History

At presentation, the signs and symptoms of erythroleukemia are usually nonspecific and are attributable to the decreased hematopoiesis resulting from the replacement of bone marrow by leukemic cells. This decrease results in anemia, thrombocytopenia, and leukopenia.^[16]

Patients rarely present with symptoms lasting longer than 6 months, and they are usually diagnosed within 1-3 months after the onset of symptoms. The most common presenting symptoms are as follows:

Fatigue or malaise
Minimal-to-modest weight loss
Easy bruising
Fever
Bone or abdominal pain
Dyspnea
Meningeal signs and symptoms (very rare, only if leukemic involvement of the central nervous system [CNS] is present)
Diffuse joint pain (nonspecific in one third of patients)

Physical

Physical examination findings may include the following:

Pallor (anemia)
Hemorrhage (thrombocytopenia) - Ecchymoses or petechiae; gum bleeding; epistaxis; retinal hemorrhage
Fever and infection (neutropenia) - Respiratory tract, urinary tract, sinuses, perirectal area, or skin
Hepatosplenomegaly (< 25% of cases)
Lymphadenopathy

Complications

Complications may include opportunistic infections and neutropenic fever, tumor lysis syndrome (ie, hyperuricemia, hyperkalemia, hyperphosphatemia), and bleeding.

Infections, even if properly treated, may be fatal. Recognizing patients who are at risk for tumor lysis syndrome (high tumor burden, elevated uric acid) is important. Intravenous hydration and allopurinol should be started before chemotherapy, and serum electrolytes and renal function should be monitored. Patients who have received multiple platelet transfusions may become refractory. To reduce alloimmunization, single-donor platelets or human leukocyte antigen (HLA)-matched platelets with leukocyte reduction filters should be used.

Differential Diagnoses

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Mazzella FM, Alvares C, Kowal-Vern A, Schumacher HR. The acute erythroleukemias. Clin Lab Med. 2000 Mar. 20(1):119-37. [QxMD MEDLINE Link].
Santos FP, Bueso-Ramos CE, Ravandi F. Acute erythroleukemia: diagnosis and management. Expert Rev Hematol. 2010 Dec. 3 (6):705-18. [QxMD MEDLINE Link].
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Acevedo-Olvera LF, Diaz-Garcia H, Parra-Barrera A, Caceres-Perez AA, Gutierrez-Iglesias G, Rangel-Corona R, et al. Inhibition of the Na+/H+ antiporter induces cell death in TF-1 erythroleukemia cells stimulated by the stem cell factor. Cytokine. 2015 Sep. 75 (1):142-50. [QxMD MEDLINE Link].
Calvo X, Arenillas L, Luño E, Senent L, Arnan M, Ramos F, et al. Erythroleukemia shares biological features and outcome with myelodysplastic syndromes with excess blasts: a rationale for its inclusion into future classifications of myelodysplastic syndromes. Mod Pathol. 2016 Dec. 29 (12):1541-1551. [QxMD MEDLINE Link]. [Full Text].
Fagnan A, Bagger FO, Piqué-Borràs MR, et al. Human erythroleukemia genetics and transcriptomes identify master transcription factors as functional disease drivers. Blood. 2020 Aug 6. 136 (6):698-714. [QxMD MEDLINE Link].
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Iacobucci I, Wen J, Meggendorfer M, et al. Genomic subtyping and therapeutic targeting of acute erythroleukemia. Nat Genet. 2019 Apr. 51 (4):694-704. [QxMD MEDLINE Link]. [Full Text].
Mazzella FM, Kowal-Vern A, Shrit MA, et al. Effects of multidrug resistance gene expression in acute erythroleukemia. Mod Pathol. 2000 Apr. 13(4):407-13. [QxMD MEDLINE Link].
Santos FP, Faderl S, Garcia-Manero G, Koller C, Beran M, O'Brien S, et al. Adult acute erythroleukemia: an analysis of 91 patients treated at a single institution. Leukemia. 2009 Sep 10. [QxMD MEDLINE Link].
Kowal-Vern A, Mazzella FM, Cotelingam JD, et al. Diagnosis and characterization of acute erythroleukemia subsets by determining the percentages of myeloblasts and proerythroblasts in 69 cases. Am J Hematol. 2000 Sep. 65(1):5-13. [QxMD MEDLINE Link].
Arber DA, Orazi A, Hasserjian RP, et al. International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022 Sep 15. 140 (11):1200-1228. [QxMD MEDLINE Link]. [Full Text].
Liu W, Hasserjian RP, Hu Y, Zhang L, Miranda RN, Medeiros LJ, et al. Pure erythroid leukemia: a reassessment of the entity using the 2008 World Health Organization classification. Mod Pathol. 2011 Mar. 24(3):375-83. [QxMD MEDLINE Link].
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Media Gallery

Bone marrow aspirate showing erythroblasts in a patient with erythroleukemia. Courtesy of Maurice Barcos, MD, PhD, Department of Pathology, Roswell Park Cancer Institute, Buffalo, NY.

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Author

Beata Holkova, MD Assistant Professor, Massey Cancer Center, Medical College of Virginia, Virginia Commonwealth University School of Medicine

Beata Holkova, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Asher A Chanan-Khan, MD Assistant Professor, Department of Medicine, Division of Lymphoma and Bone Marrow Transplantation, Roswell Park Cancer Institute, State University of New York at Buffalo

Asher A Chanan-Khan, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Hematology

Disclosure: Nothing to disclose.

Kenichi Takeshita, MD Adjunct Associate Professor, Department of Medicine, Division of Hematology, New York University School of Medicine; Medical Director, Clinical Research and Development, Celgene

Kenichi Takeshita, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

David Aboulafia, MD Medical Director, Bailey-Boushay House, Clinical Professor, Department of Medicine, Division of Hematology, Attending Physician, Section of Hematology/Oncology, Virginia Mason Clinic; Investigator, Virginia Mason Community Clinic Oncology Program/SWOG

David Aboulafia, MD is a member of the following medical societies: American College of Physicians, American Medical Association, AMDA - The Society for Post-Acute and Long-Term Care Medicine, American Society of Hematology, Infectious Diseases Society of America, Phi Beta Kappa

Disclosure: Nothing to disclose.

Erythroleukemia Clinical Presentation

History

Physical

Complications

References

Tables

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