| 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]
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Acute erythroid leukemias can be classified as follows:
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.
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.
Myeloblast- and proerythroblast-rich mixed variant.
Treatment for erythroleukemia consists of chemotherapy, frequently consisting of cytarabine, daunorubicin, and idarubicin.[2]
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]
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]
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