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Hemolytic Anemia Associated with Red Cell Membrane Defects
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Some patients with hemolytic HE have a poikilocytic anemia early in life that clinically resembles HPP. These patients appear to “outgrow” their poikilocytosis and manifest typical HE as adults. Clinically, HPP patients resemble severe, recessive HE patients. The predominance of spherocytes among the poikilocytes and the absence of numerous small red cell fragments favors HS.
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Published in Anton Sebastian, A Dictionary of the History of Medicine, 2018
Poikilocytosis [Greek: poikilos, spotted + kytos, hollow + 0515, condition] Term coined in 1877 by Heinrich Irenaeus Quincke (1842–1922) to denote changes in the shape and size of red blood cells in some cases of anemia.
Myelodysplastic Syndromes
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Blood, apart from anemia, which is the most common finding in MDS, may show neutropenia and/or thrombocytopenia. Red blood cells are most often macrocytic, but may be normocytic (microcytic anemia is unusual for MDS). The MDS category RARS is usually associated with mean corpuscular volume (MCV) values between 100 and 105. The red cells often display anisopoikilocytosis and may show basophilic stippling. Poikilocytosis includes dacrocytes (teardropshaped cells), acanthocytes (spur-like cells), and oval macrocytes. Granulocytes are often hypogranular and hypolobated. Hypolobated granulocytes are reminiscent of inborn Pelger–Huët anomaly and are therefore called pseudo-Pelger cells. Platelets may also display atypia (including giant forms) when examined on oil magnification. Blasts, nucleated red blood cells, basophils, and micromegakaryocytes may be present on blood smear. MDS with basophilia was reported to be associated with worse outcome. Presence of 2%–4% blasts in the blood is diagnostic for RAEB-1 (even if the number of blasts in the BM is <5%) and 5%–19% for RAEB-2. Based on the WHO classification, the presence of Auer rods is diagnostic for RAEB-2, even if the number of blasts in the blood is <5%.
Dominantly Inherited β-Thalassemia Caused by a Single Nucleotide Deletion in Exon 3 of the β-Globin Gene: Hb Xiangyang (HBB: c.393delT)
Published in Hemoglobin, 2022
Xiao-Mei Lin, Fan Jiang, Jian Li, Dong-Zhi Li
The proband was the only son of a non consanguineous couple. The boy was first noted to be anemic at 2 years of age. His red blood cell (RBC) parameters showed microcytic hypochromic features with a Hb level of 8.0–9.0 g/dL. Two transfusions were given during his growth, both for anemia aggravation caused by infection. The patient was treated once for iron deficiency anemia, but failed to improve the anemia. At the age of 6 years, the child was referred to our center for elucidation of the cause of anemia. Physical examination showed a mild splenomegaly. Peripheral blood smears showed anisocytosis and poikilocytosis, hypochromia, basophilic stippling and reticulocytosis. Heinz bodies were observed in RBC upon staining with brilliant cresyl blue. The blood counts showed a mild anemia with microcytosis and hypochromia (Table 1). Hemoglobin (Hb) electrophoresis showed borderline Hb A2 and elevated Hb F values (Figure 1). No abnormal Hb was detected by capillary electrophoresis and high performance liquid chromatography. The heat and isopropanol stability tests showed no abnormal Hb. General physical examination was normal in both parents, with normal hematological features (Table 1).
A novel EPB41 p.Trp704* mutation in a Korean patient with hereditary elliptocytosis: a case report
Published in Hematology, 2020
Soyoung Shin, Kyung-Ah Hwang, Kyuhyun Paik, Joonhong Park
Hereditary elliptocytosis (HE) is a hematologic disorder characterized by elliptically-shaped erythrocytes and a variable degree of hemolytic anemia. The clinical phenotype is usually mild with peripheral blood elliptocytes, but it can be moderately severe. In severe forms that achieve hereditary poikilocytosis, large red cell fragments are torn off, leaving erythrocytes with marked poikilocytosis. Usually inherited as an autosomal dominant trait, elliptocytosis results from mutation in any one of several genes encoding proteins of the red cell membrane skeleton [1]. HE-1 is caused by heterozygous or homozygous mutation in the gene encoding erythrocyte membrane protein 4.1 (EPB41) on chromosome 1p35 [2]. HE-2 is caused by mutation in the SPTA1 gene [3]. HE-3 is caused by mutation in the SPTB gene [4]. HE-4, also known as Southeast Asian ovalocytosis, is caused by mutation in the SLC4A1 gene [5]. Particularly, the complex EPB41 gene encodes a diverse family of protein 4.1R isoforms which are key components of the erythroid membrane skeleton that regulates red cell morphology and mechanical stability [6].
Protective effects of chrysin on sub-acute diazinon-induced biochemical, hematological, histopathological alterations, and genotoxicity indices in male BALB/c mice
Published in Drug and Chemical Toxicology, 2018
Majid Zeinali, Naser Tayebi Meybodi, Seyed Abdolrahim Rezaee, Houshang Rafatpanah, Hossein Hosseinzadeh
Moreover, the hematological findings of animal studies revealed that RBCs count, Hgb concentration and HCT level in the DZN treated group were decreased which could be reflected in impaired production in the rate of erythropoiesis, hemosynthesis, and/or an increase in the rate of RBC destruction in hemopoietic organs (Hariri et al.2010). Also, poikilocytosis and anisocytosis with hypochromic RBC in peripheral blood smears were seen in the DZN group. Alterations in RBCs count and Hgb concentration could be attributed to interference of free radicals from OP with Hgb biosynthesis and shortening of the life span of circulating erythrocyte. The decrease in HCT is attributable to the reduction in RBC count caused either destruction or reduction in size (Rahman and Siddiqui 2006).