Table 4.
Patient characteristics: N=44 (10 from RPCI and 34 from the literature)
| Characteristics | |
|---|---|
| Age at diagnosis of MDS, median (range) | 59.2 (16–82) |
| Age at diagnosis of AD, median (range) | 58.8 (16–82) |
| Male gender, n (%) | 31 (70) |
| Synchronous, n (%) | 25 (56) |
| MDS pathology | |
| RA, n (%) | 16 (36) |
| RAEB-1 and 2, n (%) | 15 (34) |
| RARS, n (%) | 6 (14) |
| Other (MDS/MPN), n (%) | 4 (9) |
| CMML, n (%) | 3 (6) |
| Karyotype | |
| Normal, n (%) | 21 (44) |
| ≥2 abnormalities, n (%) | 6 (13) |
| del(5q) (sole), n (%) | 5 (11) |
| del(20q), n (%) | 4 (9) |
| Trisomy 8, n (%) | 3 (6) |
| N/A, n (%) | 9 (20) |
| AD Characteristics | |
| Vasculitis, n (%) | 12 (27) |
| Seronegative arthritis, n (%) | 8 (18) |
| Skin lesions, (Sweet, pyoderma and ictyosis) n (%) | 6 (13) |
| Relapsing polychondritis, n (%) | |
| Pulmonary, n (%) | 4 (9) |
| GI (Colitis and pancreatitis), n (%) | 4 (9) |
| Thyroiditis, n(%) | |
| Hemolytic anemia with DAT, n (%) | 3 (7) |
| Othera n (%) | 3 (7) |
| 3 (7) | |
| 15 (31) | |
| Treatment of MDS | |
| Hypomethylating agents, n (%) | 13 (29) |
| Other, n (%) | 9 (20) |
| None, n (%) | 8 (18) |
| N/A, n (%) | 6 (13) |
| Low-dose cytarabine, n (%) | 4 (9) |
| Transfusion only, n (%) | 4 (9) |
| Treatment of AD | |
| Prednisone alone, n (%) | 21 (47) |
| Prednisone and additional drugs, n (%) | 11 (25) |
| Other, n (%) | 6 (13) |
| None, n (%) | 4 (9) |
| N/A, n (%) | 2 (4) |
| Outcome of AD | |
| Improvement of AD, n (%)b | 31 (70) |
| Worsening, n (%) | 8 (18) |
| N/A, n (%) | 5 (11) |
| Outcome of MDS | |
| Persistent, n (%) | 17 (38) |
| Transformation to AML, n (%) | 11 (25) |
| Improvement of MDS, n (%) | 8 (18) |
| N/A, n (%) | 8 (18) |
Only AD manifestations >5% are presented, less frequent diseases included:
Two cases of rheumatoid arthritis, ITP, nephritis, Sjogren's and myositis each.
One case of PMR, SLE, Uveitis, positive rheumatoid factor and ANA associated with athralgias each.
Only described in patients who received treatment for AD
Abbreviations: AD, autoimmune diseases; AML, acute myeloid leukemia; ANA, antinuclear antibodies; CMML, chronic myelomonocytic leukemia; DAT, direct antiglobulin test; del, deletion; Gl, gastrointestinal; ITP, idiopathic thrombocytopenic purpura; MDS, myelodysplastic syndromes; MPN, myeloproliferative neoplasm; n, number; N/A, not available; PMR, polymyalgia rheumatica; RA, refractory anemia; RAEB, refractory anemia with excess blasts; RARS, refractory anemia with ringed sideroblasts; SLE, systemic lupus erythematous;