Spur cells are susceptible to splenic sequestration and destruction, and patients are generally refractory to blood transfusions because transfused cells rapidly acquire. The erythrocytes have numerous spicules irregularly distributed over the cell surface. Feder jn, gnirke a, thomas w, tsuchihashi z, ruddy da, basava a, dormishian f, domingo r, jr, ellis mc, fullan a, et al. Liver transplantation constitutes the only therapeutic tool. The sera and red cells of three patients with severe liver disease and spur. The symptoms of spur cell anemia are related to the anemia and to the underlying disease. True iron accumulation may occur in spur cell haemolytic anaemia due to increased iron absorption and the repeated blood transfusions. Because dyslipidemia is related to the development of spur cells, sca was previously treated with plasmapheresis. Spur cell anemia acanthocytosis complicating idiopathic. Peripheral blood smear demonstrated marked anisopoikilocytosis with acanthocytes spur cells. Reversibility of spur cell anemia blood american society. A liver biopsy revealed cirrhosis with marked pericellular fibrosis and iron overload picture 2. Severe mixedtype iron overload in alcoholic cirrhosis related to advanced spur cell anemia vincent zimmer, jorg bittenbring, peter fries, frank lammert department of medicine ii, department of medicine i, clinic of diagnostic and interventional radiology.
Rbcs with spike like projections spur cells, target cells and rbc fragments are prominent. Spur cell anemia acanthocytosis complicating idiopathic he. Spur cell anemia is a type of hemolytic anemia that can develop in patients with advanced liver disease. Images in clinical medicine from the new england journal of medicine spurcell anemia. Spur cell anemia is a type of hemolytic anemia that can develop in patients with liver failure. Abetalipoproteinemia, acanthocyte, artifact, burr cell, echinocyte, liver disease, maha, spur cell. Spur cell hemolytic anemia in liver failure murphy.
Spur cell anemia is a type of hemolytic anemia that. Spur cell anemia article about spur cell anemia by the free. Oct 20, 2011 a diagnosis of spur cell anemia was made. Finally, our case report shows for the first time a very rapid reversion of sca after lt with prompt decline of hemolysis indexes already within 24 hours.
Spur cell anemia is a serious disorder in cirrhotic patients and is associated with poor prognosis. Plasmapheresis for spur cell anemia in a patient with alcoholic. The spur cell patients erythrocyte cpl molar ratios were clearly greater than either target cell patients or normal controls 1. Spur cell anemia is a hemolytic anemia seen in severe alcoholic cirrhosis that is characterized by unusual morphology and a decreased ratio of phospholipids to cholesterol in the erythrocyte membrane. Soni and others published spur cell anemia in wilsons disease. It closely resembles zieve syndrome, which can present similarly and needs to be. Spur cell anemia is an acquired hemolytic anemia, characterized by an increased percentage of abnormally shaped erythrocytes that are known as acanthocytes. Letter to the editors spur cell anemia as a cause of. Abnormal phospholipid metabolism in spur cell anemia. Hemolytic spur cellanemia hsca is due to lipid disturbances of the erythrocyte membrane and may develop in. Smith et al 11 coined the term spur cell anaemia, describing a haemolytic anaemia with red cell morphology resembling acanthocytes in patients with alcoholic cirrhosis. Review the causal information about the various more general categories of medical conditions. Complete resolution of spur cell anemia has been reported after liver transplantation.
Successful combination therapyflunarizine, pentoxifylline. If anemia is consistent with iron deficiency in a child six to 36 months of age with low mean corpuscular volume and elevated red blood cell distribution width, it is reasonable to try oral iron. Both patients spur cell anemia resolved and target cells became the major abnormal erythrocyte form. Spur cell anemia is a recognized, though rare, complication of alcoholic liver disease and indeed the patient had a regular alcohol intake of up to 50 g daily. Jul 12, 2011 of spur cells and decreased red cell survival, resulting in hemolytic anemia. Furthermore, the prevalence varies according to the different criteria used to diagnose the disease. Spurcell anemia is an acquired ltentolytic process as sociated with abnormally shaped erythrocytes, acantho cytes, which may occur in patients with severe hepatocellu tar dysfimction, usually alcoholic cirrhosis,m since the first case report of spurcell anemia, in 1964, the major em phasis in the literature concerning this entity has been. We report a case of spur cell anemia with alcoholic liver cirrhosis successfully cured by living liver transplantation ldlt. 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. Spur cell haemolytic anaemia sca is a form of anaemia that can be seen in patients with severely impaired liver function or advanced cirrhosis.
Plasmapheresis for spur cell anemia in a patient with. We present an unusual case of a young patient with hemochromatosis, alcohol abuse, decompensated cirrhosis, and spur cell anemia who had a spontaneous resolution of the spur cell anemia after orthotopic liver transplantation. The treatment options for sca secondary to cirrhosis are limited. Spur cell anemia is a type of hemolytic anemia that can develop in patients with advanced liver disease especially alcoholic cirrhosis. Other select causes of acquired hemolysis not discussed in this article include splenomegaly, endstage liver diseasespur cell acan thocyte hemolytic anemia, paroxysmal cold hemoglobinuria, paroxysmal nocturnal hemoglobinuria, insect stings, and spider bites. Hemolysis also represents a common cause of anemia in patients with liver disease, especially of alcoholic cause, and is usually attributed to spurcell anemia. Saarland university medical center, homburg, germany. Sep 18, 2019 abstinence from alcohol is also the best preventive measure for spur cell anemia. Incidence and correlation with disease severity and survival aim. Spur cells and spur cell anemia in hospitalized patients. Spur cell anemia definition of spur cell anemia by the free. Anemia with spur cells journal of clinical investigation. Spur cell anemia has been described to occur in several conditions, including cirrhosis.
The prognosis of spur cell anemia is usually extremely poor, however, we describe here a patient with spur cell anemia who was successfully treated with. Spur cell hemolytic anemia, is a form of hemolytic anemia that results secondary to severe impaired liver function or cirrhosis, and is mostly seen in alcoholics. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Feder jn, gnirke a, thomas w, tsuchihashi z, ruddy da. Sickle cell patient 36 year old black male diagnosed with sickle cell anemia at age 2 formerly had 1 painful crisis each year, but recently has had 3 4 per year last october, acute chest syndrome. Spur cell anemia associated with alcoholic cirrhosis. Spur cell anemia is an acquired ltentolytic process as sociated with abnormally shaped erythrocytes, acantho cytes, which may occur in patients with severe hepatocellu tar dysfimction, usually alcoholic cirrhosis,m since the first case report of spur cell anemia, in 1964, the major em phasis in the literature concerning this entity has been. Spur cell anemia sca is a cause of hemolytic anemia in patients with alcoholic liver cirrhosis. Spur cell anemia is a rare type of hemolytic anemia with a grave prognosis. Finally, our case report shows for the first time a very rapid reversion of sca after lt with prompt. The world health organization has defined anemia as a hemoglobin concentration below 7. A 65yearold male developed a rapidly progressive disease characterized by severe hemolysis, with spur cells acanthocytes and liver disease. It is related to abnormal cholesterol loading of the red blood cell membrane. Spur cells form as a consequence of abnormal cholesterol to phospholipid ratios in red cell membranes caused by decreased apolipoproteins in the setting of liver cirrhosis 35.
Spur cell anemia definition of spur cell anemia by medical. Spurcell anemia definition of spurcell anemia by the free. Spur cell anemia is an insufficiently investigated entity and its clinical importance has not been documented yet. Cooper 1 thorndike memorial laboratory and 2nd and 4th harvard medical services, boston city hospital, and the department of medicine, harvard medical school, boston, massachusetts 02118. Spur cell anemia is an acquired hemolytic process characterized by multiple acanthocytes in the peripheral blood smear. Spur cell acanthocyte and burr cell echinocyte comparision anemia of renal disease tags.
Spur cell anemia, a hemolytic anemia observed in patients with alcoholic cirrhosis, is characterized by unusual erythrocyte morphology and an increased ratio of free cholesterol to phospholipid in the erythrocyte membrane. University of washington, harborview medical center, seattle, washington. Such a condition is caused by a deficient number of erythrocytes red blood cells, an abnormally low level of hemoglobin in the individual cells, or both these conditions simultaneously. We present an unusual case of a young patient with hemochromatosis, alcohol abuse, decompensated cirrhosis, and spur cell anemia who had a spontaneous resolution of the.
These patients are poorly responsive to rbc transfusions, as even transfused cells undergo membrane changes when circulating through the liver, and are thus subject to decreased survival. Spur cell anemia is found in endstage alcoholic liver disease. Although the alcohol intake could have contributed to the formation of spur cells, the possible association with hemochromatosis should be considered. Spur cells have a bizarre, spiculated morphology and must comprise at least 20% of the erythrocytes seen on peripheral smear to be consistent with a diagnosis of. There are few reports on its prevalence and survival. A case of spur cell anemia resolved by living donor liver. Surface morphology of platelets in spurcell anemia. Spur cell anaemia and hepatic iron stores in patients with. Chronic liver disease impairs the livers ability to esterify cholesterol, causing free cholesterol to bind to the red cell membrane, increasing its surface area without increasing its volume. Spur cell anemia is an acquired hemolytic anemia, which may occur in patients with severe liver dysfunction, especially in those with alcoholic cirrhosis.
It is considered by some to be a variant of spur cell anemia. Spur cell anemia in the setting of progressive liver. Abstinence from alcohol is also the best preventive measure for spur cell anemia. We hypothesized that defective phospholipid repair may contribute to the red blood cell rbc phospholipid abnormalities of spur cell anemia. Spurcell anaemia in a patient with cirrhosis journal of hepatology. Red blood cells carry oxygen to the body and are normally shaped like a disc. To view the article, select the view print version pdf link above. In spur cell anemia, the hemoglobin level usually falls to less than 10 gdl and, occasionally, to levels as low as 5 gdl. Rods placed in both arms and legs show video treatment. Severe mixedtype iron overload in alcoholic cirrhosis. A 48 yearold man with cryptogenic cirrhosis was admitted to the hospital because of malaise and progressive. Spur cell anemia as a cause of unconjugated hyperbilirubinemia after liver transplantation and its resolution after retransplantation received october 25, 2010.
True iron accumulation may occur in spur cell haemolytic anaemia due to increased. Anemia in alcoholic liver disease has a broad spectrum of differential diagnosis. Resolution of spur cell anemia with liver transplantation. The morphologic changes of spur cells are caused by accumulation of excess membrane cholesterol, leading to an impaired deformability and consequently a reduced red cell survival. The patient was a 31yearold woman with a history of ethanol abuse. One of the esoteric pathology that should be considered is spur cell anemia in end stage liver disease patients. The patients clinical condition rapidly deteriorated, and she died of progressive liver failure. Spur cell anemia in the setting of progressive liver allograft failure. Santopaolo 21 rapid reversion of spur cell anemia after liver transplantation med ase rep rev, 2019 doi. Rapid reversion of spur cell anemia after liver transplantation. Spurcell anemia definition of spurcell anemia by medical. Because dyslipidemia is related to the development.
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