Acute Promyelocytic Leukemia
| Acute promyelocytic leukemia | |
|---|---|
 |
|
| Neutrophilic promyelocyte | |
| Classification and external resources | |
| Specialty | Hematology and oncology |
| ICD-10 | C92.4 |
| ICD-9-CM | 205.0 |
| ICD-O | M9866/3 |
| OMIM | 102578 |
| DiseasesDB | 34779 |
| eMedicine | med/34 |
| MeSH | D015473 |
Acute promyelocytic leukemia (APML, APL) is the M3 subtype of acute myelogenous leukemia (AML), a cancer of the white blood cells. In APL, there is an abnormal accumulation of immature granulocytes called promyelocytes. The disease is characterized by a chromosomal translocation involving the retinoic acid receptor alpha (RARα or RARA) gene and is distinguished from other forms of AML by its responsiveness to all-trans retinoic acid (ATRA; also known as tretinoin) therapy. Acute promyelocytic leukemia was first characterized in 1957 by French and Norwegian physicians as a hyperacute fatal illness, with a median survival time of less than a week. Today, prognoses have drastically improved; 10-year survival rates are estimated to be approximately 77% according to one study.
The symptoms tend to be similar to AML in general with the following being possible symptoms:
Easy bleeding from low platelets may include:
Acute promyelocytic leukemia is characterized by a chromosomal translocation involving the retinoic acid receptor-alpha gene on chromosome 17 (RARA). In 95% of cases of APL, retinoic acid receptor-alpha (RARA) gene on chromosome 17 is involved in a reciprocal translocation with the promyelocytic leukemia gene (PML) on chromosome 15, a translocation denoted as t(15;17)(q22;q12). The RAR receptor is dependent on retinoic acid for regulation of transcription.
Eight other rare gene rearrangements have been described in APL fusing RARA to promyelocytic leukemia zinc finger (PLZF also known as ZBTB16),nucleophosmin(NPM1), nuclear matrix associated (NUMA1), signal transducer and activator of transcription 5b (STAT5B), protein kinase A regulatory subunit 1α (PRKAR1A), factor interacting with PAPOLA and CPSF1 (FIP1L1), BCL6 corepressor (BCOR) or oligonucleotide/oligosaccharide-binding fold containing 2A (OBFC2A also known as NABP1) genes. Some of these rearrangements are ATRA-sensitive or have unknown sensitivity to ATRA because they are so rare; STAT5B/RARA and PLZF/RARA are known to be resistant to ATRA.
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