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. Request pdf spur cells and spur cell anemia in hospitalized patients with advanced liver disease. 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 a rare type of hemolytic anemia with a grave prognosis. Spur cell anemia is an acquired hemolytic anemia, characterized by an increased percentage of abnormally shaped erythrocytes that are known as acanthocytes. We report a case of spur cell anemia with alcoholic liver cirrhosis successfully cured by living liver transplantation ldlt. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Spur cell anemia is an acquired hemolytic process characterized by multiple acanthocytes in the peripheral blood smear. Severe mixedtype iron overload in alcoholic cirrhosis. It is related to abnormal cholesterol loading of the red blood cell membrane. 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 serious disorder in cirrhotic patients and is associated with poor prognosis.
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. Our patient is a middleaged man who developed sca and was not a candidate for liver transplantation or splenectomy. Spur cell anemia definition of spur cell anemia by medical. Incidence and correlation with disease severity and survival aim. Feder jn, gnirke a, thomas w, tsuchihashi z, ruddy da. Sep 18, 2019 abstinence from alcohol is also the best preventive measure for spur cell anemia. Spur cell anemia is a type of hemolytic anemia that can develop in.
Red blood cells carry oxygen to the body and are normally shaped like a disc. Sicklecell anemia is caused by a point mutation at the sixth. 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. Spur cell anemia is found in endstage alcoholic liver disease. Spur cell anemia is a type of hemolytic anemia that can develop in patients with advanced liver disease especially alcoholic cirrhosis. Letter to the editors spur cell anemia as a cause of. Spurcell anemia definition of spurcell anemia by medical. Resolution of spur cell anemia with liver transplantation. Finally, our case report shows for the first time a very rapid reversion of sca after lt with prompt. It closely resembles zieve syndrome, which can present similarly and needs to be. Feder jn, gnirke a, thomas w, tsuchihashi z, ruddy da, basava a, dormishian f, domingo r, jr, ellis mc, fullan a, et al. Peripheral blood smear demonstrated marked anisopoikilocytosis with acanthocytes spur cells.
Spur cell anemia is an acquired hemolytic anemia, which may occur in patients with severe liver dysfunction, especially in those with alcoholic cirrhosis. 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. A 65yearold male developed a rapidly progressive disease characterized by severe hemolysis, with spur cells acanthocytes and liver disease. 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. Images in clinical medicine from the new england journal of medicine spurcell anemia.
Spur cell anaemia and hepatic iron stores in patients with. Rapid reversion of spur cell anemia after liver transplantation. It is considered by some to be a variant of spur cell anemia. Spur cell anemia acanthocytosis complicating idiopathic. 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. The patient was a 31yearold woman with a history of ethanol abuse.
Spur cells and spur cell anemia in hospitalized patients. Spur cell anemia in the setting of progressive liver allograft failure. Anemia with spur cells journal of clinical investigation. Abnormal phospholipid metabolism in spur cell anemia. Liver transplantation constitutes the only therapeutic tool. Spur cell anemia sca is a cause of hemolytic anemia in patients with alcoholic liver cirrhosis. The treatment options for sca secondary to cirrhosis are limited.
In spur cell anemia, the hemoglobin level usually falls to less than 10 gdl and, occasionally, to levels as low as 5 gdl. We hypothesized that defective phospholipid repair may contribute to the red blood cell rbc phospholipid abnormalities of spur cell anemia. 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. Anemia in alcoholic liver disease has a broad spectrum of differential diagnosis. 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. Complete resolution of spur cell anemia has been reported after liver transplantation.
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. One of the esoteric pathology that should be considered is spur cell anemia in end stage liver disease patients. Review the causal information about the various more general categories of medical conditions. Spur cell anemia in the setting of progressive liver. Spur cell hemolytic anemia in liver failure murphy. To view the article, select the view print version pdf link above. Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal sickle or crescent shape. Abstinence from alcohol is also the best preventive measure for spur cell anemia. Spur cell acanthocyte and burr cell echinocyte comparision anemia of renal disease tags.
The patients clinical condition rapidly deteriorated, and she died of progressive liver failure. 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. 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. Rods placed in both arms and legs show video treatment. 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. 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. Both patients spur cell anemia resolved and target cells became the major abnormal erythrocyte form. Spur cell anemia is an insufficiently investigated entity and its clinical importance has not been documented yet. Hemolytic spur cellanemia hsca is due to lipid disturbances of the erythrocyte membrane and may develop in. The erythrocytes have numerous spicules irregularly distributed over the cell surface.
Plasmapheresis for spur cell anemia in a patient with. Because dyslipidemia is related to the development. A liver biopsy revealed cirrhosis with marked pericellular fibrosis and iron overload picture 2. 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. 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. 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. Spurcell anaemia in a patient with cirrhosis journal of hepatology. 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.
Successful combination therapyflunarizine, pentoxifylline. A case of spur cell anemia resolved by living donor liver. Jul 12, 2011 of spur cells and decreased red cell survival, resulting in hemolytic anemia. Surface morphology of platelets in spurcell anemia.
Spur cell anemia associated with alcoholic cirrhosis. Spur cell haemolytic anaemia sca is a form of anaemia that can be seen in patients with severely impaired liver function or advanced cirrhosis. True iron accumulation may occur in spur cell haemolytic anaemia due to increased. Santopaolo 21 rapid reversion of spur cell anemia after liver transplantation med ase rep rev, 2019 doi. Spurcell anemia definition of spurcell anemia by the free. Spur cell anemia has been described to occur in several conditions, including cirrhosis. Oct 20, 2011 a diagnosis of spur cell anemia was made. Saarland university medical center, homburg, germany. Spur cell anemia is a type of hemolytic anemia that. Plasmapheresis for spur cell anemia in a patient with alcoholic. Spur cell anemia is a type of hemolytic anemia that can develop in patients with liver failure.
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. 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. The sera and red cells of three patients with severe liver disease and spur. The world health organization has defined anemia as a hemoglobin concentration below 7. Although the alcohol intake could have contributed to the formation of spur cells, the possible association with hemochromatosis should be considered. The symptoms of spur cell anemia are related to the anemia and to the underlying disease. There are few reports on its prevalence and survival. 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.
Rbcs with spike like projections spur cells, target cells and rbc fragments are prominent. Spur cell anemia as a cause of unconjugated hyperbilirubinemia after liver transplantation and its resolution after retransplantation received october 25, 2010. Spur cell anemia is a type of hemolytic anemia that can develop in patients with advanced liver disease. Spur cell anemia definition of spur cell anemia by the free. University of washington, harborview medical center, seattle, washington. Spur cell anemia acanthocytosis complicating idiopathic he. A 48 yearold man with cryptogenic cirrhosis was admitted to the hospital because of malaise and progressive. 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. 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. Spur cell anemia article about spur cell anemia by the free.