Progression of disease in patients with available sequential BM evaluations
| UPN . | Inflammatory manifestations . | Years from disease onset . | WBC × 103/µL . | Hb g/dL . | MCV fL . | Platelets × 103/µL . | Marrow evaluation . | M:E ratio . | Cytogenetics . | Diagnosis . |
|---|---|---|---|---|---|---|---|---|---|---|
| 3 | Fever, joint effusion, synovitis, spondyloarthropathy, hearing loss | 1 | 2.89 | 10.5 | 9.2 | 259 | Normocellular, relative myeloid hyperplasia | 4:1 | Normal | — |
| 4 | 7.03 | 7.9 | 109.9 | 36 | Hypercellular, megakaryocytic and erythroid lineage dysplasia, erythroid hypoplasia, myeloid hyperplasia | 10:1 | t(3:12)(q21;q13) [17/20] | MDS-MLD | ||
| 4 | Fever, rash, medium vessel vasculitis | 3 | 7.13 | 10.2 | 103 | 200 | Normocellular, myeloid hyperplasia, erythroid hypoplasia | 7:1 | Normal | — |
| 6 | 16 | 8.9 | 109.4 | 38 | Hypercellular (100%), myeloid hyperplasia, erythroid hypoplasia, <10% dyspoiesis of myeloid and erythroid precursors | 10:1 | Normal | — | ||
| 6 | Fever, rash, vasculitis, Sweet’s syndrome | 3 | 4.08 | 8.8 | 109.4 | 213 | Normocellular, trilineage hematopoiesis | 4:1 | Normal | — |
| 10 | 2.1 | 10.2 | 102 | 43 | Hypercellular with megakaryocytic and myeloid dysplasia, erythroid hypoplasia | 10:1 | (46,XY,del(5) (q22q33), del(13)(q12q14) [16]/46,XY[4] | MDS-MLD | ||
| 10 | Sweet’s syndrome, ear chondritis, rash | 3 | 5.02 | 9.6 | 111.8 | 110 | Hypercellular with mild dyspoietic features, 8% clonal plasma cells | Normal | — | |
| 4.5 | 2.51 | 9.9 | 116.9 | 46 | Hypercellular, dysplasia in all 3 lineages, 8% clonal plasma cells | Normal | MDS-MLD | |||
| 1 | Fever, rash, flu-like symptoms, hearing loss, diarrhea | 9 | 3.76 | 11.1 | 110 | 108 | Normocellular | 3:1 | Normal | — |
| 10 | 3.08 | 11.1 | 109.8 | 79 | Normocellular | 3:1 | Normal | — | ||
| 8 | Fever, ear chondritis, pulmonary infiltrate, hearing loss | 2 | 3.79 | 10.4 | 129 | 166 | Mildly hypercellular | 3:1 | Normal | — |
| 3 | 5.09 | 8.5 | 125.9 | 115 | Mildly hypercellular | 4:1 | Normal | — |
| UPN . | Inflammatory manifestations . | Years from disease onset . | WBC × 103/µL . | Hb g/dL . | MCV fL . | Platelets × 103/µL . | Marrow evaluation . | M:E ratio . | Cytogenetics . | Diagnosis . |
|---|---|---|---|---|---|---|---|---|---|---|
| 3 | Fever, joint effusion, synovitis, spondyloarthropathy, hearing loss | 1 | 2.89 | 10.5 | 9.2 | 259 | Normocellular, relative myeloid hyperplasia | 4:1 | Normal | — |
| 4 | 7.03 | 7.9 | 109.9 | 36 | Hypercellular, megakaryocytic and erythroid lineage dysplasia, erythroid hypoplasia, myeloid hyperplasia | 10:1 | t(3:12)(q21;q13) [17/20] | MDS-MLD | ||
| 4 | Fever, rash, medium vessel vasculitis | 3 | 7.13 | 10.2 | 103 | 200 | Normocellular, myeloid hyperplasia, erythroid hypoplasia | 7:1 | Normal | — |
| 6 | 16 | 8.9 | 109.4 | 38 | Hypercellular (100%), myeloid hyperplasia, erythroid hypoplasia, <10% dyspoiesis of myeloid and erythroid precursors | 10:1 | Normal | — | ||
| 6 | Fever, rash, vasculitis, Sweet’s syndrome | 3 | 4.08 | 8.8 | 109.4 | 213 | Normocellular, trilineage hematopoiesis | 4:1 | Normal | — |
| 10 | 2.1 | 10.2 | 102 | 43 | Hypercellular with megakaryocytic and myeloid dysplasia, erythroid hypoplasia | 10:1 | (46,XY,del(5) (q22q33), del(13)(q12q14) [16]/46,XY[4] | MDS-MLD | ||
| 10 | Sweet’s syndrome, ear chondritis, rash | 3 | 5.02 | 9.6 | 111.8 | 110 | Hypercellular with mild dyspoietic features, 8% clonal plasma cells | Normal | — | |
| 4.5 | 2.51 | 9.9 | 116.9 | 46 | Hypercellular, dysplasia in all 3 lineages, 8% clonal plasma cells | Normal | MDS-MLD | |||
| 1 | Fever, rash, flu-like symptoms, hearing loss, diarrhea | 9 | 3.76 | 11.1 | 110 | 108 | Normocellular | 3:1 | Normal | — |
| 10 | 3.08 | 11.1 | 109.8 | 79 | Normocellular | 3:1 | Normal | — | ||
| 8 | Fever, ear chondritis, pulmonary infiltrate, hearing loss | 2 | 3.79 | 10.4 | 129 | 166 | Mildly hypercellular | 3:1 | Normal | — |
| 3 | 5.09 | 8.5 | 125.9 | 115 | Mildly hypercellular | 4:1 | Normal | — |
No patients had blasts ≥5%.
Hb, hemoglobin; MCV, mean corpuscular volume; WBC, white blood cell.