Comparison of clinical and biological characteristics of ECD patients with or without concomitant myeloid neoplasm
| Variables . | Myeloid neoplasm (n = 19) . | Others (n = 170) . | P . |
|---|---|---|---|
| MH, % | 6 (31.5) | 18 (10.6) | .02 |
| Male sex, % | 17 (89.4) | 115 (68.8) | .06 |
| Age, y | 68 (60-73) | 56.5 (46-66) | .0005 |
| BRAFV600E mutation in ECD or MH, % | 12 (63.2) | 89 (52.3) | .37 |
| JAK2V617F mutation in MPN/MDS, % | 7 (36.8) | 0 (0) | <.0001 |
| Elevated CRP, % | 15 (78.9) | 130 (76.4) | 1.00 |
| Bone scintigraphy or PET uptake, % | 14 (73.7) | 155 (91.1) | .018 |
| Number of sites | 3 (2-4) | 3 (2-5) | .21 |
| Coated aorta, % | 8 (42.1) | 66 (38.8) | .78 |
| Pericardial infiltration, % | 6 (31.5) | 42 (24.7) | .51 |
| Right auricular pseudotumor, % | 6 (31.5) | 57 (33.5) | .86 |
| Xanthelasmas, % | 2 (10.5) | 41 (24.1) | .25 |
| Exophthalmos, % | 4 (21.0) | 36 (21.1) | 1.00 |
| CNS infiltration, % | 2 (10.5) | 68 (40.0) | .01 |
| Pituitary infiltration, % | 2 (10.5) | 35 (20.6) | .37 |
| Perirenal infiltration, % | 14 (73.6) | 94 (55.9) | .12 |
| IFN treatment, % | 13 (68.4) | 110 (64.7) | .74 |
| BRAF inhibitor treatment, % | 7 (36.8) | 30 (17.6) | .045 |
| Death, % | 8 (42.1) | 33 (19.4) | .002 |
| Survival, mo | 82 (40-99) | 364 (129-364) | .001 |
| Variables . | Myeloid neoplasm (n = 19) . | Others (n = 170) . | P . |
|---|---|---|---|
| MH, % | 6 (31.5) | 18 (10.6) | .02 |
| Male sex, % | 17 (89.4) | 115 (68.8) | .06 |
| Age, y | 68 (60-73) | 56.5 (46-66) | .0005 |
| BRAFV600E mutation in ECD or MH, % | 12 (63.2) | 89 (52.3) | .37 |
| JAK2V617F mutation in MPN/MDS, % | 7 (36.8) | 0 (0) | <.0001 |
| Elevated CRP, % | 15 (78.9) | 130 (76.4) | 1.00 |
| Bone scintigraphy or PET uptake, % | 14 (73.7) | 155 (91.1) | .018 |
| Number of sites | 3 (2-4) | 3 (2-5) | .21 |
| Coated aorta, % | 8 (42.1) | 66 (38.8) | .78 |
| Pericardial infiltration, % | 6 (31.5) | 42 (24.7) | .51 |
| Right auricular pseudotumor, % | 6 (31.5) | 57 (33.5) | .86 |
| Xanthelasmas, % | 2 (10.5) | 41 (24.1) | .25 |
| Exophthalmos, % | 4 (21.0) | 36 (21.1) | 1.00 |
| CNS infiltration, % | 2 (10.5) | 68 (40.0) | .01 |
| Pituitary infiltration, % | 2 (10.5) | 35 (20.6) | .37 |
| Perirenal infiltration, % | 14 (73.6) | 94 (55.9) | .12 |
| IFN treatment, % | 13 (68.4) | 110 (64.7) | .74 |
| BRAF inhibitor treatment, % | 7 (36.8) | 30 (17.6) | .045 |
| Death, % | 8 (42.1) | 33 (19.4) | .002 |
| Survival, mo | 82 (40-99) | 364 (129-364) | .001 |
CNS, central nervous system; CRP, C-reactive protein; IFN, interferon-α.