Erythroleukemia
| 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) where the myeloproliferation is of erythroblastic precursors. It is defined as type "M6" under the FAB classification.
The most common symptoms of AEL are related to pancytopenia (a shortage of all types of blood cells), including fatigue, infections, and mucocutaneous bleeding. Almost half of people with AEL exhibit weight loss, fever and night sweats at the time of diagnosis. Almost all people with AEL are anemic, and 77% have a hemoglobin level under 10.0 g/dl.Signs of thrombocytopenia are found in about half of people with AEL.
The causes of AEL are unknown. 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. These cases are now likely to instead be classified as acute myeloid leukemia with myelodysplasia-related changes or therapy-related AML.
Acute erythroid leukemias can be classified as follows:
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.
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