Abstract
Five patients, 11 months to 34/12 years old with severe normochromic, normocytis anemia and reticulocytopenia are reported. At the height of erythropoietic arrest when erythroid precursor cells were completely absent, undifferentiated stem cells (transitional cells), accumulated in the bone marrow. They disappeared again upon spontaneous resumption of erythropoiesis. We suggest that the erythropoietic arrest had occurred at the level of the hematopoietic stem cell.
All patients recovered within 1 week of diagnosis. No steroid therapy was given. Failure to recognize this clinical entity leads to unnecessary diagnostic and therapeutic procedures including the transfusion of blood.
Zusammenfassung
Es wird über 5 Patienten berichtet, die im Alter zwischen 11 Monaten und 34/12 Jahren an einer schweren normochromen normocytären Anämie mit Reticulocytopenie erkrankten. Während des Tiefpunktes der Erythropoese, als die unreifen erythrocytären vorstufen im Knochenmark völlig fehlten, häuften sich undifferenzierte hämopoetische Stammzellen (sog. transitional cells) im Knochenmark an. Wir vermuten, daß der vorübergehende Stop der Erythropoese auf einer Hemmung der Differenzierung hämatopoetischer Stammzellen beruht.
Alle Patienten erholten sich innerhalb 1 Woche nach Stellung der Diagnose, ohne daß eine Behandlung mit Glucocorticoiden eingeleitet wurde. Die Nichterkennung dieser transitorische Form der Aplasie der Erythropoese ist der Anlaß zu unnötigen diagnostischen und therapeutischen Maßnahmen, einschließlich Bluttransfusionen.
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References
Ben-Ishay, Z., Yoffey, J. M.: Ultrastructural studies of erythroblastic islands of rat bone marrow. II. The resumption of erythropoiesis in erythropoietically depressed rebound marrow. Lab. Invest. 26, 637 (1972)
Beutler, E., Dern, R. J., Alving, A. S.: The hemolytic effect of primaquine. VI. An in vitro test for sensitivity of erythrocytes to primaquine. J. Lab. clin. Med. 45, 40 (1955)
Bloom, G. E.: Disorders of bone marrow production. Pediat. Clin. N. Amer. 19, 983 (1972)
Cartwright, G. E.: Diagnostic laboratory hematology. New York-London: Grune and Stratton 1968
Chernoff, A. J., Josephson, A. M.: Acute erythrobastopenia in sickle cell anemia and infectious mononucleosis. Amer. J. Dis. Child. 82, 310 (1951)
Foy, H., Kondi, A., Macdougall, L.: Pure red cell aplasia in marasmus and kwashiorkor treated with riboflavin. Brit. med. J. 1961 I, 937
Gasser, C.: Akute Erythroblastopenie. 10 Fälle aplastischer Erythroblastenkrisen mit Riesenproerythroblasten bei allergisch-toxischen Zustandsbildern. Helv. paediat. Acta 2, 107 (1949)
Gasser, C.: Aplasia of erythropoiesis. Pediat. Clin. N. Amer. 4, 445 (1957)
Kho, L. K., Odang, O., Thajeh, S., Markum, A. H.: Erythroblastopenia (pure red cell aplasia) in childhood in Djakarta. Blood 19, 168 (1962)
Krantz, S. B., Moore, W. H., Zaentz, S. D.: Studies on red cell aplasia. V. Presence of erythroblast cytotoxicity in γ G-globulin fraction of plasma. J. clin. Invest. 52, 324 (1973)
Lovric, V. A.: Anaemia and temporary erythroblastopaenia in children. A syndrome. Aust. Ann. Med. 19, 34 (1970)
Mentzer, W. C., Wang, W. C., Diamond, L. K.: Differentiation of congenital hypoplastic anemia from transient erythroblastopenia (Abstract). Pediat. Res. 9, 324 (1975)
Owren, P. A.: Congenital hemolytic jaundice. The pathogenesis of the “hemolytic crisis”. Blood 3, 231 (1948)
Prindull, G., Prindull, B., Ron, A., Yoffey, J. M.: Cells in spontaneous DNA synthesis in cord blood of premature and full-term newborn infants. An autoradiographic study. J. Pediat. 86, 773 (1975)
Rosse, C., Griffiths, D. A., Edwards, A. E., Gaches, C. G. C., Long, A. H. L., Wright, J. L. W., Yoffey, J. M.: Identity of erythroblast precursors in rat bone marrow. Acta haemat. (Basel) 43, 80 (1970)
Rosse, C., Trotter, J. A.: Cytochemical and radioautographic identification of cells induced to synthesize hemoglobin. Blood 43, 885 (1974)
Sass, M. D., Vorsanger, E., Spear, W.: Enzyme activity as an indicator of red cell age, Clin. chim. Acta 10, 21 (1964)
Schröter, W.: Erythrocyte enzymes. In: Clinical biochemistry (eds. H. C. Curtius, M. Roth) p. 1178. Berlin-New York: Walter de Gruyter 1974
Seip, M.: Aplastic crisis in a case of immunohemolytic anemia. Acta med. scand. 153, 137 (1955)
Selvyn, J. G., Dacie, J. V.: Autohemolysis and other changes resulting from incubation in vitro of red cells from patients with congenital hemolytic anemia. Blood 9, 414 (1954)
Walt, F., Taylor, J., Magill, F. B., Nestadt, A.: Erythroid hypoplasia in kwashiorkor. Brit. med. J. 1962 I, 73
Wehinger, H., Alebouye, M.: Densitrometrisch-quantitative Bestimmung von Hämoglobinen nach Mikrozonen-Elektrophorese auf Cellulose-Acetat-Folie. Klin. Wschr. 48, 701 (1970)
Winter, G. C. B., Byles, A. B., Yoffey, J. M.: Blood lymphocytes in newborn and adult. Lancet 1965 II, 932
Wintrobe, M. M.: Clinical hematology, p. 71. Philadelphia: Lea and Febiger 1974
Wranne, L., Bonnevier, J. O., Killander, A., Killander, J.: Pure red-cell anaemia with proerythroblast maturation arrest. Scand. J. Haemat 7, 73 (1970)
Yoffey, J. M., Thomas, D. B., Moffat, D. J., Sutherland, J. H., Rosse, C.: Non-immunological functions of the lymphocyte. In: Ciba Foundation Study Group No. 10 (eds. G. F. W. Wolstenholme, M. O'Connor), p. 45. London: J. and A. Churchill, Ltd. 1961
Yoffey, J. M.: Bone marrow in hypoxia and rebound. Springfield (Ill.): C. C. Thomas 1974
Young, L. E., Izzo, M. J., Swisher, S. N.: Studies on spontaneous in vitro autohemolysis in hemolytic disorders. Blood 11, 977 (1956)
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Supported by the Deutsche Forschungsgemeinschaft, Bonn-Bad Godesberg.
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Tillmann, W., Prindull, G. & Schröter, W. Severe anemia due to transient pure red cell aplasia in early childhood. Eur J Pediatr 123, 51–58 (1976). https://doi.org/10.1007/BF00497680
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DOI: https://doi.org/10.1007/BF00497680