Refractory Cytopenia with Unilineage Dysplasia (RCUD)
Clinical
- Can occur at any age but most frequent in older adults
- RCUD has a low (2%) rate of transformation to acute leukemia
- Median survival 70 months
- May respond to immunosuppressive therapy
International Prognostic Scoring System (IPSS)
Points are assigned based on abnormal findings
| Points: | 0 | 0.5 | 1 | 1.5 | 2 |
|---|---|---|---|---|---|
| BM blast % | <5 | 5-10 | - | 11-20 | 21-30 |
| Cytogenetic Group | Good | Intermediate | Poor | ||
| Number of cytopenic lineages | 0-1 | 2-3 |
- Cytogenetic groups
- Good: Normal, -Y, del(5q), del(20q)
- Intermediate: All others
- Poor: Complex, chromosome 7 abnormalities
- Cytopenias
- Hemoglobin <10 mg/dl
- Absolute neutrophil count <1.8 x 103/μL
- Platelets <100 x 103/μL
Risk groups are determined based on points assigned as above
| Risk Group | Points | Median Survival | Evolution to Acute Leukemia |
|---|---|---|---|
| Low | 0 | 5.7 years | 9.4 years |
| Intermediate-1 | 0.5-1.0 | 3.5 years | 3.3 years |
| Intermediate-2 | 1.5-2.0 | 1.2 years | 1.1 years |
| High | >2.5 | 0.4 years | 0.2 years |
- Treatment based on risk group assigned and ability to tolerate therapy including
- Supportive care
- Immunosuppressive agents
- Bone marrow transplantation
- Intensive cytotoxic treatment
- Demethylating agents
- Farnesyl transferase inhibitors
Blood 89:2079, 1997
