Clinical and laboratory features of 89 consecutive adult patients with moderate to severe eosinophilia
. | . | SMCD-eos . | . | Clonal eosinophilia . | . | . | ||
|---|---|---|---|---|---|---|---|---|
| Study population . | HES* . | Pos . | Neg . | Pos . | Neg . | Reactive eosinophilia* . | ||
| No. of patients | 57 | 10 | 9 | 1 | 4 | 8 | ||
| Age, y, median (range) | 52 (18-83) | 43 (26-72) | 78 (53-85) | 46 (NA) | 60 (20-88) | 50.5 (30-80) | ||
| No. of men (%) | 37 (65) | 10 (100) | 3 (33) | 1 (100) | 4 (100) | 4 (50) | ||
| Eosinophil count, × 109/L, median (range) | 4.2 (1.5-126.8) | 10.3 (2.1-51.3) | 6.1 (2.4-27.7) | 2.1 (NA) | 4.9 (1.7-11.9) | 3.3 (2.0-36.7) | ||
| Organ involvement (% of patients affected) | Skin (40) | Cardiac (30) | Gut (67) | NA | NA | NA | ||
| Lung (42) | Gut (30) | Liver/spleen (67) | ||||||
| Gut (21) | Skin (30) | Skin (44) | ||||||
| Cardiac (10) | Liver/spleen (30) | Lung (11) | ||||||
| Neurologic (8) | Neurologic (30) | |||||||
| Thrombosis (8) | Other (30) | |||||||
| Other (14) | NA | |||||||
| BM mast cell, %, median (range) | NA | 10 (5-30) | 25 (5-70) | NA | NA | NA | ||
| Predominant BM mast cell pattern (no.) | NA | Tight (3) | Tight (7) | NA | NA | |||
| Loose (7) | Loose (2) | |||||||
| No. of patients with abnormal karyotype (total no. studied) | 0 (48) | 2 (9) trisomy 8 + X | 0 (9) | 0 (1) | 4 (4) | 0 (7) | ||
| Imatinib therapy, no. | 10 | 7 | 5 | 1 | 2 | 0 | ||
| OR, no. (%) | 4 (40) | 7 (100) | 0 | 1 (100) | 0 | 0 | ||
| CR, no. (%) | 0 | 7 (100) | 0 | 1 (100) | 0 | 0 | ||
| PR, no. (%) | 4 | 0 | 0 | 0 | 0 | 0 | ||
. | . | SMCD-eos . | . | Clonal eosinophilia . | . | . | ||
|---|---|---|---|---|---|---|---|---|
| Study population . | HES* . | Pos . | Neg . | Pos . | Neg . | Reactive eosinophilia* . | ||
| No. of patients | 57 | 10 | 9 | 1 | 4 | 8 | ||
| Age, y, median (range) | 52 (18-83) | 43 (26-72) | 78 (53-85) | 46 (NA) | 60 (20-88) | 50.5 (30-80) | ||
| No. of men (%) | 37 (65) | 10 (100) | 3 (33) | 1 (100) | 4 (100) | 4 (50) | ||
| Eosinophil count, × 109/L, median (range) | 4.2 (1.5-126.8) | 10.3 (2.1-51.3) | 6.1 (2.4-27.7) | 2.1 (NA) | 4.9 (1.7-11.9) | 3.3 (2.0-36.7) | ||
| Organ involvement (% of patients affected) | Skin (40) | Cardiac (30) | Gut (67) | NA | NA | NA | ||
| Lung (42) | Gut (30) | Liver/spleen (67) | ||||||
| Gut (21) | Skin (30) | Skin (44) | ||||||
| Cardiac (10) | Liver/spleen (30) | Lung (11) | ||||||
| Neurologic (8) | Neurologic (30) | |||||||
| Thrombosis (8) | Other (30) | |||||||
| Other (14) | NA | |||||||
| BM mast cell, %, median (range) | NA | 10 (5-30) | 25 (5-70) | NA | NA | NA | ||
| Predominant BM mast cell pattern (no.) | NA | Tight (3) | Tight (7) | NA | NA | |||
| Loose (7) | Loose (2) | |||||||
| No. of patients with abnormal karyotype (total no. studied) | 0 (48) | 2 (9) trisomy 8 + X | 0 (9) | 0 (1) | 4 (4) | 0 (7) | ||
| Imatinib therapy, no. | 10 | 7 | 5 | 1 | 2 | 0 | ||
| OR, no. (%) | 4 (40) | 7 (100) | 0 | 1 (100) | 0 | 0 | ||
| CR, no. (%) | 0 | 7 (100) | 0 | 1 (100) | 0 | 0 | ||
| PR, no. (%) | 4 | 0 | 0 | 0 | 0 | 0 | ||
The study population was 49 individuals. HES indicates hypereosinophilic syndrome; SMCD-eos, eosinophilia-associated systemic mast cell disease; Neg, negative CHIC2 deletion; Pos, positive CHIC2 deletion; NA, not applicable; BM, bone marrow; OR, overall response; CR, complete response; and PR, partial response.
CHIC2 deletions were all negative for this group.