Abstract
A severe anemia called pure red cell aplasia (PRCA) develops in 2%–6% of patients with thymomas (Jacobs et al. 1959; Schmid et al. 1965; Salyer and Eggelston 1976), and in certain patients after resection of thymomas (Hirst and Robertson 1967; Safder et al. 1970). Physicians who care for patients with thymomas must therefore be able to recognize PRCA, and must understand its pathogenesis and treatment.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsPreview
Unable to display preview. Download preview PDF.
References
Bjorkholm M, Holm G, Mellsledt H, Carlberger G, Nisill J (1976) Membrane bound IgG on erythroblasts in pure red cell aplasia following thymectomy. A case report. Scand J Haematol 17: 341–346
Brittingham TE, Lutcher CL, Murphy DL (1964) Reversible erythroid aplasia induced by diphenylhydantoin. Arch Intern Med 113: 764–768
Broccia G, Dessalvi P (1983) Acute pure red cell aplasia following bromosulphonphtalein injection in a patient with non Hodgkin lymphoma. Haematologica [Pavia] 68: 680–683
Cavalcant J, Shadduck RK, Winkelstein A, Zeigler Z, Mendelow H (1978) Red-cell hypoplasia and increased bone marrow reticulin in systemic lupus erythematosus: reversal with corticosteroid therapy. Am J Hematol 5: 253–263
Clark DA, Dessypris EN, Krantz SB (1984) Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients. Blood 63: 277–286
Clauvel JP, Vainchenker W, Herrera A, Dellagi K, Vinci G, Tabilio A, Lacombe C (1983) Treatment of pure red cell aplasia by high dose intravenous immunoglobulins. Br J Haematol 55: 380–382
Cornet A, Cornu P, Barbier JP, Favriel JM, Massouline G (1974) Un cas d’érythroblastopénie réversible due au thiamphénicol. Sem Hôp (Paris) 50: 1567–1570
Debusscher L, Paridaens R, Stryckmans P, Delwiche F (1985) Cyclosporine for pure red cell aplasia. Blood 65: 249
Dessypris EN, Krantz SB, Roloff JS, Lukens JN (1982) Mode of action of the IgG inhibitor of erythropoiesis in transient erythroblastopenia of childhood. Blood 59: 114–123
Dessypris EN, Baer MR, Sergent JS, Krantz SB (1984) Rheumatoid arthritis and pure red cell aplasia. Ann Intern Med 100: 202–206
Dessypris EN, Redline S, Harris JW, Krantz SB (1985) Diphenylhydantoin-induced pure red cell aplasia. Blood 65: 789–794
Dunn AM, Kerr GD (1981) Pure red cell aplasia associated with sulphasalazine. Lancet 2: 1288
Fairley KF, Barrie JU, Johnson W (1972) Sterility and testicular atrophy related to cyclophosphamide therapy. Lancet 1: 568–569
Field EO, Caughi MN, Blackett NM, Smithers DW (1968) Marrow-suppressing factors in the blood in pure red-cell aplasia, thymoma and Hodgkin’s disease. Br J Haematol 15: 101–110
Fon GT, Bein ME, Mancuso AA, Kessey JC, Lupetin AR, Wong WS (1982) Computed tomography of the anterior mediastinum in myastenia gravis. Radiology 142: 135–141
Gasser C (1949) Akute Erythroblastopenie: 10 Fälle aplastischer Erythroblastenkrisen mit Riesenproerythroblasten bei allergisch-toxischen Zustandsbildern. Helv Paediatr Acta 2: 107–143
Gasser C (1957) Aplasia of erythropoiesis: acute and chronic erythroblastopenias or pure (red cell) aplastic anemias in childhood. Pediatr Clin North Am 4: 445–468
Goodman SB, Block M H (1964) A case of red cell aplasia oc- curing as a result of antituberculous therapy. Blood 24: 616–623
Greene MH, Boice JD Jr, Greer BE, Blessing JA, Dembo AJ (1982) Acute nonlymphocytic leukemia after therapy with alkylating agents for ovarian cancer: a study of five randomized clinical trials. N Engl J Med 307: 1416–1421
Hamilton CR Jr, Conley CL (1969) Pure red cell aplasia and thymoma. Johns Hopkins Med J 125: 262–269
Hanada T, Abe T, Nakamura H, Aoki Y (1984) Pure red cell aplasia: relationship between inhibitory activity of T cells to CFU-E and erythropoiesis. Br J Haematol 58: 107–113
Hanada T, Abe T, Takita H (1985) T-cell mediated inhibition of erythropoiesis in transient erythroblastopenia of childhood. Br J Haematol 59: 391–392
Hartmann RC, Krantz SB (1974) Paroxysmal nocturnal hemoglobinuria and pure red cell aplasia. Two rare anemias with immunologic implications. Postgrad Med J 55: 141–147
Hilali MA, Joyner MV (1983) Pure red-cell aplasia secondary to angioimmunoblastic lymphadenopathy. J R Soc Med 76: 894–895
Hirst E, Robertson TI (1967) The syndrome of thymoma and erythroblastopenic anemia: a review of 56 cases including 3 case reports. Medicine (Baltimore) 46: 225–264
Ibrahim JM, Rawstron J, Booth J (1966) A case of red cell aplasia in a Negro child. Arch Dis Child 41: 213–215
Jacobs EM, Hutter RVP, Pool JL, Ley AB (1959) Benign thymoma and selective erythroid aplasia of the bone marrow. Cancer 12: 47–57
Jepson JH, Lowenstein L (1968) Panhypoplasia of the bone marrow. I. Demonstration of a plasma factor with anti- erythropoietin-like activity. Can Med Assoc J 99: 99–101
Jurgensen JC, Abraham JP, Hardy WW (1970) Erythroid aplasia after halothane hepatitis: report of a case. Am J Dig Dis 15: 577–581
Krantz SB (1978) Implications of studies on pure red cell aplasia (PRCA) for the study of aplastic anemia. In: Japan Medical Research Foundation (ed) Aplastic anemia. University of Tokyo Press, Tokyo, pp 305–319
Krantz SB, Dessypris EN (1985) Pure red cell aplasia. In: Golde DW, Takaku F (eds) Hematopoietic stem cells. Dekker, New York, pp 229–251
Krantz SB, Kao V (1967) Studies on red cell aplasia. I. Demonstration of a plasma inhibitor to heme synthesis and an antibody to erythroblast nuclei. Proc Nat Acad Sci USA 58: 493–500
Krantz SB, Kao V (1969) Studies on red cell aplasia. II. Report of a second patient with an antibody to erythroblast nuclei and a remission after immunosuppressive therapy. Blood 34: 1–13
Krantz SB, Zaentz SD (1977) Pure red cell aplasia. In: Gordon AS, Silber R, LoBue J (eds) The year in hematology - 1977. Plenum, New York, pp 153–190
Krantz SB, Moore WH, Zaentz SD (1973) Studies on red cell aplasia. V. Presence of erythroblast cytotoxicity in /G-glu- bulin fraction of plasma. J Clin Invest 52: 324–336
Krivoy N, Ben-Arieh Y, Carter A, Alroy G (1981) Methazol- amide-induced hepatitis and pure RBC aplasia. Arch Intern Med 141: 1229–1230
LeGolvan DP, Abell MR (1977) Thymomas. Cancer 39: 2142–2157
Lipton JM, Nadler LM, Canellos GP, Kudisch M, Reiss CS, Nathan DG (1983) Evidence for genetic restriction in the suppression of erythropoiesis by a unique subset of T lymphocytes in man. J Clin Invest 72: 694–706
Litman SD, Zanjani ED (1977) Lymphocytes suppressing both immunoglobulin production and erythroid differentiation in hypogammaglobulinemia. Nature 266: 57–58
MacCulloch D, Jackson JM, Venerys J (1974) Drug-induced red cell aplasia. Br Med J 4: 163–164
MacDougall LG (1982) Pure red cell aplasia associated with sodium valproate therapy. JAMA 247: 53–54
Marmont A, Peschle C, Sanguineti M, Condorelli M (1975) Pure red cell aplasia (PRCA): response of three patients to cyclophosphamide and/or antilymphocyte globullin ( ALG) and demonstration of two types of serum IgG inhibitors to erythropoiesis. Blood 45: 247–261
McGrath BP, Ibels LS, Raik E, Hargrave M, Mahony JF, Stewart JH (1975) Erythroid toxicity of azathioprine: macro- cytosis and selective marrow hypoplasia. Q J Med 44: 57–63
Messner HA, Fauser AA, Curtis JE, Dotten D (1981) Control of antibody-mediated pure red-cell aplasia by plasmapheresis. N Engl J Med 304: 1334–1338
Mielke HG (1958) Aplastische Anämie ( Erythroblastophthise) nach INH-Behandlung. Folia Haematol: 2–10
Min KW, Waddell CC, Pircher FJ, Granville GE, Gyorkey F (1978) Selective uptake of 75Se-selenomethionine by thymoma with pure red cell aplasia. Cancer 41: 1323–1328
Nagasawa T, Abe T, Nakagawa T (1981) Pure red cell aplasia and hypogammaglobulinemia associated with Tr-cell chronic lymphocytic leukemia. Blood 57: 1025–1031
Nicholls MD, Concannon AJ (1982) Maloprim-induced agranulocytosis and red-cell aplasia. Med J Aust2: 564–566
Peschle C, Marmont AM, Marone G, Genovese A, Sasso GF, Condorelli M (1975) Pure red cell aplasia: studies on an IgG serum inhibitor neutralizing erythropoietin. Br J Haematol 30: 411–417
Peschle C, Marmont A, Perugini S, Bernasconi C, Brunetti P, Fontana G, Ghio R, Resegotti L, Rizzo SC, Condorelli M (1978) Physiopathology and therapy of adult pure red cell aplasia (PRCA): a cooperative study. In: Japan Medical Research Foundation (ed) Aplastic anemia. University of Tokyo Press, Tokyo, pp 285–303
Pezzimenti JF, Lindenbaum J (1972) Megaloblastic anemia associated with erythroid hypoplasia. Am J Med 53: 748–754
Recker RR, Hynes HE (1969) Pure red blood cell aplasia associated with chlorpropamide therapy. Arch Intern Med 123: 445–447
Reid G, Patterson AC (1977) Pure red-cell aplasia after gold treatment. Br Med J 2: 1457
Reitz CL, Bottomley SS (1984) Pure red cell aplasia associated with fenoprofen. Am J Med Sci 287: 62–63
Roland AS (1964) The syndrome of benign thymoma and primary aregenerative anemia: an analysis of forty-three cases. Am J Med Sci 247: 719–731
Romano E, Layrisse M, Romano M, Soyano A, Layrisse Z (1980) Electron microscopic demonstration of IgG antibodies directed to erythroblast in primary acquired pure red cell aplasia. Clin Immunol, Immunopathol 17: 330–334
Safdar SH, Krantz SB, Brown EB (1970) Successful immunosuppressive treatment of erythroid aplasia appearing after thymectomy. Br J Haematol 19: 435–443
Salyer WR, Eggleston JC (1976) Thymoma: a clinical and pathological study of 65 cases. Cancer 37: 229–249
Schmid JR, Kiely JM, Pease GL, Hargraves MM (1963) Acquired pure red cell agenesis: report of 16 cases and review of the literature. Acta Haematol (Basel) 30: 255–270
Schmid JR, Kiely JM, Harrison EG Jr, Bayrd ED, Pease GL (1965) Thymoma associated with pure red-cell agenesis: review of literature and report of 4 cases. Cancer 18: 216–230
Serjeant GR, Topley JM, Mason K, Serjeant BE, Pattison JR, Jones SE, Mohamed R (1981) Outbreak of aplastic crises in sickle cell anaemia associated with parvovirus-like agent. Lancet 2: 595–597
Sharff O, Neumann H (1944) Über eine seltene Form von Knochenmarkschädigung durch Salvarsan. Med Klin40: 500
Soutter L, Emerson CP (1960) Elective thymectomy in the treatment of aregenerative anemia associated with onocytic leukemia. Am J Med 28: 609–614
Stephens MEM (1974) Transient erythroid hypoplasia in a patient on long-term co-trimoxazole therapy. Postgrad Med J 50: 235–237
Strauss AM (1943) Erythrocyte aplasia following sulfathiazole. Am J Clin Pathol 13: 249–252
Swineford O Jr, Curry JC, Cumbia JW (1958) Phenylbutazone toxicity: despression of erythropoiesis: a case report. Arthritis Rheum 1: 174–177
Torok-Storb BJ, Sieff C, Storb R, Adamson J, Thomas ED (1980) In vitro tests for distinguishing possible immune mediated aplastic anemia from transfusion-induced sensitization. Blood 55: 211–215
Tötterman TH, Nisell J, Killander A, Gahrton G, Lönnqvist B (1984) Successful treatment of pure red-cell aplasia with cyclosporin. Lancet 2: 693
Tsai SY, Levin WC (1957) Chronic erythrocytic hypoplasia in adults: review of literature and report of a case. Am J Med 22: 322–330
Uldall PR, Kerr DNS, Tacchi D (1972) Sterility and cyclophosphamide. Lancet 1: 693–694
Vilan J, Rhyner K, Ganzoni AM (1973) Pure red cell aplasia: successful treatment with cyclophosphamide. Blut 26: 27–34
Weinberger KA (1979) Fenoprofen and red cell aplasia. J Rheumatol 6: 475
Young N, Harrison M, Moore J, Mortimer P, Humphries RK (1984) Direct demonstration of the human parvovirus in erythroid progenitor cells infected in vitro. J Clin Invest 74: 2024–2032
Young NS, Mortimer PP, Moore JG, Humphries RK (1984) Characterization of a virus that causes transient aplastic crisis. J Clin Invest 73: 224–230
Zaentz SD, Krantz SB, Sears DA (1975) Studies on pure red cell aplasia. VII. Presence of proerythroblasts and response to splenectomy: a case report. Blood 46: 261–270
Zaentz SD, Krantz SB, Brown EB (1976) Studies on pure red cell aplasia. VIII. Maintenance therapy with immunosuppressive drugs. Br J Haematol 32: 47–54
Zeok JV, Todd EP, Dillon M, DeSimone P, Utley JR (1979) The role of thymectomy in red cell aplasia. Ann Thorac Surg 28: 257–260
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1990 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Krantz, S.B. (1990). Pure Red Cell Aplasia. In: Givel, JC., Merlini, M., Clarke, D.B., Dusmet, M. (eds) Surgery of the Thymus. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-71076-6_13
Download citation
DOI: https://doi.org/10.1007/978-3-642-71076-6_13
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-71078-0
Online ISBN: 978-3-642-71076-6
eBook Packages: Springer Book Archive