Acute erythroid leukemia

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Acute erythroid leukemia
Classification and external resources
Specialty Hematology and oncology
ICD-10 C94.0
ICD-9-CM 207.0
ICD-O M9840/3
OMIM 133180
eMedicine med/729
MeSH D004915

Acute erythroid leukemia or Di Guglielmo syndrome is a rare form of acute myeloid leukemia (less than 5% of AML cases[1]) where the myeloproliferation is of erythroblastic precursors. It is defined as type "M6" under the FAB classification.

Signs and symptoms[edit]

The most common symptoms of AEL are related to pancytopenia (a shortage of all types of blood cells), including fatigue, infections, and mucocutaneous bleeding.[2] Almost half of people with AEL exhibit weight loss, fever and night sweats at the time of diagnosis.[2] Almost all people with AEL are anemic, and 77% have a hemoglobin level under 10.0 g/dl.[2] Signs of thrombocytopenia are found in about half of people with AEL.[2]

Causes[edit]

The causes of AEL are unknown.[3] Prior to a 2008 reclassification by the World Health Organization, cases that evolved from myelodysplastic syndromes, myeloproliferative neoplasms, chemotherapy for other cancers or exposure to toxins were defined as secondary AEL.[1] These cases are now likely to instead be classified as acute myeloid leukemia with myelodysplasia-related changes or therapy-related AML.[1]

Diagnosis[edit]

Acute erythroid leukemias can be classified as follows:

M6a (Erythroleukemia)[edit]

Bone marrow smear from a case of erythroleukemia

50% or more of all nucleated bone marrow cells are erythroblasts, Dyserythropoiesis is prominent and 20% or more of the remaining cells (non- erythroid) are myeloblasts.[4][5]

M6b (Pure erythroid leukemia)[edit]

In rare cases the erythroid lineage is the only obvious component of an acute leukemia; a myeloblast component is not apparent. The erythroid component consists predominantly or exclusively of proerythroblasts and early basophilic erythroblasts. These cells may constitute 90% or more of the marrow elements. Despite this lack of myeloblasts, these cases should be considered acute leukemias. In a WHO proposal the blastic leukemias that are limited to the erythroid series are designated pure erythroid malignancies.[6]

M6c (Erythroleukemia and Pure erythroid leukemia)[edit]

Myeloblast- and proerythroblast-rich mixed variant.[7]

Treatment[edit]

Treatment for erythroleukemia generally follows that for other types of AML, not otherwise specified.[1] It consists of chemotherapy, frequently consisting of cytarabine, daunorubicin, and idarubicin.[8] It can also involve bone marrow transplantation.[1]

Prognosis[edit]

Information on prognosis is limited by the rarity of the condition. Prognosis appears to be no different to AML in general, taking into account other risk factors.[9][10] Acute erythroid leukemia (M6) has a relatively poor prognosis. A 2010 study of 124 patients found a median overall survival of 8 months.[10] A 2009 study on 91 patients found a median overall survival for erythroleukemia patients of 36 weeks, with no statistically significant difference to other AML patients. AEL patients did have a significantly shorter disease free survival period, a median of 32 weeks, but this effect was explained by other prognostic factors. That is, AEL is often associated with other risk factors, like monosomal karyotypes and a history of myelodysplastic syndrome.[9] Prognosis is worse in elderly patients, those with a history of myelodysplastic syndrome, and in patients who had previously received chemotherapy for the treatment of a different neoplasm.[1][11]

Epidemiology[edit]

Acute erythroid leukemia is rare, accounting for only 3–5% of all acute myeloid leukemia cases.[2] One study estimated an occurrence rate of 0.077 cases per 100,000 people each year.[12] 64–70% of people with this condition are male, and most are elderly, with a median age of 65.[2]

History[edit]

The first known case of acute erythroid leukemia was described in 1912 by M. Copelli under the name erythromatosis.[2][13] In 1917, Italian hematologist Giovanni Di Guglielmo (1886–1962), expanded on the description, coining the name "eritroleucemia" (Italian for erythroleukemia).[2][14] Di Guglielmo was the first to recognize the leukemic nature of the condition, and it is sometimes referred to as Di Guglielmo's syndrome in recognition of his work.[2]

Chris Squire, bassist from the progressive rock group Yes, died from complications related to acute erythroid leukemia on June 27, 2015.

References[edit]

  1. ^ a b c d e f Zuo Z, Polski JM, Kasyan A, Medeiros LJ (2010). "Acute erythroid leukemia". Arch. Pathol. Lab. Med. 134 (9): 1261–70. PMID 20807044. doi:10.1043/2009-0350-RA.1. 
  2. ^ a b c d e f g h i Santos FP, Bueso-Ramos CE, Ravandi F (2010). "Acute erythroleukemia: diagnosis and management". Expert Rev Hematol. 3 (6): 705–18. PMID 21091147. doi:10.1586/ehm.10.62. 
  3. ^ Naiem F, Rao PN (2009). "Acute Myeloid Leukemia". Hematopathology: Morphology, Immunophenotype, Cytogenetics, and Molecular Approaches. Academic Press. p. 236. ISBN 9780080919485. 
  4. ^ Schwab, Manfred (2011-09-23). Encyclopedia of Cancer. Springer Science & Business Media. p. 1313. ISBN 9783642164828. 
  5. ^ Cheng, Liang; Bostwick, David G. (2011-03-18). Essentials of Anatomic Pathology. Springer Science & Business Media. p. 764. ISBN 9781441960436. 
  6. ^ Armitage, James O. (2004). Atlas of Clinical Hematology. Lippincott Williams & Wilkins. p. 148. ISBN 9780781751285. 
  7. ^ Raghavan, Derek; Brecher, Martin L.; Johnson, David H.; Meropol, Neal J.; Moots, Paul L.; Rose, Peter G. (2006-07-11). Textbook of Uncommon Cancer. John Wiley & Sons. p. 546. ISBN 9780470030554. 
  8. ^ Erythroleukemia ~treatment at eMedicine
  9. ^ a b Santos FP, Faderl S, Garcia-Manero G, et al. (December 2009). "Adult acute erythroleukemia: an analysis of 91 patients treated at a single institution". Leukemia. 23 (12): 2275–80. PMID 19741728. doi:10.1038/leu.2009.181. 
  10. ^ a b Hasserjian RP, Zuo Z, Garcia C, Tang G, Kasyan A, Luthra R, Abruzzo LV, Kantarjian HM, Medeiros LJ, Wang SA (2010). "Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification". Blood. 115 (10): 1985–92. PMC 2942006Freely accessible. PMID 20040759. doi:10.1182/blood-2009-09-243964. 
  11. ^ Orazi, Attilio; O'Malley, Dennis P.; Arber, Daniel A. (2006-07-20). Illustrated Pathology of the Bone Marrow. Cambridge University Press. p. 59. ISBN 9781139455527. 
  12. ^ Wells AW, Bown N, Reid MM, Hamilton PJ, Jackson GH, Taylor PR (2001). "Erythroleukaemia in the north of England: a population based study". J. Clin. Pathol. 54 (8): 608–12. PMC 1731487Freely accessible. PMID 11477115. doi:10.1136/jcp.54.8.608. 
  13. ^ Kasyan A, Medeiros LJ, Zuo Z, Santos FP, Ravandi-Kashani F, Miranda R, Vadhan-Raj S, Koeppen H, Bueso-Ramos CE (2010). "Acute erythroid leukemia as defined in the World Health Organization classification is a rare and pathogenetically heterogeneous disease". Mod. Pathol. 23 (8): 1113–26. PMID 20473273. doi:10.1038/modpathol.2010.96. 
  14. ^ Di Guglielmo G. (1917). "Richerche di ematologia. I. Un caso di eritroleucemia. Megacariociti in circolo e loro funzione piastrinopoietico". Folia Medica (Pavia). 13: 386. 

External links[edit]