Erythroleukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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

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

Types

It can be classified as follows:

  • M6a; Erythroid/Myeloid
  • M6b; Pure Erythroid Malignancy

Criteria for diagnosis of M6

M6a

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 Malignancy )

In rare cases the erythroid lineage is the only obvious components 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 myeloblast, these cases should be considered acute leukemias. In WHO proposal the blastic leukeimas that are limited to the erythroid series are designated pure erythroid malignancy.

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References

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. PMID 10936857.

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