Acute erythroid leukemia

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.

Types
Acute erythroid leukemias can be classified as follows:
 * M6a; Erythroleukemia: Both Erythroid/Myeloid neoplastic proliferation


 * M6b; Pure erythroid leukemia

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.

Prognosis
Acute erythroid leukemia (M6) has a relatively poor prognosis, with median survival for erythroleukemia patients of 36 weeks. 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).