Comparison of demographic, clinical, and laboratory characteristics between SM subgroups
| Characteristic . | No. (%) of patients . | Median (range) . | ISM . | ASM . | SM-AHNMD . | P for ISM vs ASM . | P for ASM vs AHNMD . | P for ISM vs AHNMD . | Overall P . |
|---|---|---|---|---|---|---|---|---|---|
| Total no. of SM | 342 | 159 (46) | 41 (12) | 138 (40) | |||||
| Demographic characteristics | |||||||||
| Male | 188 (55) | 69 (43) | 19 (46) | 97 (70) | NS | .005 | < .001 | < .001 | |
| Age, y | 57 (19-87) | 49 (19-84) | 65 (32-85) | 65 (20-87) | < .001 | NS | < .001 | < .001 | |
| Time from symptoms to SM diagnosis, mo | 33 (0-516) | 72 (0-516) | 18 (0.7-372) | 15.1 (0.6-360) | < .001 | NS | < .001 | < .001 | |
| Clinical characteristics | |||||||||
| Urticaria pigmentosa | 140 (41) | 100 (63) | 15 (37) | 25 (18) | .002 | .01 | < .001 | < .001 | |
| Cutaneous symptoms* | 182 (53) | 119 (75) | 20 (49) | 42 (30) | .001 | .03 | < .001 | < .001 | |
| Constitutional symptoms† | 142 (42) | 30 (19) | 24 (59) | 85 (62) | < .001 | NS | < .001 | < .001 | |
| Mediator-related symptoms‡ | 160 (47) | 110 (69) | 9 (22) | 39 (28) | < .001 | NS | < .001 | < .001 | |
| Idiopathic and/or recurrent anaphylactoid reaction | 57 (17) | 53 (33) | 2 (5) | 2 (1) | < .001 | NS | < .001 | < .001 | |
| Musculoskeletal symptoms§ | 107 (31) | 48 (30) | 17 (41) | 41 (30) | NS | NS | NS | .3 | |
| Gastrointestinal symptoms‖ | 221 (65) | 113 (71) | 26 (63) | 79 (57) | NS | NS | .01 | .05 | |
| Hepatomegaly | 92 (27) | 22 (14) | 16 (39) | 53 (38) | < .001 | NS | < .001 | < .001 | |
| Splenomegaly, N = 335 | 123 (37) | 26 (17) | 18 (44) | 76 (57) | < .001 | NS | < .001 | < .001 | |
| Both hepatomegaly and splenomegaly, N = 335 | 72 (21) | 15 (10) | 13 (32) | 43 (32) | < .001 | NS | < .001 | < .001 | |
| Lymphadenopathy¶ | 73 (21) | 22 (14) | 11 (27) | 40 (29) | .05 | NS | .001 | .005 | |
| B-findings | 168 (49) | 53 (33) | 22 (54) | 90 (65) | .02 | NS | < .001 | < .001 | |
| BM MC > 30% or serum tryptase > 200 ng/mL | 60 (18) | 23 (14) | 9 (22) | 26 (19) | NS | NS | NS | .4 | |
| Hypercellular BM or dysmyelopoiesis, without cytopenias | 20 (6) | 13 (8) | 6 (15) | n/a | NS | n/a | n/a | n/a | |
| Hepatosplenomegaly and/or LNP without functional impairment | 144 (42) | 41 (26) | 18 (44) | 83 (60) | .02 | NS | < .001 | < .001 | |
| C-findings | 80 (23) | n/a | 41 (100) | 36 (26) | n/a | < .001 | n/a | n/a | |
| BM dysfunction with cytopenia(s)# | 17 (5) | n/a | 13 (32) | n/a | n/a | n/a | n/a | n/a | |
| Hepatomegaly with functional impairment | 31 (9) | n/a | 11 (27) | 20 (14) | n/a | NS | n/a | n/a | |
| Splenomegaly with hypersplenism | 27 (8) | n/a | 9 (22) | 16 (12) | n/a | NS | n/a | n/a | |
| Osteolysis/pathological fractures | 23 (7) | n/a | 18 (44) | 5 (4) | n/a | < .001 | n/a | n/a | |
| Malabsorption with weight loss | 3 (1) | n/a | 2 (5) | 1 (1) | n/a | NS | n/a | n/a | |
| Leukemic transformation, AML or MCL | 21 (6) | 1 (< 1) | 2 (5) | 18 (13) | NS | NS | < .001 | < .001 | |
| Laboratory characteristics | |||||||||
| Hemoglobin, g/L | 341 (99) | 128 (51-174) | 139 (81-167) | 113 (51-165) | 109 (64-174) | < .001 | NS | < .001 | < .001 |
| Hb < normal | 155 (45) | 105 (51-134) | 28 (18) | 24 (59) | 99 (72) | < .001 | NS | < .001 | < .001 |
| Hb < 100 | 64 (19) | 90 (51-99) | 4 (3) | 10 (24) | 48 (35) | < .001 | NS | < .001 | < .001 |
| White blood cell count, ×109/L | 340 (99) | 7.6 (1.2-87.2) | 6.6 (1.6-19.3) | 8.2 (1.7-37.1) | 10.2 (1.2-87.2) | .01 | NS | < .001 | < .001 |
| WBC > 10 | 109 (32) | 15.4 (10.1-87.2) | 22 (14) | 17 (41) | 70 (51) | < .001 | NS | < .001 | < .001 |
| WBC 4-10 | 198 (58) | 6.6 (4.1-10) | 124 (79) | 21 (51) | 50 (36) | < .001 | NS | < .001 | < .001 |
| ANC, ×109/L | 333 (97) | 4.3 (0.2-42.5) | 4.2 (0.6-12.4) | 4.2 (0.9-17.8) | 4.8 (0.2-42.5) | NS | NS | NS | .3 |
| ANC < 1.0 | 15 (5) | 0.7 (0.2-1) | 2 (1) | 2 (5) | 11 (8) | NS | NS | .005 | .02 |
| AMC, ×109/L | 333 (97) | 0.4 (0-81) | 0.3 (0-81) | 0.5 (0-5.2) | 0.7 (0-11.2) | .003 | NS | < .001 | < .001 |
| ALC, ×109/L | 333 (97) | 1.9 (0-15.8) | 1.9 (0.1-7.6) | 1.9 (0.5-7.4) | 1.7 (0.3-15.8) | NS | NS | NS | .5 |
| AEC, ×109/L | 332 (97) | 0.2 (0-38.4) | 0.1 (0-2.1) | 0.2 (0-9.2) | 0.4 (0-38.4) | .02 | NS | < .001 | < .001 |
| AEC 1.5 or more | 49 (15) | 3.8 (1.5-38.4) | 2 (1) | 10 (25) | 37 (28) | < .001 | NS | < .001 | < .001 |
| Platelet count, ×109/L | 334 (98) | 212 (2-1625) | 260 (39-570) | 179 (20-561) | 129 (2-1625) | .001 | NS | < .001 | < .001 |
| PLT < 150 | 101 (30) | 79 (2-149) | 6 (4) | 16 (39) | 76 (55) | < .001 | NS | < .001 | < .001 |
| PLT < 100 | 65 (19) | 58 (2-97) | 2 (1) | 11 (27) | 50 (37) | < .001 | NS | < .001 | < .001 |
| Albumin, 35-50 g/L | 265 (77) | 39 (20-51) | 41 (30-51) | 38 (20-48) | 38 (20-49) | < .001 | NS | < .001 | < .001 |
| Albumin < 35 | 51 (19) | 31 (20-34) | 10 (9) | 10 (26) | 29 (27) | .005 | NS | .001 | .001 |
| Total protein, 63-79 g/L | 265 (77) | 72 (47-94) | 73 (60-89) | 73 (55-85) | 70 (47-94) | NS | NS | .007 | .03 |
| WNL | 210 (79) | 72 (63-79) | 101 (87) | 28 (76) | 78 (72) | NS | NS | .006 | .02 |
| SAP, 45-115 U/L | 318 (93) | 181 (19-3680) | 142.5 (43-1957) | 269.5 (33-3375) | 206.5 (19-3680) | < .001 | NS | < .001 | < .001 |
| SAP > UNL | 127 (40) | 395 (111-3680) | 36 (25) | 24 (60) | 65 (50) | < .001 | NS | < .001 | < .001 |
| AST, 8-48 U/L | 320 (94) | 18 (1-194) | 19 (1-194) | 17 (6-93) | 18 (5-185) | NS | NS | NS | 1 |
| AST > UNL | 37 (12) | 46 (32-194) | 10 (7) | 5 (13) | 22 (17) | NS | NS | .008 | .03 |
| ALT, 7-55 U/L | 99 (29) | 20 (4-222) | 19.5 (6-117) | 15 (4-122) | 21.5 (7-222) | NS | NS | NS | .3 |
| ALT > UNL | 10 (10) | 82 (47-222) | 4 (7) | 1 (9) | 5 (16) | NS | NS | NS | .4 |
| Total bilirubin, 1.7-17.1 μM/L | 292 (85) | 12 (1.7-133.4) | 10.3 (1.7-68.4) | 12 (3.4-88.9) | 13.7 (3.4-133.4) | NS | NS | < .001 | < .001 |
| T bili > UNL | 65 (22) | 25.7 (18.8-133.4) | 13 (11) | 10 (28) | 42 (32) | .01 | NS | < .001 | < .001 |
| LDH, 122-222 U/L | 164 (48) | 150.5 (11-926) | 127 (11-283) | 146 (97-390) | 170.5 (46-926) | NS | NS | < .001 | < .001 |
| LDH > UNL | 28 (17) | 324.5 (213-926) | 2 (4) | 1 (9) | 25 (25) | NS | NS | .001 | .002 |
| Ferritin, 20-300 μg/L | 105 (31) | 215.9 (6-17 980) | 81 (7-384) | 332.5 (37-2244) | 328 (6-17 980) | .002 | NS | < .001 | < .001 |
| > UNL | 47 (45) | 658 (207-17 980) | 5 (18) | 7 (58) | 34 (54) | .02 | NS | .001 | .004 |
| Serum tryptase, < 11.5 ng/mL | 160 (47) | 63.6 (3.7-2000) | 53.6 (11.4-1410) | 145 (10-2000) | 75.4 (3.7-1360) | .02 | NS | NS | .03 |
| Tryptase 11.5 or less | 154 (96) | 66.6 (11.9-2000) | 89 (99) | 14 (93) | 49 (92) | NS | NS | NS | .08 |
| Tryptase 200 or more | 33 (21) | 303 (200-2000) | 11 (12) | 6 (40) | 15 (28) | .007 | NS | .02 | .009 |
| Urine histamine, < 35 μg/g Cr/24 h | 58 (17) | 62.3 (12-985.6) | 49 (17-985.6) | 210.1 (105-409) | 85.6 (12-843) | .01 | NS | NS | .05 |
| U histamine 35 or more | 41 (71) | 107.6 (36.7-985.6) | 21 (62) | 4 (100) | 15 (79) | NS | NS | NS | .2 |
| Urine N-methylhistamine, 30-200 μg/g Cr | 64 (19) | 354.5 (5-5447) | 335 (33-4156) | 445 (37-775) | 1332.5 (5-5447) | NS | NS | NS | .4 |
| U M-histamine > UNL | 52 (81) | 508.5 (98-5447) | 41 (80) | 6 (86) | 5 (83) | NS | NS | NS | .9 |
| Urine beta PGF2α, 1000 ng or less/24 h | 99 (29) | 2215 (119-21 608) | 1880 (119-13 100) | 1952 (184-21 608) | 4083 (572-18 680) | NS | NS | .007 | .03 |
| U PGF2α > UNL | 74 (75) | 3262 (1132-21 608) | 50 (69) | 9 (82) | 14 (93) | NS | NS | NS | .1 |
| Characteristic . | No. (%) of patients . | Median (range) . | ISM . | ASM . | SM-AHNMD . | P for ISM vs ASM . | P for ASM vs AHNMD . | P for ISM vs AHNMD . | Overall P . |
|---|---|---|---|---|---|---|---|---|---|
| Total no. of SM | 342 | 159 (46) | 41 (12) | 138 (40) | |||||
| Demographic characteristics | |||||||||
| Male | 188 (55) | 69 (43) | 19 (46) | 97 (70) | NS | .005 | < .001 | < .001 | |
| Age, y | 57 (19-87) | 49 (19-84) | 65 (32-85) | 65 (20-87) | < .001 | NS | < .001 | < .001 | |
| Time from symptoms to SM diagnosis, mo | 33 (0-516) | 72 (0-516) | 18 (0.7-372) | 15.1 (0.6-360) | < .001 | NS | < .001 | < .001 | |
| Clinical characteristics | |||||||||
| Urticaria pigmentosa | 140 (41) | 100 (63) | 15 (37) | 25 (18) | .002 | .01 | < .001 | < .001 | |
| Cutaneous symptoms* | 182 (53) | 119 (75) | 20 (49) | 42 (30) | .001 | .03 | < .001 | < .001 | |
| Constitutional symptoms† | 142 (42) | 30 (19) | 24 (59) | 85 (62) | < .001 | NS | < .001 | < .001 | |
| Mediator-related symptoms‡ | 160 (47) | 110 (69) | 9 (22) | 39 (28) | < .001 | NS | < .001 | < .001 | |
| Idiopathic and/or recurrent anaphylactoid reaction | 57 (17) | 53 (33) | 2 (5) | 2 (1) | < .001 | NS | < .001 | < .001 | |
| Musculoskeletal symptoms§ | 107 (31) | 48 (30) | 17 (41) | 41 (30) | NS | NS | NS | .3 | |
| Gastrointestinal symptoms‖ | 221 (65) | 113 (71) | 26 (63) | 79 (57) | NS | NS | .01 | .05 | |
| Hepatomegaly | 92 (27) | 22 (14) | 16 (39) | 53 (38) | < .001 | NS | < .001 | < .001 | |
| Splenomegaly, N = 335 | 123 (37) | 26 (17) | 18 (44) | 76 (57) | < .001 | NS | < .001 | < .001 | |
| Both hepatomegaly and splenomegaly, N = 335 | 72 (21) | 15 (10) | 13 (32) | 43 (32) | < .001 | NS | < .001 | < .001 | |
| Lymphadenopathy¶ | 73 (21) | 22 (14) | 11 (27) | 40 (29) | .05 | NS | .001 | .005 | |
| B-findings | 168 (49) | 53 (33) | 22 (54) | 90 (65) | .02 | NS | < .001 | < .001 | |
| BM MC > 30% or serum tryptase > 200 ng/mL | 60 (18) | 23 (14) | 9 (22) | 26 (19) | NS | NS | NS | .4 | |
| Hypercellular BM or dysmyelopoiesis, without cytopenias | 20 (6) | 13 (8) | 6 (15) | n/a | NS | n/a | n/a | n/a | |
| Hepatosplenomegaly and/or LNP without functional impairment | 144 (42) | 41 (26) | 18 (44) | 83 (60) | .02 | NS | < .001 | < .001 | |
| C-findings | 80 (23) | n/a | 41 (100) | 36 (26) | n/a | < .001 | n/a | n/a | |
| BM dysfunction with cytopenia(s)# | 17 (5) | n/a | 13 (32) | n/a | n/a | n/a | n/a | n/a | |
| Hepatomegaly with functional impairment | 31 (9) | n/a | 11 (27) | 20 (14) | n/a | NS | n/a | n/a | |
| Splenomegaly with hypersplenism | 27 (8) | n/a | 9 (22) | 16 (12) | n/a | NS | n/a | n/a | |
| Osteolysis/pathological fractures | 23 (7) | n/a | 18 (44) | 5 (4) | n/a | < .001 | n/a | n/a | |
| Malabsorption with weight loss | 3 (1) | n/a | 2 (5) | 1 (1) | n/a | NS | n/a | n/a | |
| Leukemic transformation, AML or MCL | 21 (6) | 1 (< 1) | 2 (5) | 18 (13) | NS | NS | < .001 | < .001 | |
| Laboratory characteristics | |||||||||
| Hemoglobin, g/L | 341 (99) | 128 (51-174) | 139 (81-167) | 113 (51-165) | 109 (64-174) | < .001 | NS | < .001 | < .001 |
| Hb < normal | 155 (45) | 105 (51-134) | 28 (18) | 24 (59) | 99 (72) | < .001 | NS | < .001 | < .001 |
| Hb < 100 | 64 (19) | 90 (51-99) | 4 (3) | 10 (24) | 48 (35) | < .001 | NS | < .001 | < .001 |
| White blood cell count, ×109/L | 340 (99) | 7.6 (1.2-87.2) | 6.6 (1.6-19.3) | 8.2 (1.7-37.1) | 10.2 (1.2-87.2) | .01 | NS | < .001 | < .001 |
| WBC > 10 | 109 (32) | 15.4 (10.1-87.2) | 22 (14) | 17 (41) | 70 (51) | < .001 | NS | < .001 | < .001 |
| WBC 4-10 | 198 (58) | 6.6 (4.1-10) | 124 (79) | 21 (51) | 50 (36) | < .001 | NS | < .001 | < .001 |
| ANC, ×109/L | 333 (97) | 4.3 (0.2-42.5) | 4.2 (0.6-12.4) | 4.2 (0.9-17.8) | 4.8 (0.2-42.5) | NS | NS | NS | .3 |
| ANC < 1.0 | 15 (5) | 0.7 (0.2-1) | 2 (1) | 2 (5) | 11 (8) | NS | NS | .005 | .02 |
| AMC, ×109/L | 333 (97) | 0.4 (0-81) | 0.3 (0-81) | 0.5 (0-5.2) | 0.7 (0-11.2) | .003 | NS | < .001 | < .001 |
| ALC, ×109/L | 333 (97) | 1.9 (0-15.8) | 1.9 (0.1-7.6) | 1.9 (0.5-7.4) | 1.7 (0.3-15.8) | NS | NS | NS | .5 |
| AEC, ×109/L | 332 (97) | 0.2 (0-38.4) | 0.1 (0-2.1) | 0.2 (0-9.2) | 0.4 (0-38.4) | .02 | NS | < .001 | < .001 |
| AEC 1.5 or more | 49 (15) | 3.8 (1.5-38.4) | 2 (1) | 10 (25) | 37 (28) | < .001 | NS | < .001 | < .001 |
| Platelet count, ×109/L | 334 (98) | 212 (2-1625) | 260 (39-570) | 179 (20-561) | 129 (2-1625) | .001 | NS | < .001 | < .001 |
| PLT < 150 | 101 (30) | 79 (2-149) | 6 (4) | 16 (39) | 76 (55) | < .001 | NS | < .001 | < .001 |
| PLT < 100 | 65 (19) | 58 (2-97) | 2 (1) | 11 (27) | 50 (37) | < .001 | NS | < .001 | < .001 |
| Albumin, 35-50 g/L | 265 (77) | 39 (20-51) | 41 (30-51) | 38 (20-48) | 38 (20-49) | < .001 | NS | < .001 | < .001 |
| Albumin < 35 | 51 (19) | 31 (20-34) | 10 (9) | 10 (26) | 29 (27) | .005 | NS | .001 | .001 |
| Total protein, 63-79 g/L | 265 (77) | 72 (47-94) | 73 (60-89) | 73 (55-85) | 70 (47-94) | NS | NS | .007 | .03 |
| WNL | 210 (79) | 72 (63-79) | 101 (87) | 28 (76) | 78 (72) | NS | NS | .006 | .02 |
| SAP, 45-115 U/L | 318 (93) | 181 (19-3680) | 142.5 (43-1957) | 269.5 (33-3375) | 206.5 (19-3680) | < .001 | NS | < .001 | < .001 |
| SAP > UNL | 127 (40) | 395 (111-3680) | 36 (25) | 24 (60) | 65 (50) | < .001 | NS | < .001 | < .001 |
| AST, 8-48 U/L | 320 (94) | 18 (1-194) | 19 (1-194) | 17 (6-93) | 18 (5-185) | NS | NS | NS | 1 |
| AST > UNL | 37 (12) | 46 (32-194) | 10 (7) | 5 (13) | 22 (17) | NS | NS | .008 | .03 |
| ALT, 7-55 U/L | 99 (29) | 20 (4-222) | 19.5 (6-117) | 15 (4-122) | 21.5 (7-222) | NS | NS | NS | .3 |
| ALT > UNL | 10 (10) | 82 (47-222) | 4 (7) | 1 (9) | 5 (16) | NS | NS | NS | .4 |
| Total bilirubin, 1.7-17.1 μM/L | 292 (85) | 12 (1.7-133.4) | 10.3 (1.7-68.4) | 12 (3.4-88.9) | 13.7 (3.4-133.4) | NS | NS | < .001 | < .001 |
| T bili > UNL | 65 (22) | 25.7 (18.8-133.4) | 13 (11) | 10 (28) | 42 (32) | .01 | NS | < .001 | < .001 |
| LDH, 122-222 U/L | 164 (48) | 150.5 (11-926) | 127 (11-283) | 146 (97-390) | 170.5 (46-926) | NS | NS | < .001 | < .001 |
| LDH > UNL | 28 (17) | 324.5 (213-926) | 2 (4) | 1 (9) | 25 (25) | NS | NS | .001 | .002 |
| Ferritin, 20-300 μg/L | 105 (31) | 215.9 (6-17 980) | 81 (7-384) | 332.5 (37-2244) | 328 (6-17 980) | .002 | NS | < .001 | < .001 |
| > UNL | 47 (45) | 658 (207-17 980) | 5 (18) | 7 (58) | 34 (54) | .02 | NS | .001 | .004 |
| Serum tryptase, < 11.5 ng/mL | 160 (47) | 63.6 (3.7-2000) | 53.6 (11.4-1410) | 145 (10-2000) | 75.4 (3.7-1360) | .02 | NS | NS | .03 |
| Tryptase 11.5 or less | 154 (96) | 66.6 (11.9-2000) | 89 (99) | 14 (93) | 49 (92) | NS | NS | NS | .08 |
| Tryptase 200 or more | 33 (21) | 303 (200-2000) | 11 (12) | 6 (40) | 15 (28) | .007 | NS | .02 | .009 |
| Urine histamine, < 35 μg/g Cr/24 h | 58 (17) | 62.3 (12-985.6) | 49 (17-985.6) | 210.1 (105-409) | 85.6 (12-843) | .01 | NS | NS | .05 |
| U histamine 35 or more | 41 (71) | 107.6 (36.7-985.6) | 21 (62) | 4 (100) | 15 (79) | NS | NS | NS | .2 |
| Urine N-methylhistamine, 30-200 μg/g Cr | 64 (19) | 354.5 (5-5447) | 335 (33-4156) | 445 (37-775) | 1332.5 (5-5447) | NS | NS | NS | .4 |
| U M-histamine > UNL | 52 (81) | 508.5 (98-5447) | 41 (80) | 6 (86) | 5 (83) | NS | NS | NS | .9 |
| Urine beta PGF2α, 1000 ng or less/24 h | 99 (29) | 2215 (119-21 608) | 1880 (119-13 100) | 1952 (184-21 608) | 4083 (572-18 680) | NS | NS | .007 | .03 |
| U PGF2α > UNL | 74 (75) | 3262 (1132-21 608) | 50 (69) | 9 (82) | 14 (93) | NS | NS | NS | .1 |
ISM indicates indolent systemic mastocytosis (SM); ASM, aggressive SM; SM-AHNMD, SM with associated clonal hematologic non–mast cell lineage disease; MCL, mast cell leukemia; AML, acute myeloid leukemia; No., number; BM, bone marrow; ANC, absolute neutrophil count; AMC, absolute monocyte count; ALC, absolute lymphocyte count; AEC, absolute eosinophil count; LNP, lymphadenopathy; SAP, serum alkaline phosphatase; AST, aspartate transaminase; ALT, alanine transaminase; LDH, lactate dehydrogenase; PG prostaglandin; g, grams; dL, deciliter; L, liter; UNL upper normal limit; WNL, within normal limits; U, units; μg, micrograms; mg, milligrams; ng, nanograms; Cr, creatinine; NS, not significant; and n/a, not applicable.
*, †, ‡, §, ‖, and ¶, please see Table 1 legend.
Absolute neutrophil count < 1.0 ×109/L; hemoglobin < 100 g/L; or platelet count < 100 ×109/L.