Clinical and laboratory characteristics at diagnosis of SM patients with associated myeloid neoplasm
| Characteristic . | No (%) of patients . | Median (range) . | SM-MPN . | SM-CMML . | SM-MDS . | SM-AL . | P . | |||
|---|---|---|---|---|---|---|---|---|---|---|
| SM-MPN vs SM-CMML . | SM-CMML vs SM-MDS . | SM-MPN vs SM-MDS . | Overall . | |||||||
| Total no. of SM | 342 | — | — | — | — | — | — | — | — | — |
| SM associated with myeloid neoplasm | 123 (36) | — | 55 (45) | 36 (29) | 28 (23) | 4 (3) | — | — | — | — |
| Male | 86 (70) | 41 (75) | 24 (67) | 17 (61) | 4 (100) | NS | NS | NS | NS | |
| Age, y | — | 67 (20-87) | 58 (26-83) | 70 (44-87) | 69 (50-80) | 50 (20-67) | < .001 | NS | < .001 | < .001 |
| Follow-up from SM diagnosis, mo | — | 14.5 (0-182.2) | 19.9 (0.9-182.2) | 12 (0.1-135.8) | 12.5 (0-136.3) | 13.3 (2.6-33.8) | NS | NS | .04 | NS |
| Clinical findings | ||||||||||
| Urticaria pigmentosa | 20 (16) | — | 11 (20) | 5 (14) | 4 (14) | 0 | NS | NS | NS | NS |
| Cutaneous symptoms* | 39 (32) | — | 19 (35) | 10 (28) | 10 (36) | 0 | NS | NS | NS | NS |
| Constitutional symptoms† | 80 (65) | — | 35 (64) | 27 (75) | 15 (54) | 3 (75) | NS | NS | NS | NS |
| Mediator-related symptoms‡ | 34 (28) | — | 17 (31) | 9 (25) | 6 (21) | 2 (50) | NS | NS | NS | NS |
| Musculoskeletal symptoms§ | 33 (27) | — | 21 (38) | 7 (19) | 3 (11) | 2 (50) | NS | NS | .01 | .02 |
| Splenomegaly (n = 119) | 67 (61) | — | 30 (56) | 28 (82) | 14 (52) | 1 (25) | .01 | .01 | NS | .02 |
| Leukemic transformation | 16 (13) | — | 6 (11) | 2 (6) | 8 (29) | NA | NS | .02 | NS | .02 |
| Transfusion-dependent | 32 (26) | — | 10 (18) | 8 (22) | 13 (46) | 1 (25) | NS | NS | .01 | .04 |
| Laboratory findings | ||||||||||
| Hemoglobin, g/dL | 123 (100) | 10.6 (6.4-17.4) | 11.8 (6.6-17.4) | 10.6 (7.7-15.4) | 9.5 (6.4-13.3) | 10.3 (7.5-13.4) | .02 | NS | < .001 | < .001 |
| White blood count, × 109/L | 123 (100) | 10.8 (1.2-87.2) | 12.5 (2.2-87.2) | 16.4 (2.8-51.1) | 5.6 (1.2-30.4) | 7.7 (4.6-30.1) | NS | < .001 | < .001 | < .001 |
| AEC ≥ 1.5 | 42 (34) | 31 (56) | 7 (19) | 3 (11) | 1 (25) | < .001 | NS | < .001 | < .001 | |
| Platelets, × 109/L | 123 (100) | 118 (2-1625) | 146 (7-1625) | 111 (12-723) | 122 (2-555) | 215 (90-469) | .02 | NS | NS | NS |
| Albumin (3.5-5.0 g/dL) | 94 (76) | 3.8 (2-4.9) | 3.8 (2-4.9) n = 38 | 3.8 (2.4-4.8) n = 31 | 3.8 (2.3-4.6) n = 22 | 4.1 (3.8-4.4) n = 3 | NS | NS | NS | NS |
| SAP (45-115 U/L) | 117 (95) | 219 (31-3680) | 212.5 (58-2586) n = 50 | 348 (63-3680) n = 35 | 179.5 (31-1546) n = 28 | 194 (67-265) n = 4 | NS | .02 | NS | NS |
| LDH (122-222 U/L) | 89 (72) | 168 (46-926) | 192 (74-912) n = 39 | 167 (91-926) n = 29 | 166 (46-432) n = 19 | 266 (244-288) n = 2 | NS | NS | NS | NS |
| Serum tryptase (< 11.5 ng/mL) | 50 (41) | 69.5 (3.7-1360) | 53.2 (3.7-1360) n = 26 | 94.6 (5.2-303) n = 16 | 56.2 (13.2-517) n = 8 | NA | NS | NS | NS | NS |
| BM, percentage MC | 107 (87) | 15 (1-70) | 10 (1-70) | 15 (5-70) | 15 (5-60) | 5 (5) | NS | NS | NS | NS |
| Abnormal karyotype (n = 102) | 29 (28) | — | 8 (18) n = 44 | 8 (25) n = 32 | 9 (41) n = 22 | 4 (100) n = 4 | NS | NS | NS | .003 |
| FIP1L1-PDFGRA mutation (n = 36) | 12 (33) | — | 12 (50) n = 24 | 8 (0) n = 8 | 4 (0) n = 4 | NA | .01 | NS | NS | .01 |
| KITD816V mutation (n = 80) | 50 (63) | — | 17 (52) n = 33 | 22 (76) n = 29 | 10 (59) n = 17 | 1 (100) n = 1 | NS | NS | NS | NS |
| JAK2V617F mutation (n = 80) | 6 (8) | — | 4 (12) n = 33 | 1 (3) n = 29 | 1 (6) n = 17 | 0 (0) n = 1 | NS | NS | NS | NS |
| Characteristic . | No (%) of patients . | Median (range) . | SM-MPN . | SM-CMML . | SM-MDS . | SM-AL . | P . | |||
|---|---|---|---|---|---|---|---|---|---|---|
| SM-MPN vs SM-CMML . | SM-CMML vs SM-MDS . | SM-MPN vs SM-MDS . | Overall . | |||||||
| Total no. of SM | 342 | — | — | — | — | — | — | — | — | — |
| SM associated with myeloid neoplasm | 123 (36) | — | 55 (45) | 36 (29) | 28 (23) | 4 (3) | — | — | — | — |
| Male | 86 (70) | 41 (75) | 24 (67) | 17 (61) | 4 (100) | NS | NS | NS | NS | |
| Age, y | — | 67 (20-87) | 58 (26-83) | 70 (44-87) | 69 (50-80) | 50 (20-67) | < .001 | NS | < .001 | < .001 |
| Follow-up from SM diagnosis, mo | — | 14.5 (0-182.2) | 19.9 (0.9-182.2) | 12 (0.1-135.8) | 12.5 (0-136.3) | 13.3 (2.6-33.8) | NS | NS | .04 | NS |
| Clinical findings | ||||||||||
| Urticaria pigmentosa | 20 (16) | — | 11 (20) | 5 (14) | 4 (14) | 0 | NS | NS | NS | NS |
| Cutaneous symptoms* | 39 (32) | — | 19 (35) | 10 (28) | 10 (36) | 0 | NS | NS | NS | NS |
| Constitutional symptoms† | 80 (65) | — | 35 (64) | 27 (75) | 15 (54) | 3 (75) | NS | NS | NS | NS |
| Mediator-related symptoms‡ | 34 (28) | — | 17 (31) | 9 (25) | 6 (21) | 2 (50) | NS | NS | NS | NS |
| Musculoskeletal symptoms§ | 33 (27) | — | 21 (38) | 7 (19) | 3 (11) | 2 (50) | NS | NS | .01 | .02 |
| Splenomegaly (n = 119) | 67 (61) | — | 30 (56) | 28 (82) | 14 (52) | 1 (25) | .01 | .01 | NS | .02 |
| Leukemic transformation | 16 (13) | — | 6 (11) | 2 (6) | 8 (29) | NA | NS | .02 | NS | .02 |
| Transfusion-dependent | 32 (26) | — | 10 (18) | 8 (22) | 13 (46) | 1 (25) | NS | NS | .01 | .04 |
| Laboratory findings | ||||||||||
| Hemoglobin, g/dL | 123 (100) | 10.6 (6.4-17.4) | 11.8 (6.6-17.4) | 10.6 (7.7-15.4) | 9.5 (6.4-13.3) | 10.3 (7.5-13.4) | .02 | NS | < .001 | < .001 |
| White blood count, × 109/L | 123 (100) | 10.8 (1.2-87.2) | 12.5 (2.2-87.2) | 16.4 (2.8-51.1) | 5.6 (1.2-30.4) | 7.7 (4.6-30.1) | NS | < .001 | < .001 | < .001 |
| AEC ≥ 1.5 | 42 (34) | 31 (56) | 7 (19) | 3 (11) | 1 (25) | < .001 | NS | < .001 | < .001 | |
| Platelets, × 109/L | 123 (100) | 118 (2-1625) | 146 (7-1625) | 111 (12-723) | 122 (2-555) | 215 (90-469) | .02 | NS | NS | NS |
| Albumin (3.5-5.0 g/dL) | 94 (76) | 3.8 (2-4.9) | 3.8 (2-4.9) n = 38 | 3.8 (2.4-4.8) n = 31 | 3.8 (2.3-4.6) n = 22 | 4.1 (3.8-4.4) n = 3 | NS | NS | NS | NS |
| SAP (45-115 U/L) | 117 (95) | 219 (31-3680) | 212.5 (58-2586) n = 50 | 348 (63-3680) n = 35 | 179.5 (31-1546) n = 28 | 194 (67-265) n = 4 | NS | .02 | NS | NS |
| LDH (122-222 U/L) | 89 (72) | 168 (46-926) | 192 (74-912) n = 39 | 167 (91-926) n = 29 | 166 (46-432) n = 19 | 266 (244-288) n = 2 | NS | NS | NS | NS |
| Serum tryptase (< 11.5 ng/mL) | 50 (41) | 69.5 (3.7-1360) | 53.2 (3.7-1360) n = 26 | 94.6 (5.2-303) n = 16 | 56.2 (13.2-517) n = 8 | NA | NS | NS | NS | NS |
| BM, percentage MC | 107 (87) | 15 (1-70) | 10 (1-70) | 15 (5-70) | 15 (5-60) | 5 (5) | NS | NS | NS | NS |
| Abnormal karyotype (n = 102) | 29 (28) | — | 8 (18) n = 44 | 8 (25) n = 32 | 9 (41) n = 22 | 4 (100) n = 4 | NS | NS | NS | .003 |
| FIP1L1-PDFGRA mutation (n = 36) | 12 (33) | — | 12 (50) n = 24 | 8 (0) n = 8 | 4 (0) n = 4 | NA | .01 | NS | NS | .01 |
| KITD816V mutation (n = 80) | 50 (63) | — | 17 (52) n = 33 | 22 (76) n = 29 | 10 (59) n = 17 | 1 (100) n = 1 | NS | NS | NS | NS |
| JAK2V617F mutation (n = 80) | 6 (8) | — | 4 (12) n = 33 | 1 (3) n = 29 | 1 (6) n = 17 | 0 (0) n = 1 | NS | NS | NS | NS |
Differences in proportions of nominal variables and medians of continuous variables were tested by using Fisher exact test or χ2 test, and Mann-Whitney test or Kruskal-Wallis test, respectively.
SM indicates systemic mastocytosis; AEC, absolute eosinophil count; SAP, serum alkaline phosphatase; LDH, lactate dehydrogenase; MC, mast cell; —, not applicable; NA, not applicable or not assessed; and NS, not significant.
Includes pruritus, flushing, urticaria, and angioedema.
Includes weight loss, fever, chills, and night sweats.
Includes headache, dizziness/lightheadedness, syncope/presyncope, hypotension, anaphylaxis, palpitation/tachycardia, bronchoconstriction/wheezing, and peptic ulcer disease.
Includes bone pain, arthralgias, and myalgias.