Table 1.
Patient 1 | Patient 2 | |
---|---|---|
Age (y) | 78 | 58 |
Sex | F | F |
Hb level (g/L) | 85 | 114 |
MCV (fL) | 92 | 97 |
Platelet count (109/L) | 743 | 710 |
Leukocytes (109/L) | 6.4 | 7.4 |
Ring sideroblasts (%) | 64 | 24 |
Erythroid dysplasia | Yes | Yes |
Megakaryocytic dysplasia | Marked | Marked |
Excess of blasts | No | No |
Karyotype | Normal | Normal |
SF3B1 | Mutated | N/A |
JAK2V617F | No | No |
MPLW515K/L | No | N/A |
CALR | Unmutated | N/A |
First treatment | RBC transfusions | Watch and wait |
Evolution after 1st treatment | Increased transfusion dependency | Marked increase in the platelet count: 2000 × 109/L |
Second treatment | Lenalidomide 5 mg daily 21 d/28 | Hydroxyurea 500 then 1000 mg/d |
Evolution after 2nd treatment |
Normal platelet count Hb increase to 100 g/L Transfusion frequency reduced |
Adverse effects on hemoglobin levels. Irregular elevated platelet counts ranging from 1700 × 109/L to 3622 × 109/L. The decision to stop hydroxyurea and to start lenalidomide was made after a new increase in platelet count at 3106 × 109/L. EPO was maintained once a week. |
Third treatment | Not applicable | Lenalidomide 5 then 10 mg daily 21 d/28 |
Evolution after 3rd treatment | Not applicable |
Major decrease in platelet count Subnormal Hb levels: 118 g/L EPO every 2 wk |
Abbreviations: EPO, erythropoietin; F, female; Hb, hemoglobin; M, male; MCV, mean corpuscular volume; N, normal; N/A, nonavailable; RBC, red blood cells; y, years.