(MTTP) gene.


Pathophysiology

Acanthocytes arise from either alterations in membrane lipids or structural proteins. Alterations in membrane lipids are seen in abetalipoproteinemia and liver dysfunction. Alteration in membrane structural proteins are seen in neuroacanthocytosis and McLeod syndrome. In liver dysfunction, apolipoprotein A-II deficient lipoprotein accumulates in plasma causing increased cholesterol in RBCs. This causes abnormalities of membrane of RBC causing remodeling in spleen and formation of acanthocytes. In abetalipoproteinemia, there is deficiency of lipids and vitamin E causing abnormal morphology of RBCs.


Differential diagnoses

The diagnosis of acanthocytosis should be differentiated from: acute or chronic anemia, hepatitis A, B, and C, hepatorenal syndrome, hypopituitarism, malabsorption syndromes, and malnutrition. Acanthocytosis secondary to malnourishment, such as anorexia nervosa and cystic fibrosis, remits with resolution of the nutritional deficiency. Acanthocyte-like cells may be found in hypothyroidism, after splenectomy, and in myelodysplasia. 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. Burr cells usually imply uremia, but are seen in many conditions, including mild hemolysis in hypomagnesemia and hypophosphatemia, hemolytic anemia in long-distance runners, and pyruvate kinase deficiency. Burr cells can also arise in vitro due to elevated pH, blood storage, ATP depletion, calcium accumulation, and contact with glass. Acanthocytes should also be distinguished from keratocytes, also called 'horn cells' which have a few very large protuberances.


See also

*
List of hematologic conditions :''This is an incomplete list, which may never be able to satisfy certain standards for completion.'' There are many conditions of or affecting the human hematologic system—the biological system that includes plasma, platelets, leukocytes, and ...


References


External links


Acanthocyte
Presented by the University of Virginia * {{Abnormal clinical and laboratory findings Abnormal clinical and laboratory findings for RBCs Histopathology