Acute erythroid leukemia

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

Acute erythroid leukemia (or "acute Di Guglielmo syndrome") is a rare form of acute myeloid leukemia where the myeloproliferation is of erythroblastic precursors.

It is defined at type "M6" under the FAB classification.^[1]

Types

Acute erythroid leukemias can be classified as follows:

• M6a; Erythroleukemia: Both Erythroid/Myeloid neoplastic proliferation

• M6b; Pure erythroid leukemia

Criteria for diagnosis of M6

M6a (Erythroleukemia)

50% or more of all nucleated bone marrow cells are erythroblasts, Dyserythropoiesis is prominent and 30% or more of the remaining cells (non- erythroid) are myeloblasts.

M6b (Pure erythroid leukemia)

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.

M6c (Erythroleukemia and Pure erythroid leukemia)

Myeloblast- and proerythroblast-rich mixed variant.

Treatment

Treatment for erythroleukemia consists of chemotherapy, frequently consisting of cytarabine, daunorubicin, and idarubicin.^[2]

Prognosis

Acute erythroid leukemia (M6) has a relatively poor prognosis, with median survival for erythroleukemia patients of 36 weeks.^[3] The 36 week prognosis is due to the rareness of M6b. When looked at separately, prognosis comes back a little differently: M6B (3 ± 3.6 months) versus M6A (25 ± 28 months), and M6C (10 ± 13 months).^[4]

In pregnancy

Leukemia is rarely associated with pregnancy, affecting only about 1 in 10,000 pregnant women.^[5] How it is handled depends primarily on the type of leukemia. Acute leukemias normally require prompt, aggressive treatment, despite significant risks of pregnancy loss and birth defects, especially if chemotherapy is given during the developmentally sensitive first trimester.^[5]

See also

• List of hematologic conditions

References

1. ^ "Acute Myeloid Leukemia – Signs and Symptoms". http://www.ucsfhealth.org/adult/medical_services/cancer/leukemia/conditions/aml/signs.html. 
2. ^ Erythroleukemia ~treatment at eMedicine
3. ^ 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. doi:10.1038/leu.2009.181. PMID 19741728. http://www.nature.com/leu/journal/v23/n12/abs/leu2009181a.html. 
4. ^ Kowal-Vern A, Mazzella FM, Cotelingam JD, Shrit MA, Rector JT, Schumacher HR (September 2000). "Diagnosis and characterization of acute erythroleukemia subsets by determining the percentages of myeloblasts and proerythroblasts in 69 cases". Am. J. Hematol. 65 (1): 5–13. doi:10.1002/1096-8652(200009)65:1<5::AID-AJH2>3.0.CO;2-U. PMID 10936857. 
5. ^ ^{a b} Shapira T, Pereg D, Lishner M (September 2008). "How I treat acute and chronic leukemia in pregnancy". Blood Rev. 22 (5): 247–59. doi:10.1016/j.blre.2008.03.006. PMID 18472198. 

External links

• Kowal-Vern A, Mazzella FM, Cotelingam JD, Shrit MA, Rector JT, Schumacher HR (2000). "Diagnosis and characterization of acute erythroleukemia subsets by determining the percentages of myeloblasts and proerythroblasts in 69 cases". Am. J. Hematol. 65 (1): 5–13. doi:10.1002/1096-8652(200009)65:1<5::AID-AJH2>3.0.CO;2-U. PMID 10936857. 
• Histology at University of Virginia
• Overview at Marist College
• Images at Nagoya University