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Journal of Clinical Oncology, Vol 26, No 2 (January 10), 2008: pp. 330-331 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.14.1481
Severe Hemolysis As Presenting Sign of Acute ErythroleukemiaDivision of Hematology, Medical University of Graz, Graz, Austria A 45-year-old white male was admitted with fatigue and exercise-induced dyspnea. Apart from pallor, physical examination was unremarkable. Medical history did not reveal previous illnesses or exposure to drugs or toxic agents. Laboratory evaluation revealed signs of marked hemolysis with RBCs 2.05 x 1012/L, hemoglobin 6.4 g/dL, mean corpuscular volume 102.9 fL (80.0 to 98.0 fL), mean corpuscular hemoglobin 31.2 pg (28.0 to 33.0 pg), reticulocytes 0.206 x 1012/L (0.027 to 0.095 x 1012/L), serum lactate dehydrogenase 401 U/L (120 to 240 U/L), total bilirubin 2.04 mg/dL (0.1 to 1.20 mg/dL), and haptoglobin less than 0.07 g/L (0.3 to 2.0 g/L). The indirect and direct antiglobulin tests were negative. A peripheral blood smear stained with May-Grünwald-Giemsa showed marked aniso- and poikilocytosis with dysplastic red cell precursors, but absence of red cell fragments, elliptocytes, and sickle cells (Fig 1A). Leukocytes were 1.57 x 109/L with 15% neutrophils, 80% lymphocytes, 4% monocytes, and 1% basophils; the platelet count was 91 x 109/L. Bone marrow aspiration revealed hypercellularity, whereby 70% of nucleated cells were dysplastic proerythroblasts and erythroblasts, and 45% of nonerythroid cells were myeloblasts, leading to a diagnosis of acute erythroleukemia: FAB M6a according to the FAB and erythroid/myeloid leukemia according to the WHO classification (Fig 1B). Cytogenetic analysis revealed a normal male karyotype. The patient was treated with one cycle cytarabine/idarubicin and two cycles cytarabine/mitoxantrone. Partial remission was achieved and allogeneic cord blood stem cell transplantation performed. Currently, 12 months after transplantation, the patient is still alive without evidence of leukemia.
Acute erythroleukemia is a rare entity comprising 5% to 6% of cases of acute myeloid leukemia. According to the WHO classification it is defined as having  50% erythroid precursors in the entire population of nucleated bone marrow cells, and myeloblasts that account for at least 20% of the nonerythroid cell population (acute erythroid/myeloid leukemia, formerly FAB M6a), or as having 80% immature erythroid precursors with no significant myeloblastic component (pure erythroid leukemia, formerly FAB M6b).1 Patients usually present with symptoms of anemia which most often is profound, but fever, bleeding, and hepatosplenomegaly are also frequently observed at disease onset.2 A clear diagnosis can be established by bone marrow cytology and immunophenotyping, whereas peripheral blood smears can be misleading—approximately half of the patients with acute erythroleukemia are aleukemic.3 This acute myeloid leukemia (AML) subtype is characterized by an aggressive clinical course with low complete remission rates and poor overall survival.2,4 However, through introduction of hematopoietic stem-cell transplantation (HSCT), the prognosis of patients with acute erythroleukemia has substantially improved. In a study by the European Group of Blood and Marrow Transplantation,5 the outcome 5 years after HLA-identical sibling HSCT revealed a leukemia-free survival of 57%.
Hemolytic anemia is caused by premature degradation of RBCs. Although differential diagnoses are manifold, their likelihood varies depending on the patient's race/ethnicity. Approximately 20% to 40% of Africans are heterozygous for sickle cell disease, β-thalassemia is encountered with greatest frequency in people of Mediterranean descent, and glucose-6-phosphate dehydrogenase deficiency occurs with a particularly high frequency in the tropical and subtropical zones of the Eastern Hemisphere.6 Among Northern European and American whites, hereditary spherocytosis, with a prevalence of one in 5,000, as well as autoimmune hemolytic anemia, are the most common reasons for hemolysis.6 Autoimmune hemolytic anemia can be associated with malignant disorders, mainly lymphoid neoplasms,7 but has also been reported in patients with myelodysplastic syndrome 8,9 and AML.10 In addition, This case demonstrates that malignant hematopoietic disorders should also be taken into account in cases of newly diagnosed hemolysis, particularly when dysplastic red cell precursors and/or concomitant cytopenias are present. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES
1. Vardiman JW, Harris NL, Brunning RD: The World Health Organization (WHO) classification of myeloid neoplasms. Blood 100:2292-2302, 2002 2. Olopade OI, Thangavelu M, Larson RA, et al: Clinical, morphologic, and cytogenetic characteristics of 26 patients with acute erythroblastic leukemia. Blood 80:2873-2882, 1992 3. Park S, Picard F, Dreyfus F: Erythroleukemia: A need for a new definition. Leukemia 16:1399-1401, 2002[CrossRef][Medline] 4. Colita A, Belhabri A, Chelghoum Y, et al: Prognostic factors and treatment effects on survival in acute myeloid leukemia of M6 subtype: A retrospective study of 54 cases. Ann Oncol 12:451-455, 2001 5. Fouillard L, Labopin M, Gorin NC, et al: Hematopoietic stem cell transplantation for de novo erythroleukemia: A study of the European Group for Blood and Marrow Transplantation (EBMT). Blood 100:3135-3140, 2002 6. Mentzer WC, Glader B, Parker CJ, et al: Hemolytic Anemia, in Greer JP, Foerster J, Lukens JN, et al (eds): Wintrobe's Clinical Hematology (ed 11). Philadelphia, PA, Lippincott Williams & Wilkins, 2004, pp 1089-1247 7. Engelfriet CP, Overbeeke MA, von dem Borne AE: Autoimmune hemolytic anemia. Semin Hematol 29:3-12, 1992[Medline] 8. Sokol RJ, Hewitt S, Booker DJ: Erythrocyte autoantibodies, autoimmune haemolysis, and myelodysplastic syndromes. J Clin Pathol 42:1088-1091, 1989 9. Pendry K, Harrison C, Geary CG: Myelodysplasia presenting as autoimmune haemolytic anaemia. Br J Haematol 79:133-134, 1991[Medline] 10. Tamura H, Ogata K, Yokose N, et al: Autoimmune hemolytic anemia in patients with de novo acute myelocytic leukemia. Ann Hematol 72:45-47, 1996[CrossRef][Medline] 11. Steensma DP, Gibbons RJ, Higgs DR: Acquired alpha-thalassemia in association with myelodysplastic syndrome and other hematologic malignancies. Blood 105:443-452, 2005 12. Hoyle C, Kaeda J, Leslie J, et al: Acquired beta thalassaemia trait in MDS. Br J Haematol 79:116-117, 1991[Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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- and β-thalassemia may occur as an acquired abnormality in the context of myelodysplastic syndrome and sporadically of other hematologic malignancies, including AML.
