These changes are associated with development of spur cells and decreased red cell. Conclusionsthe hfe mutation was not present in these patients with advanced ald and heavy iron loading. The world health organization has defined anemia as a hemoglobin concentration below 7. Studies performed in an attempt to determine the cause for the abnormal shape of the erythrocytes demonstrated that the patients serum could induce spur formation in normal compatible cells. The occurrence of spur cell anaemia in the course of cirrhosis is rare. Spurcell anemia definition of spurcell anemia by the free. Images in clinical medicine from the new england journal of medicine spurcell anemia. The reticulocyte count is used to assess the appropriateness of the bone marrow response to anemia.
Hemolytic anaemiaclassificationhereditary intracorpuscular hemoglobinopathies hbs,thalassemias enzymopathies g6pd def. 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. A 48 yearold man with cryptogenic cirrhosis was admitted to the hospital because of malaise and progressive. Letter to the editors spur cell anemia as a cause of. Spur cells are susceptible to splenic sequestration and destruction, and patients are generally refractory to blood transfusions because transfused cells rapidly acquire. A 52yearold japanese man with sca associated with alcoholic liver cirrhosis childpugh c underwent two rounds of plasmapheresis. Red blood cells carry oxygen to the body and are normally shaped like a disc. In between episodes of sickling, people with scd are normally well. 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. Approximately 1% of circulating rbcs are removed daily. This result from single point replacement of glutamine by valine at position 6 of. Review the causal information about the various more general categories of medical conditions.
Reversibility of spur cell anemia blood american society. Changes in the lipid composition of the red cell membranes are found in endstage liver disease. The syndrome in the case presented below has been designated spurcell anemia because of the curious projections from the surfaces of the red blood cells. Rbcs with spike like projections spur cells, target cells and rbc fragments are prominent. In spur cell anemia, the hemoglobin level usually falls to less than 10 gdl and, occasionally, to levels as low as 5 gdl. Spur cell anemia definition of spur cell anemia by the. Sep 18, 2019 olivieri o, guarini p, negri m, et al. Increased proteolytic activity of erythrocyte membrane in spur cell anaemia. Sickle cell anaemia is a homozygous form of hbshbss.
Role of lipoproteins in the formation of spur cell anaemia. The name of the disease comes from the spikelike projections on the erythrocyte membrane. Another crucial point is represented by the reversibility of. Severe mixedtype iron overload in alcoholic cirrhosis. Spur cell anemia article about spur cell anemia by the. The aim of the present study was to evaluate the incidence of spur cells and spur cell anemia in patients with advanced liver disease and to correlate the presence of spur cell anemia with survival. Acanthocytogenesisor how the red cell won its spurs. Spur cell anaemia and acute haemolysis in patients with. Spur cell anemia sca is a cause of hemolytic anemia in patients with alcoholic liver cirrhosis.
Soni and others published spur cell anemia in wilsons disease. Spur cell anemia is found in endstage alcoholic liver disease. Resultsnone of the eight patients with grade 3 or 4 hepatic iron had evidence of the c282y mutation. 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. Read spur cell anemia explains the iron overload in advanced cirrhosis c282y negative, hepatology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. In this chapter we will discuss about red cell indices and also about classification of. Membrane lipid composition of red blood cells in liver. It affects the red blood cells, causing episodes of sickling, which produce episodes of pain and other symptoms. Alterations of the lipid composition and fluidity of erythrocyte rbc ghosts may be present and participate in the phenomenon. Spur cell anemia in the setting of progressive liver. Spur cell anemia is a rare cause of anemia that has traditionally been described as a consequence of alcoholic liver cirrhosis 1 but has recently been described in patients with advanced liver disease from other causes 2. The prognosis of patients with spur cell anemia is poor.
Spur cells and spur cell anemia in hospitalized patients. Queirozandrade m, blasbalg r, ortega cd, rodstein ma, baroni rh, rocha ms, cerri gg. The syndrome in the case presented below has been designated spur cell anemia because of the curious projections from the surfaces of the red blood cells. Oct 20, 2011 a diagnosis of spur cell anemia was made. H emolysis is the destruction or removal of red blood cells from the circulation before their normal life span of 120 days. Jul 12, 2011 with endstage liver disease and the finding of many spur cells on peripheral smear, the etiology of a nonimmune hemolytic anemia is likely spur cell anemia. 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. The erythrocytes have numerous spicules irregularly distributed over the cell surface. Successful combination therapyflunarizine, pentoxifylline. Several lines of evidence pointed to the possibility that the.
Liver transplant introduction spur cell anemia sca is a form of hemolytic anemia historically associated with alcoholic liver cirrhosis. Pernicious anaemia pa is a disease of the stomach that is. Therefore, the presence of spur cell rate higher or equal to 5% is a strong independent predictor of mortality and merits further investigation as a prognostic indicator in patients awaiting liver transplantation. Plasmapheresis for spur cell anemia in a patient with. The irregular folds of the spur cells regressed when polyunsaturated lecithin was infused 2 g daily for five days in eight patients with spur cell anaemia. This results in an increase in the the surface areavolume ratio of the red cell, which produces the target cell. 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.
Because dyslipidemia is related to the development of spur cells, sca was previously treated with plasmapheresis. A peripheral blood smear showed numerous spur cells. A 59yearold male patient with alcoholic cirrhosis developed severe spurcell haemolytic anaemia before death. Spur cell anaemia and hepatic iron stores in patients with alcoholic liver disease undergoing orthotopic liver transplantation. 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. Anemia with spur cells journal of clinical investigation. With endstage liver disease and the finding of many spur cells on peripheral smear, the etiology of a nonimmune hemolytic anemia is likely spur cell anemia.
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. Spur cell anemia article about spur cell anemia by the free. Spur cell haemolytic anaemia sca is a form of anaemia that can be seen in patients with severely impaired liver function or advanced cirrhosis. 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 anaemia was present in six of the eight patients with heavy iron deposition and only one of the remaining patients.
Spurcell hemolytic anemia in severe alcoholic cirrhosis. Spur cell anemia has been described to occur in several conditions, including cirrhosis. Liver alcoholic cirrhosis and spurcell acanthocytic. Spur cell anemia definition of spur cell anemia by medical. Our patient is a middleaged man who developed sca and was not a candidate for liver transplantation or splenectomy. Anemias are classified etiologically and morphologically. Spur cell anemia definition of spur cell anemia by the free. Spur cell anemia sca is a form of acquired hemolytic anemia seen in patients with advanced cirrhosis and particularly in patients with alcoholic cirrhosis. Because dyslipidemia is related to the development. Highdose steroids as a therapeutic option in the management. True iron accumulation may occur in spur cell haemolytic anaemia due to increased iron absorption and the repeated blood transfusions. 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 anemia is a rare type of hemolytic anemia with a grave prognosis. Arienti g, carlini e, scionti l, puxeddu e, brunetti p.
The symptoms of spur cell anemia are related to the anemia and to the underlying disease. We compared his rbc ghosts with those of 10 cirrhotic patients. Sickle cell disease and sickle cell anaemia sickle cell disease scd is a serious, inherited condition affecting the blood and various organs in the body. Spur cell anemia in the setting of progressive liver allograft failure. This could be a statistical discrepancy probably due to limited statistical power because a relatively small number of patients were followed up for at least 1 year or could be attributed to the fact that patients with spur cells have lower 3. A study of erythrocyte ghost composition and fluidity. Anemia due to hemolysis or bleeding is characterized by the presence of a reticulocytosis.
The normal reticulocyte count in a patient with a normal hb and hct is about 1%. Spur cell anemia is an acquired hemolytic anemia, characterized by an increased percentage of abnormally shaped erythrocytes that are known as acanthocytes. Liver alcoholic cirrhosis and spur cell acanthocytic anaemia. Aplastic anaemia can be congenital fanconis anaemia or acquired due to drugs benzene compounds, insecticides, gold or penicillamine. Spurcell anemia definition of spurcell anemia by the. These are due to a disorder in the cholesterol metabolism causing. The sera and red cells of three patients with severe liver disease and spur. The treatment options for sca secondary to cirrhosis are limited. Spur cell haemolytic anaemia was diagnosed when the haemoglobin was 105 gl in the presence of notable acanthocytosis. Spur cell anemia is a serious disorder in cirrhotic patients and is associated with poor prognosis. None of the remaining 28 patients tested showed homozygous hfe mutations. 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. Rapid reversion of spur cell anemia after liver transplantation. Spur cell haemolytic anaemia provides an alternative potential mechanism for the heavy iron loading.
Spur cell anemia definition of spur cell anemia by. Spur cell anaemia and hepatic iron stores in patients with. Sicklecell anemia is caused by a point mutation at the. Plasmapheresis for spur cell anemia in a patient with alcoholic. The clustering of several cases of nonalcoholrelated spur cell anaemia during a short time, in such a small population, is most unusual, and doubtless reflects the unexpectedly high prevalence of hbv infection 82. Causes and types causes of broader categories of spurcell anemia. The occurrence of spurcell anaemia in the course of cirrhosis is rare. Another crucial point is represented by the reversibility of spur cell anemia after liver transplantation.
Hemolytic spur cellanemia hsca is due to lipid disturbances of the erythrocyte membrane and may develop in. Spurcell anemia hemolytic anemia with red cells resembling. Acanthocyte from the greek word 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. Membranecytoskeletal defectshs, elliptocytosis, ovalocytosis,stomatocytosis aquired red cell membrane abnormalitypnh. Spur cell anemia as a cause of unconjugated hyperbilirubinemia after liver transplantation and its resolution after retransplantation received october 25, 2010. 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. Spur cells and spur cell anemia in hospitalized patients with. Note the acanthocytes and target cells, as well as the deeply invaginated spherocytic spur cells. The lipid abnormalities lead to the increased partitioning of free cholesterol into the red cell membrane. Rods placed in both arms and legs show video treatment.
Spurcell anemia definition of spurcell anemia by medical. Liver alcoholic cirrhosis and spurcell acanthocytic anaemia. Spur cell anemia is a type of hemolytic anemia that. Spur cell anemia acanthocytosis complicating idiopathic. Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal sickle or crescent shape. Liver transplantation constitutes the only therapeutic tool. A 59yearold male patient with alcoholic cirrhosis developed severe spur cell haemolytic anaemia before death. Often they may be confused with echinocytes or schistocytes acanthocytes have coarse, weirdly spaced, variably sized crenations, resembling manypointed stars. Spurcell anaemia in a patient with cirrhosis journal of hepatology. It closely resembles zieve syndrome, which can present similarly and needs to be. A 65yearold male developed a rapidly progressive disease characterized by severe hemolysis, with spur cells acanthocytes and liver disease. Peripheral blood smear demonstrated marked anisopoikilocytosis with acanthocytes spur cells. Dec 01, 2000 the clustering of several cases of nonalcoholrelated spur cell anaemia during a short time, in such a small population, is most unusual, and doubtless reflects the unexpectedly high prevalence of hbv infection 82.