Page 500 - Williams Hematology ( PDFDrive )

474 Part VI: The Erythrocyte Chapter 31: Structure and Composition of the Erythrocyte 475

erythrocytes varies greatly, from 1 to 98 percent. Qualitative and quan- REFERENCES
titative anomalies of spectrin and protein 4.1, the major proteins of the
membrane skeleton, are associated with hereditary elliptocytosis. 49,64 1. Malik P, Fischer TC, Barsky LL, et al: An in vitro model of human red blood cell pro-
duction from hematopoietic progenitor cells. Blood 91:2664, 1998.
Severe hemolytic anemia is seen only in the homozygous or compound 2. Sato T, Maekawa T, Watanabe S, et al: Erythroid progenitors differentiate and mature in
heterozygotes form of the disease (hereditary pyropoikilocytosis) where response to endogenous erythropoietin. J Clin Invest 106:263, 2000.
extensive cell fragmentation produces pyropoikilocytes with marked 3. Giarratana MC, Kobari L, Lapillonne HC et al: Ex vivo generation of fully mature
human red blood cells from hematopoietic stem cells. Nat Biotechnol 23:69, 2005.
decreases in SA:V ratio. 4. Hu J, Liu J, Xue F, et al: Isolation and functional characterization of human erythrob-
lasts at distinct stages: Implications for understanding of normal and disordered ery-
Acanthocytes thropoiesis in vivo. Blood 121:3246, 2013.
The acanthocyte (Chap. 46) is irregularly shaped, with two to 10 hemi- 5. Li J, Hale J, Bhagia P et al: Isolation and transcriptome analysis of human erythroid
progenitors. Blood 124:3636, 2014.
spherically tipped spicules of variable length and diameter. The bases of 6. Southcott MJG, Tanner MJA, Anstee DJ: The expression of human blood group anti-
the spicules on the acanthocyte are of varying girth, unlike the spicules gens during erythropoiesis in a cell culture system. Blood 93:4425, 1999.
on echinocytes, which have remarkably uniform dimensions. Acantho- 7. Palis J: Ontogeny of erythropoiesis. Curr Opin Hematol 15:155, 2008.
cytes are seen in neuroacanthocytosis and in abetalipoproteinemia. 8. Palis J: Primitive and definitive erythropoiesis in mammals. Front Physiol 5:3, 2014.
65
The lack of anemia in these conditions suggests that these cells have 9. Zambidis ET, Peault B, Park TS, et al: Hematopoietic differentiation of human embry-
onic stem cells progresses through sequential hematoendothelial, primitive, and defin-
near normal life span in circulation. itive stages resembling human yolk sac development. Blood 106:860, 2005.
10. Pereda J, Niimi G: Embryonic erythropoiesis in human yolk sac: Two different com-
Target Cells (Codocytes) partments for two different processes. Microsc Res Tech 71:856, 2008.
A relative excess of membrane surface area or decreased cell volume 11. Schmid-Schonbein H, Wells R: Fluid drop-like transition of erythrocytes under shear.
Science 165:288, 1969.
leading to increased SA:V ratio results in target cells. Target cells may 12. Sadahira Y, Mori M: Role of the macrophage in erythropoiesis. Pathol Int 49:841, 1999.
66
be seen in obstructive liver disease, hemoglobinopathies (S and C), 13. Bessis M: Living Blood Cells and Their Ultrastructure. Springer-Verlag, Berlin, 1973.
thalassemia, iron deficiency, postsplenectomy, and lecithin cholesterol 14. Gregory CJ, Eaves AC: Three stages of erythropoietic progenitor cell differentiation dis-
tinguished by a number of physical and biologic properties. Blood 51:527, 1978.
acetyltransferase deficiency. In patients with obstructive liver disease, 15. McLeod DL, Shreeve MM, Axelrad AA: Improved plasma culture system for produc-
lecithin cholesterol acetyltransferase activity is depressed. This increases tion of erythrocytic colonies in vitro: Quantitative assay method for CFU-E. Blood
the cholesterol-to-phospholipid ratio and produces an absolute increase 44:517, 1974.
in the surface area of the red cell membrane. In contrast, membrane 16. Chasis JA, Mohandas N: Erythroblastic islands: Niches for erythropoiesis. Blood
112:470, 2008.
excess is only relative in patients with iron-deficiency anemia and tha- 17. Manwani D, Bieker JJ: The erythroblastic island. Curr Top Dev Biol 82:23, 2008.
lassemia because of the reduced cell volume. In contrast to spherocytes 18. Yokoyama T, Etoh T, Kitagawa H, et al: Migration of erythroblastic islands toward the
which exhibit increased osmotic fragility, target red cells are osmotically sinusoid as erythroid maturation proceeds in rat bone marrow. J Vet Med Sci 65:449,
2003.
resistant. 19. Lichtman MA, Santillo P: Red cell egress from the marrow—Vis-à-tergo. Blood Cells
12:11, 1986.
Sickle Cells (Drepanocytes) 20. Chamberlain JK, Lichtman MA: Marrow cell egress: Specificity of the site of penetra-
tion into the sinus. Blood 52:959, 1978.
The sickle cell (Chap. 49) displays a characteristic variation of form on 21. Waugh RE: Reticulocyte rigidity and passage through endothelial-like pores. Blood
stained blood films. The fusiform cell in the crescent shape with two 78:3037, 1991.
pointed extremities is encountered most commonly in deoxygenated 22. Yoshida H, Kawane K, Koike M et al: Phosphatidylserine-dependent engulfment by
blood samples as a result of polymerization of sickle hemoglobin. If macrophages of nuclei from erythroid precursor cells. Nature 437:754, 2005.
sickle cell formation is observed by phase-contrast microscopy, the 23. Jacobsen RN, Forristal CE, Raggatt LJ, et al: Mobilization with granulocyte colony-
stimulating factor blocks medullar erythropoiesis by depleting F4/80(+)VCAM1(+)
earliest change with deoxygenation is loss of flicker, followed by slight CD169(+)ER-HR3(+)Ly6G(+) erythroid island macrophages in the mouse. Exp Hema-
deformation at the discocyte border with displacement of the hemo- tol 42:547, 2014.
globin to one region of the cell. The cell then elongates and becomes 24. Rhodes MM, Kopsombut P, Bondurant MC, et al: Adherence to macrophages in ery-
throblastic islands enhances erythroblast proliferation and increases erythrocyte pro-
rigid as a result of polymerization of hemoglobin S. Upon reoxygena- duction by a different mechanism than erythropoietin. Blood 111:1700, 2008.
tion, the sickle cell resumes the discocyte form and, in so doing, loses 25. Kawane K, Fukuyama H, Kondoh G, et al: Requirement of DNase II for definitive ery-
membrane by microspherulation and fragmentation during retraction thropoiesis in the mouse fetal liver. Science 292:1546, 2001.
67
of long spicules. Evidence suggests that the more typical sickle-shaped 26. Bowman WD Jr: Abnormal (“ringed”) sideroblasts in various hematologic and non-
hematologic disorders. Blood 18:662, 1961.
cells form under slow deoxygenation. With each sickling–unsickling 27. Hines JD, Grasso JA: The sideroblastic anemias. Semin Hematol 7:86, 1970.
cycle, membrane damage accumulates resulting in the formation of 28. Konstantinidis DG, Pushkaran S, Johnson JF, et al: Signaling and cytoskeletal require-
ments in erythroblast enucleation. Blood 119:6118, 2012.
irreversibly sickled cells (ISCs). 68,69 These cells are incapable of rever- 29. Ubukawa K, Guo YM, Takahashi M, et al: Enucleation of human erythroblasts involves
sion to the biconcave disc shape, even when fully oxygenated. They have non-muscle myosin IIB. Blood 119:1036, 2012.
an increased hemoglobin concentration, increased cation permeability, 30. Keerthivasan G, Small S, Liu H, et al: Vesicle trafficking plays a novel role in erythrob-
decreased potassium, and increased sodium. last enucleation. Blood 116: 3331, 2010.
31. Pan BT, Johnstone RM: Fate of the transferrin receptor during maturation of sheep
reticulocytes in vitro: Selective externalization of the receptor. Cell 33:967, 1983.
Fragmented Cells (Schistocytes) 32. Chasis JA, Prenant M, Leung A, et al: Membrane assembly and remodeling during
Schistocytes (Chap. 51) are seen in microangiopathic hemolytic ane- reticulocyte maturation. Blood 74:1112, 1989.
mias (thrombotic thrombocytopenic purpura [TTP], disseminated 33. Liu J, Guo X, Mohandas N, et al: Membrane remodeling during reticulocyte matura-
tion. Blood 115: 2021, 2010.
intravascular coagulation [DIC], vasculitis, glomerulonephritis, renal 34. Brecher G, Haley JE, et al: Macronormoblasts, macroreticulocytes and macrocytes.
graft rejection), carcinomatosis, heart valve hemolysis (prosthetic or Blood Cells 1:547, 1975.
pathologic valves), severe burns, and march hemoglobinuria (Chap. 35. Jolly JMJ: Recherches sur la formation des globules rouges des mammiféres. Arch Anat
Microsc 9:133, 1907.
51). Fibrin strands in damaged blood vessels can be arrayed so that 36. Felka T, Lemke J, Lemke C, et al: DNA degradation during maturation of erythrocytes—
they sieve the passing red cells. If a passing red cell folds over or other- Molecular cytogenetic characterization of Howell-Jolly bodies. Cytogenet Genome Res
wise attaches to the strand, the bloodstream pulls on the arrested cell, 119:2, 2007.
stretches it, and eventually fragments it. The spleen rapidly removes 37. Holroyde CP, Gardner FH: Acquisition of autophagic vacuoles by human erythrocytes.
70
Physiological role of the spleen. Blood 36:566, 1970.
the schistocytes with a low relative SA:V ratio; the remainder may cir- 38. O’Grady JG, Harding B, Egan EL, et al: “Pitted” erythrocytes: Impaired formation in
culate for many days. splenectomized subjects with congenital spherocytosis. Br J Haematol 57:441, 1984.

Kaushansky_chapter 31_p0459-0478.indd 475 9/18/15 10:59 PM