Clinical and pathologic features of patients with RARS-T
. | . | . | . | . | . | . | Bone marrow morphology . | . | . | . | . | . | . | . | . | . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| WHO diagnosis . | Age . | Hgb, g/L . | Platelets, × 109/L . | MCV, fL . | ANC, × 109/L . | AMC, × 109/L . | Blasts, % . | Cellularity, % . | Megakaryocytes . | Megakaryocyte morphology . | Reticulin fibrosis . | RS, % . | Cytogenetics . | IPSS score . | Splenomegaly, Y/N . | JAK2 V617F mutation . | |||||
| RARS-T | 71 | 93 | 667 | 96.7 | 14.025 | 0.660 | 1 | 90 | Increased and clustered | Some hypolobate nuclei | 2+ | 77 | 47,XX,+8[4]/46,XX[16] | 0.5 | N | Wild type | |||||
| RARS-T | 78 | 89 | 277* | 103.0 | 3.195 | 0.405 | 1 | 70 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 88 | 46,XY[20] | 0 | N | Wild type | |||||
| RARS-T | 73 | 90 | 664 | 86.1 | 5.103 | 0.252 | 1 | 65 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 86 | 46,XY[20] | 0 | Y | Wild type | |||||
| RARS-T | 70 | 102 | 496∥ | 106.3 | 2.337 | 0.369 | 2 | 20 | Normal | Normal | 0 | 51 | 5q- | 0.5 | Y | G/T | |||||
| RARS-T | 61 | 86 | 329† | 86.6‡ | 5.384 | 0.269 | 1 | 95 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 79 | 46,XY[20] | 0 | Y | G/T | |||||
| RARS-T | 85 | 114 | 598 | 104 | 9.690 | 0.800 | 2 | 85 | Increased and focally clustered | Normal | 1+ to 2+ | 42 | 46,XX[20] | 0 | Y | G/T | |||||
| RARS-T | 76 | 102 | 894 | 95.3 | 10.416 | 1.848 | 0 | 90 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 72 | 46,XX[20] | 0 | N | G/T | |||||
| RARS-T | 72 | 94 | 512¶ | 110.8 | 3.490 | 0.820 | 1 | 90 | Increased | Normal | 0 | 55 | 45,X,-X,inv(10) (q21.2q24.3) [3]/46,XX,inv(10) (q21.2q24.3)[17]§ | 0.5 | N | G/T | |||||
| RARS-T | 77 | 124 | 587 | 103.0 | 17.108 | 1.226 | 1 | 60 | Increased and clustered | Large, complex lobulated nuclei | 1+ | 55 | 46,XY[20] | 0 | N | T/T | |||||
. | . | . | . | . | . | . | Bone marrow morphology . | . | . | . | . | . | . | . | . | . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| WHO diagnosis . | Age . | Hgb, g/L . | Platelets, × 109/L . | MCV, fL . | ANC, × 109/L . | AMC, × 109/L . | Blasts, % . | Cellularity, % . | Megakaryocytes . | Megakaryocyte morphology . | Reticulin fibrosis . | RS, % . | Cytogenetics . | IPSS score . | Splenomegaly, Y/N . | JAK2 V617F mutation . | |||||
| RARS-T | 71 | 93 | 667 | 96.7 | 14.025 | 0.660 | 1 | 90 | Increased and clustered | Some hypolobate nuclei | 2+ | 77 | 47,XX,+8[4]/46,XX[16] | 0.5 | N | Wild type | |||||
| RARS-T | 78 | 89 | 277* | 103.0 | 3.195 | 0.405 | 1 | 70 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 88 | 46,XY[20] | 0 | N | Wild type | |||||
| RARS-T | 73 | 90 | 664 | 86.1 | 5.103 | 0.252 | 1 | 65 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 86 | 46,XY[20] | 0 | Y | Wild type | |||||
| RARS-T | 70 | 102 | 496∥ | 106.3 | 2.337 | 0.369 | 2 | 20 | Normal | Normal | 0 | 51 | 5q- | 0.5 | Y | G/T | |||||
| RARS-T | 61 | 86 | 329† | 86.6‡ | 5.384 | 0.269 | 1 | 95 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 79 | 46,XY[20] | 0 | Y | G/T | |||||
| RARS-T | 85 | 114 | 598 | 104 | 9.690 | 0.800 | 2 | 85 | Increased and focally clustered | Normal | 1+ to 2+ | 42 | 46,XX[20] | 0 | Y | G/T | |||||
| RARS-T | 76 | 102 | 894 | 95.3 | 10.416 | 1.848 | 0 | 90 | Increased and clustered | Large; complex lobulated nuclei | 2+ | 72 | 46,XX[20] | 0 | N | G/T | |||||
| RARS-T | 72 | 94 | 512¶ | 110.8 | 3.490 | 0.820 | 1 | 90 | Increased | Normal | 0 | 55 | 45,X,-X,inv(10) (q21.2q24.3) [3]/46,XX,inv(10) (q21.2q24.3)[17]§ | 0.5 | N | G/T | |||||
| RARS-T | 77 | 124 | 587 | 103.0 | 17.108 | 1.226 | 1 | 60 | Increased and clustered | Large, complex lobulated nuclei | 1+ | 55 | 46,XY[20] | 0 | N | T/T | |||||
RARS-T indicates refractory anemia with ringed sideroblasts associated with marked thrombocytosis; MCV, mean cell volume; ANC, absolute neutrophil count; AMC, absolute monocyte count; BM, bone marrow; RS, ringed sideroblasts; and NA, not available.
Documented history of thrombocytosis over previous 6 months (to 1353 × 109/L).
Documented history of thrombocytosis over previous 6 months (546 to 1213 × 109/L).
On hydroxyurea at time of CBC.
Constitutional karyotype of peripheral-blood lymphocytes.
The highest recorded plateiet count was 691 × 109/L; at the time of biopsy patient was receiving Anagrelide.
A platelet count at the time of biopsy was 512 × 109/L blood. The true platelet count was estimated to be higher (per pathologist estimate “close to 1 000 × 109/L”).