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N Engl J Med 2018 Aug;379(8):774

Oregon Health and Science University, Portland, OR

Case Rep Hematol 2018 21;2018:9513946. Epub 2018 Jun 21.

Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji 611-0042, Japan.

Background: Spur cell anemia (SCA) is a cause of hemolytic anemia in patients with alcoholic liver cirrhosis. Because dyslipidemia is related to the development of spur cells, SCA was previously treated with plasmapheresis.

Case Report: A 52-year-old Japanese man with SCA associated with alcoholic liver cirrhosis (Child-Pugh C) underwent two rounds of plasmapheresis. Read More

BMJ Case Rep 2018 Jan 31;2018. Epub 2018 Jan 31.

Medical Oncology/Medicine, Captain James A. Lovell Federal Health Care Center, North Chicago, Illinois, USA.

Spur cell haemolytic anaemia (SCA) is a form of anaemia that can be seen in patients with severely impaired liver function or advanced cirrhosis. It is associated with high mortality. The treatment options for SCA secondary to cirrhosis are limited. Read More

Dig Dis Sci 2018 Jan 25;63(1):16-26. Epub 2017 Nov 25.

Department of Clinical and Experimental Medicine, Garibaldi Hospital, University of Catania, Via Palermo, 636, 95122, Catania, Italy.

Progressive lipoprotein impairment occurs in liver cirrhosis and is associated with increased morbidity and mortality. The present review aims to summarize the current evidence regarding the prognostic value of lipoprotein abnormalities in liver cirrhosis and to address the need of a better prognostic stratification of patients, including lipoprotein profile assessment. Low levels of lipoproteins are usual in cirrhosis. Read More

Blood 2017 12 6;130(25):2786-2798. Epub 2017 Nov 6.

Institute for Pathophysiology, West German Heart and Vascular Center and.

The hepatocyte nuclear factor (HNF) family regulates complex networks of metabolism and organ development. Human mutations in its prototypical member HNF1A cause maturity-onset diabetes of the young (MODY) type 3. In this study, we identified an important role for HNF1A in the preservation of erythrocyte membrane integrity, calcium homeostasis, and osmotic resistance through an as-yet unrecognized link of HNF1A to sphingolipid homeostasis. Read More

Clin Chim Acta 2017 May 20;468:81-84. Epub 2017 Feb 20.

Department of Hematology, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India. Electronic address:

Introduction: Inherited anemias diagnostic workup requires a step-wise algorithm. Causal genes implicated in congenital hemolytic anemia are numerous, making a gene-by-gene approach by Sanger sequencing time consuming, expensive and labour intensive. Targeted resequencing can be of great use in explaining these cases. Read More

Med Clin North Am 2017 Mar 10;101(2):417-429. Epub 2016 Dec 10.

Division of Hematology/Medical Oncology, Department of Medicine, Oregon Health Sciences University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97201-3098, USA; Division of Hematology/Medical Oncology, Department of Medicine, Knight Cancer Institute, Oregon Health and Science University, MC L586, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA. Electronic address:

Many processes lead to anemia. This review covers anemias that are less commonly encountered in the United States. These anemias include hemoglobin defects like thalassemia, bone marrow failure syndromes like aplastic anemia and pure red cell aplasia, and hemolytic processes such as paroxysmal nocturnal hemoglobinuria. Read More

Int J Lab Hematol 2017 Jun 22;39(3):e64-e65. Epub 2016 Dec 22.

Department of Pediatrics, UCMS and GTB Hospital, New Delhi, India.

Gastroenterol Hepatol Bed Bench 2016 ;9(4):335-339

Department of Clinical and Experimental Medicine, Garibaldi Hospital, University of Catania, Italy.

Chronic anemia is common in liver cirrhosis. In this setting, the pathogenesis of anemia is complex and multifactorial. Spur cell anemia is a serious disorder in cirrhotic patients and is associated with poor prognosis. Read More

Transfusion 2016 Apr;56(4):787-8

University of Washington, Harborview Medical Center, Seattle, Washington.

Am J Hematol 2016 Oct 22;91(10):1061. Epub 2016 Mar 22.

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

Rev Clin Esp 2016 May 5;216(4):233. Epub 2015 Dec 5.

Servicio de Hematología, Hospital Universitario de Móstoles, Móstoles, Madrid, España.

Nature 2015 Dec 9;528(7582):422-6. Epub 2015 Dec 9.

The Lunenfeld-Tanenbaum Research Institute, Mount Sinai Hospital, 600 University Avenue, Toronto, Ontario M5G 1X5, Canada.

DNA repair by homologous recombination is highly suppressed in G1 cells to ensure that mitotic recombination occurs solely between sister chromatids. Although many homologous recombination factors are cell-cycle regulated, the identity of the events that are both necessary and sufficient to suppress recombination in G1 cells is unknown. Here we report that the cell cycle controls the interaction of BRCA1 with PALB2-BRCA2 to constrain BRCA2 function to the S/G2 phases in human cells. Read More

J Med Case Rep 2014 Nov 27;8:390. Epub 2014 Nov 27.

Department of Clinical Medicine, Faculty of Medicine, University of Colombo, 25, Kynsey Road, Colombo 08, Sri Lanka.

Introduction: Klippel-Trenaunay syndrome is a rare congenital mesodermal abnormality characterized by bone and soft tissue hypertrophy, extensive hemangioma and venous abnormalities. We report the case of a patient with two additional rare clinical manifestations in the background of Klippel-Trenaunay syndrome, namely, acanthocytosis and splenic and retroperitoneal lymphangioma.

Case Presentation: A 24-year-old Sri Lankan man from North Central Province in Sri Lanka presented to our general medical unit with symptomatic anaemia. Read More

Clin Med Insights Pathol 2014 15;7:11-4. Epub 2014 Apr 15.

Institute of Environmental Health, China Medical University College of Public Health, Taichung, Taiwan.

Introduction: Unlike autoimmune hemolytic anemia (AIHA), literature on the etiological study of non-autoimmune hemolytic anemia (non-AIHA) is scarce. The incidence and prevalence of non-AIHA in different geographic regions are largely unknown perhaps owing to the lack of perspective investigation and different profiles of etiologies from different geographic regions. We aimed to examine the real-world etiology or mechanisms of the non-hereditary non-AIHA from a nationwide population-based administrative claim database in Taiwan. Read More

Zhonghua Yi Xue Za Zhi 2014 Mar;94(8):603-5

Department of Hematology, Affiliated Nanjing First Hospital, Nanjing Medical University, Nanjing 210006, China. Email:

Objective: To explore the clinical characteristic of adult hereditary spherocytosis (HS) and the efficacies of splenectomy.

Methods: Six cases with adult HS were collected from July 2006 to July 2012. And their clinical features, laboratory profile and treatment were retrospectively analyzed. Read More

Ann Hepatol 2014 May-Jun;13(3):396-8

Department of Medicine II, Saarland University Medical Center, Homburg, Germany.

Ann Indian Acad Neurol 2014 Jan;17(1):113-6

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.

Abetalipoproteinemia is an uncommon cause of ataxia and retinitis pigmentosa (RP). Most of the neurological and ocular manifestations occur secondary to deficiency syndromes that is consequent to fat malabsorption from the small intestine. In this report, we have described the phenotype of a young adult female who manifested with recurrent diarrheal illness in her first decade, followed by anemia, RP, and neurological involvement with progressive deafness, cerebellar and sensory ataxia, and subclinical neuropathy in her second decade of life. Read More

Am J Emerg Med 2014 Aug 25;32(8):944.e3-4. Epub 2014 Jan 25.

SUNY Upstate Medical University, Syracuse, NY 13210. Electronic address:

Intern Med 2013 ;52(24):2831

Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Japan.

J Gastroenterol Hepatol 2014 Apr;29(4):830-4

2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.

Background And Aim: The presence of spur-cell anemia (SCA) is due to lipid disturbances of the erythrocyte membrane and may develop in patients with advanced liver cirrhosis. The accurate predicting value of SC for survival has not been clarified. The aim of this study was to evaluate SCA as a prognostic indicator in patients with cirrhosis. Read More

Vet Clin Pathol 2013 Dec;42(4):465-70

Department of Clinical Sciences, Animal Cancer Center, Colorado State University, Ft Collins, CO, USA.

Background: An acanthocyte is an abnormally shaped erythrocyte. In veterinary medicine, acanthocytes have historically been associated with canine hemangiosarcoma. In human medicine, acanthocytes are rarely observed with neoplastic disease and are more commonly associated with a variety of hereditary and acquired diseases. Read More

Semin Hematol 2013 Jul;50(3):216-21

Center for Biologics Evaluation and Research, United States Food and Drug Administration, Rockville, MD 20852, USA.

The liver plays a key role in both protein biosynthesis and lipid metabolism. As a result, hepatic synthetic dysfunction can have adverse effects on both cellular and soluble components of blood. Anemia may occur due to the hemolysis of acanthocytes (spur cells), which is ultimately due to abnormal lipid composition of the red blood cell membrane. Read More

Blood 2013 Jul 21;122(2):262-71. Epub 2013 May 21.

Cell Signalling Group, Laboratory for Cancer Medicine, Western Australian Institute for Medical Research and Centre for Medical Research, University of Western Australia, Perth, WA, Australia.

Lyn is involved in erythropoietin (Epo)-receptor signaling and erythroid homeostasis. Downstream pathways influenced following Lyn activation and their significance to erythropoiesis remain unclear. To address this, we assessed a gain-of-function Lyn mutation (Lyn(up/up)) on erythropoiesis and Epo receptor signaling. Read More

Transfusion 2013 Feb;53(2):260

Service d'Hématologie Biologique, Centre Hospitalier Universitaire, Nancy, France.

Bone 2013 Apr 19;53(2):541-5. Epub 2013 Jan 19.

Department of Internal Medicine I and Clinical Chemistry, University of Heidelberg General Hospital, Im Neuenheimer Feld 671, 69120 Heidelberg, Germany.

A 47-year-old woman consulted her dermatologist complaining whole body dermatitis, urticaria and irritating bullous eruptions on the plantar and side surfaces of her feet. She had had multiple hypopigmented spots on her skin since her early adulthood. The patient was treated with topical medication without significant improvement of symptoms. Read More

Biochim Biophys Acta 2012 Dec 31;1821(12):1493-500. Epub 2012 Aug 31.

Laboratory of Red Blood Cell Diagnostics, Dept. of Blood Cell Research, Sanquin Blood Supply Foundation, Amsterdam, The Netherlands.

Maintenance of the asymmetric distribution of phospholipids across the plasma membrane is a prerequisite for the survival of erythrocytes. Various stimuli have been shown to induce scrambling of phospholipids and thereby exposure of phosphatidylserine (PS). In two types of patients, both with aberrant plasma cholesterol levels, we observed an aberrant PS exposure in erythrocytes upon stimulation. Read More

Blood 2011 Oct;118(16):4304

University Hospital Zurich.

Eurasian J Med 2011 Dec;43(3):173-6

Department of Internal Medicine, Kayseri Education and Research Hospital, Kayseri, Turkey.

Objective: Therapy-related myeloid neoplasms (t-MN) account for approximately 10% to 20% of all cases of AML (acute myeloid leukemia), MDS (myelodysplastic syndrome) and MDS/MPN (myelodysplastic syndrome/myeloproliferative neoplasms), MDS, and MDS/MPN. In our study, we evaluated peripheral blood smear samples and hemogram values in breast cancer patients who were receiving adjuvant anthracycline regimens and were in remission.

Materials And Methods: A total of 78 patients receiving anthracycline-based adjuvant chemotherapy treatment from Kayseri Research and Training Hospital and Mersin State Hospital were enrolled in the study. Read More

Liver Transpl 2011 Mar;17(3):349-50

Toxicol Pathol 2010 Oct 30;38(6):907-22. Epub 2010 Aug 30.

Merck Research Laboratories, Lafayette, NJ 07848, USA.

CXCR3 is a chemokine receptor, upregulated upon activation of T cells and expressed on nearly 100% of T cells in sites of inflammation. SCH 900875 is a selective CXCR3 receptor antagonist. Thrombocytopenia and severe hemolytic anemia with acanthocytosis occurred in rats at doses of 75, 100, and 150 mg/kg/day. Read More

Hepatol Res 2010 Feb 11;40(2):161-70. Epub 2010 Jan 11.

Gastroenterology and Hepatology Division, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece.

Aim: Spur cell anemia (SCA) is a form of acquired hemolytic anemia seen in patients with advanced cirrhosis and particularly in patients with alcoholic cirrhosis. 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.

Methods: During a 33-month period, all patients with advanced cirrhosis (Child-Pugh-Turcott score [CPT]>/=7] who were hospitalized in our department for various reasons were included in this study. Read More

World J Gastroenterol 2009 Dec;15(46):5864-6

Department of Gastroenterology, University Hospital Birmingham, Vincent Drive, Birmingham B15 2TH, UK.

In this case report we describe the relationship between ferritin levels and hepcidin in a patient with alcohol-related spur cell anemia who underwent liver transplantation. We demonstrate a reciprocal relationship between serum or urinary hepcidin and serum ferritin, which indicates that inadequate hepcidin production by the diseased liver is associated with elevated serum ferritin. The ferritin level falls with increasing hepcidin production after transplantation. Read More

Haematologica 2009 Dec 16;94(12):1753-7. Epub 2009 Jul 16.

Department of Pediatrics, Second University of Naples, Naples, Italy.

We describe a beta-spectrin variant, named beta-spectrin Bari, characterized by a truncated chain and associated with hereditary spherocytosis. The clinical phenotype consists of a moderately severe hemolytic anemia, splenomegaly, and spherocytes and acanthocytes in the blood smear. The occurrence of the truncated protein, that represents about 8% of the total beta-spectrin occurring on the membrane, results in a marked spectrin deficiency. Read More

J Gastroenterol Hepatol 2008 Sep;23(9):1463

Department of Gastrointestinal Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Hepatol Res 2007 Jun;37(6):482-5

Department of Medicine and Gastroenterology, Tokyo Women’s Medical University, Tokyo, Japan.

Spur cell anemia is an acquired hemolytic anemia, which may occur in patients with severe liver dysfunction, especially in those with alcoholic cirrhosis. Recently, non-alcoholic steatohepatitis (NASH) has been highlighted as one of the causes of chronic liver diseases, which could progress to cirrhosis. Histological features of NASH are indistinguishable from those of alcoholic hepatitis. Read More

Ann Emerg Med 2007 Sep 3;50(3):289-91. Epub 2006 Nov 3.

Department of Clinical and Experimental Medicine, Section of Internal Medicine, University of Verona, Verona, Italy.

We report a case of acute hemolytic anemia in a 21-year-old Nigerian woman after high-dose misoprostol (4 mg), used for medical abortion. The major causes of inherited or immune hemolytic anemia were excluded. The patient's peripheral blood smear showed acanthocytes and anisopoikilocytosis, which progressively disappeared in the days postingestion. Read More

Dig Dis Sci 2006 Jul;51(7):1203-5

Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of Virginia Health System, Charlottesville, Virginia 22908-0708, USA.

Gene 2006 Mar 20;370:6-16. Epub 2006 Jan 20.

The Lindsley F. Kimball Research Institute of the New York Blood Center, 310 East 67th Street, New York, NY 10021, USA.

XK, a putative membrane transporter, is a component of the XK/Kell complex of the Kell blood group system. XK's substrate is unknown but absence of the protein, as occurs in the McLeod phenotype, is associated with red cell acanthocytosis and late onset central nervous system and neuromuscular abnormalities known as the McLeod syndrome. We have cloned two cDNAs, XPLAC (GenBank accession no. Read More

J Hepatol 2004 Jul;41(1):167

Liver Unit, Institut de Malalties Digestives, Hospital Clínic, Villarroel 170, 08036 Barcelona, Spain.

Med Hypotheses 2004 ;62(6):966-9

Department of Oncology, McGill University, 546 Pine Avenue West, Montreal, Que., Canada H2W 1S6.

Acanthocytosis refers to the transformation of the normal biconcave disc erythrocyte into one with a few irregularly shaped external projections distributed unevenly at its membrane surface. It is associated with a variety of inherited and acquired disorders. A relationship between the acanthocytosis in chorea-acanthocytosis and an alteration of Band 3, the anion exchange protein, has been previously suggested. Read More

Zhonghua Er Ke Za Zhi 2003 May;41(5):379

Int Surg 2002 Oct-Dec;87(4):201-4

Department of Medicine, Sturgis Hospital, Sturgis, Michigan, USA.

Spur cell anemia is an acquired form of hemolytic anemia caused by a structural abnormality of red cell membranes that results in spiculated erythrocytes. These peculiarly shaped red blood cells, called acanthocytes, have a shortened survival and undergo splenic sequestration and destruction. Spur cell anemia has been known to occur in several conditions, including chronic liver disease, and more specifically in alcoholic cirrhosis. Read More

J Egypt Soc Parasitol 1999 ;29(1):203-13

Department of Surgery, Faculty of Medicine, Ain-Shams University, Cairo 11566, Egypt.

This study was carried out on forty cases, classified into 3 groups; group I: 10 healthy controls subjects, group II: 20 patients with hepatosplenic schistosomiasis and group III: 10 bilharzial patients who underwent total splenectomy. All cases were subjected to clinical examination, abdominal ultrasonography, rectal snips and laboratory investigations which included: stool and urine analysis, complete blood picture, IHAT for bilharziasis, liver function tests, viral markers, estimaton of T-lymphocyte subpopulations (CD(+)3, CD(+)4, & CD(+)8) by flow Cytometry, silver stained blood films to detect argyrophilic inclusions and 99mTc sulphur colloid splenic scan which was applied to group II only. The present results revealed varying degrees of hypersplenism (anaemia, leukopenia & thrombocytopenia) in GII. Read More

Am J Med Sci 2002 Nov;324(5):281-4

Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.

We report the case of a 17-year-old woman with anorexia nervosa (AN) who developed an abetalipoproteinemia-like lipid profile and acanthocytosis. These abnormalities resolved slowly as her nutritional status improved. We considered 3 possible causes of an abetalipoproteinemia-like lipid profile in AN: (1) depletion of hepatic substrate for apolipoprotein B synthesis, (2) lack of exogenous fatty acids with exhaustion of endogenous stores of triglycerides in adipose tissue, and (3) preservation of the lipoprotein lipase (LPL) mass. Read More

Braz J Med Biol Res 2002 Aug;35(8):921-5

Hemocentro, Universidade Estadual de Campinas, Campinas, SP, Brasil.

Hereditary spherocytosis (HS) is a common inherited anemia characterized by the presence of spherocytic red cells. Defects in several membrane protein genes have been involved in the pathogenesis of HS. beta-Spectrin-related HS seems to be common. Read More

Biochim Biophys Acta 2002 Feb;1558(2):142-50

Laboratorio de Análisis Biológicos, Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Pabellón II, Piso 4. Ciudad Universitaria, 1428 Buenos Aires, Argentina.

There is evidence that anaemia is associated with aluminium (Al). We have already reported on the sensitivity to Al, showed by erythroid cell populations of animals chronically exposed to the metal. In order to investigate whether Al could also affect human cells, experiments were carried out both on immature and mature human erythroid cells. Read More

Turk J Pediatr 2001 Jul-Sep;43(3):243-5

Department of Pediatrics, Atatürk University Faculty of Medicine, Erzurum, Turkey.

Abetalipoproteinemia is a rare autosomal recessive disorder characterized by steatorrhea, poor weight gain, acanthocytosis and retinitis pigmentosa. Here we peresent a six-month-old patient with abetaliporoteinemia. He had a history of chronic diarrhea from the first month of life. Read More

Int J Hematol 2001 Apr;73(3):351-5

First Department of Internal Medicine, University of Tokushima School of Medicine, Japan.

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. 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 combination therapy consisting of flunarizine, pentoxifylline, and cholestyramine. Initial therapy with flunarizine alone for 6 weeks did not significantly decrease the number of spur cells on peripheral blood smears. Read More

Acta Trop 2000 Dec;77(3):257-62

Infectious Diseases Section, Tropical Medicine Institute, Universidad Central de Venezuela, 'Dr Felix Pifano C', Apartado 47019, 1041-A, Caracas, Venezuela.

A prospective study, aimed to investigate the aetiology of an unusual clustering of cases of severe acute haemolytic anaemia affecting a high percentage of the adult population, was carried out in two isolated Yanomamo communities of the Upper Orinoco basin in Venezuela. Twenty-six patients with active or recent episodes of severe haemolysis were evaluated. All of them exhibited massive liver and spleen enlargement and fulfilled the diagnostic criteria of the hyperreactive malarious splenomegaly (HMS) syndrome. Read More