Degmacyte

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A degmacyte or bite cell is an abnormally shaped mature red blood cell with one or more semicircular portions removed from the cell margin, known as "bites". [1] [2] These "bites" result from the mechanical removal of denatured hemoglobin during splenic filtration as red cells attempt to migrate through endothelial slits from splenic cords into the splenic sinuses. [3] Bite cells are known to be a result from processes of oxidative hemolysis, such as Glucose-6-phosphate dehydrogenase deficiency, in which uncontrolled oxidative stress causes hemoglobin to denature and form Heinz bodies. [4] Bite cells can contain more than one "bite." The "bites" in degmacytes are smaller than the missing red blood cell fragments seen in schistocytes.[ citation needed ]

Contents

BITE CELLS BITE CELLS.jpg
BITE CELLS

Degmacytes usually appear smaller, denser, and more contracted than a normal red blood cell due to the bites. [5] The appearance of the "bites" in red blood cells may vary in number, smoothness, and size. [5] These cells can also exhibit other peripheral effects. [2]

Blister cell

In blister cells, an outer rim of cytoplasm is maintained, and when this ruptures, the resulting bite cells have horn-like projections. Blister cells appear as red blood cells containing a peripherally located vacuole. [6]

Causes

Bite cells are caused by G6PD deficiency, NADPH deficiency, thalassemia, [2] glutathione synthase deficiency, [6] and other red-cell enzymopathies involving the pentose phosphate shunt, oxidative drugs and unstable hemoglobins. [6] People receiving large quantities of oxidative drugs such as dapsone or sulfasalazine are more prone to have degmacytes. [1] The underlying cause of degmacytes is a result of the splenic macrophages removing Heinz bodies from the membrane of red blood cells, which results in a "bitten" appearance of the cell. [7] [8] [9]

Diagnosis

The diagnosis of degmacytes is performed by using a peripheral blood smear to analyze the red blood cells in the blood. The degmacyte look similar to a "bitten apple", which is their defining feature. However, bite cells may be difficult to distinguish from helmet cells, a fragmented red blood cell, due to the similarity in shape. [6] Confirming the presence of degmacytes in patients likely indicates a form of red blood cell oxidant injury or hemolysis. [10]

Treatment and Prevention

Bite cells are primarily formed due to a G6PD deficiency combined with oxidative stress or other trigger, such as fava beans and certain antimalarial or sulfa drugs. Patients may attempt to decrease exposure to hypoxia, which can then lead to hemolytic disorders. If one is not G6PD deficient, lessening the use of oxidative triggers may help prevent the formation of degmacytes. [2]

As there are currently no means of reversing red blood cell damage and the formation of degmacytes, treatment options are limited to blood transfusions. [6]

Etymology

From Ancient Greek δῆγμα (dêgma): “to bite” + -cyte.

Related Research Articles

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Hemolysis or haemolysis, also known by several other names, is the rupturing (lysis) of red blood cells (erythrocytes) and the release of their contents (cytoplasm) into surrounding fluid. Hemolysis may occur in vivo or in vitro.

<span class="mw-page-title-main">Anemia</span> Medical condition

Anemia or anaemia is a blood disorder in which the blood has a reduced ability to carry oxygen due to a lower than normal number of red blood cells, a reduction in the amount of hemoglobin or hemoglobin abnormalities. The name is derived from Ancient Greek: ἀναιμία anaimia, meaning 'lack of blood', from ἀν- an-, 'not' and αἷμα haima, 'blood'. When anemia comes on slowly, the symptoms are often vague, such as tiredness, weakness, shortness of breath, headaches, and a reduced ability to exercise. When anemia is acute, symptoms may include confusion, feeling like one is going to pass out, loss of consciousness, and increased thirst. Anemia must be significant before a person becomes noticeably pale. Symptoms of anemia depend on how quickly hemoglobin decreases. Additional symptoms may occur depending on the underlying cause. Preoperative anemia can increase the risk of needing a blood transfusion following surgery. Anemia can be temporary or long term and can range from mild to severe.

<span class="mw-page-title-main">Spherocytosis</span> Medical condition

Spherocytosis is the presence of spherocytes in the blood, i.e. erythrocytes that are sphere-shaped rather than bi-concave disk shaped as normal. Spherocytes are found in all hemolytic anemias to some degree. Hereditary spherocytosis and autoimmune hemolytic anemia are characterized by having only spherocytes.

<span class="mw-page-title-main">Glucose-6-phosphate dehydrogenase deficiency</span> Medical condition

Glucose-6-phosphate dehydrogenase deficiency (G6PDD), which is the most common enzyme deficiency worldwide, is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those who are affected have no symptoms. Following a specific trigger, symptoms such as yellowish skin, dark urine, shortness of breath, and feeling tired may develop. Complications can include anemia and newborn jaundice. Some people never have symptoms.

<span class="mw-page-title-main">Hereditary spherocytosis</span> Medical condition

Hereditary spherocytosis (HS) is a congenital hemolytic disorder, wherein a genetic mutation coding for a structural membrane protein phenotype leads to a spherical shaping of erythrocytic cellular morphology. As erythrocytes are sphere-shaped (spherocytosis), rather than the normal biconcave disk-shaped, their morphology interferes with these cells' abilities to be flexible during circulation throughout the entirety of the body - arteries, arterioles, capillaries, venules, veins, and organs. This difference in shape also makes the red blood cells more prone to rupture under osmotic and/or mechanical stress. Cells with these dysfunctional proteins are degraded in the spleen, which leads to a shortage of erythrocytes resulting in hemolytic anemia.

<span class="mw-page-title-main">Paroxysmal nocturnal hemoglobinuria</span> Medical condition

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to deficiency of the red blood cell surface protein DAF, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system, the red blood cell destruction (hemolysis) is considered an intravascular hemolytic anemia. Other key features of the disease, such as the high incidence of venous blood clot formation, are incompletely understood.

<span class="mw-page-title-main">Hemolytic anemia</span> Medical condition

Hemolytic anemia or haemolytic anaemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels or elsewhere in the human body (extravascular). This most commonly occurs within the spleen, but also can occur in the reticuloendothelial system or mechanically. Hemolytic anemia accounts for 5% of all existing anemias. It has numerous possible consequences, ranging from general symptoms to life-threatening systemic effects. The general classification of hemolytic anemia is either intrinsic or extrinsic. Treatment depends on the type and cause of the hemolytic anemia.

<span class="mw-page-title-main">Pyruvate kinase deficiency</span> Medical condition

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<span class="mw-page-title-main">Codocyte</span> Type of red blood cell

Codocytes, also known as target cells, are red blood cells that have the appearance of a shooting target with a bullseye. In optical microscopy these cells appear to have a dark center surrounded by a white ring, followed by dark outer (peripheral) second ring containing a band of hemoglobin. However, in electron microscopy they appear very thin and bell shaped. Because of their thinness they are referred to as leptocytes. On routine smear morphology, some people like to make a distinction between leptocytes and codocytes- suggesting that in leptocytes the central spot is not completely detached from the peripheral ring, i.e. the pallor is in a C shape rather than a full ring.

Maxwell Myer Wintrobe was an Austrian-born American physician who was a 20th-century authority in the medical field of hematology. His 1942 textbook on hematology, Clinical Hematology, was the first dedicated work in the field and he contributed to the diagnostic approach of anemia and copper metabolism, amongst many other achievements.

Heinz bodies are inclusions within red blood cells composed of denatured hemoglobin. They are not visible with routine blood staining techniques, but can be seen with supravital staining. The presence of Heinz bodies represents damage to hemoglobin and is classically observed in G6PD deficiency, a genetic disorder that causes hemolytic anemia. In veterinary medicine, Heinz bodies may be seen following the consumption of foods containing thiosulfate and propylene glycol compounds by cats, dogs and certain primates.

The term macrocytic is from Greek words meaning "large cell". A macrocytic class of anemia is an anemia in which the red blood cells (erythrocytes) are larger than their normal volume. The normal erythrocyte volume in humans is about 80 to 100 femtoliters. In metric terms the size is given in equivalent cubic micrometers. The condition of having erythrocytes which are too large, is called macrocytosis. In contrast, in microcytic anemia, the erythrocytes are smaller than normal.

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<span class="mw-page-title-main">6-phosphogluconate dehydrogenase deficiency</span> Medical condition

6-Phosphogluconate dehydrogenase deficiency, or partial deficiency, is an autosomal hereditary disease characterized by abnormally low levels of 6-phosphogluconate dehydrogenase (6PGD), a metabolic enzyme involved in the Pentose phosphate pathway. It is very important in the metabolism of red blood cells (erythrocytes). 6PDG deficiency affects less than 1% of the population, and studies suggest that there may be race variant involved in many of the reported cases. Although it is similar, 6PDG deficiency is not linked to glucose-6-phosphate dehydrogenase (G6PD) deficiency, as they are located on different chromosomes. However, a few people have had both of these metabolic diseases.

<span class="mw-page-title-main">Basophilic stippling</span> Cytoplasmic granules in basophils

Basophilic stippling, also known as punctate basophilia, is the presence of numerous basophilic granules that are dispersed through the cytoplasm of erythrocytes in a peripheral blood smear. They can be demonstrated to be RNA. They are composed of aggregates of ribosomes; degenerating mitochondria and siderosomes may be included in the aggregates. In contrast to Pappenheimer bodies, they are negative with Perls' acid ferrocyanide stain for iron. Basophilic stippling is indicative of disturbed erythropoiesis. It can also be found in some normal individuals.

<span class="mw-page-title-main">Elliptocyte</span> Abnormal red blood cell

Elliptocytes, also known as ovalocytes or cigar cells, are abnormally shaped red blood cells that appear oval or elongated, from slightly egg-shaped to rod or pencil forms. They have normal central pallor with the hemoglobin appearing concentrated at the ends of the elongated cells when viewed through a light microscope. The ends of the cells are blunt and not sharp like sickle cells.

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References

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