|
| | Lim et al. series [20] | This case (MCL-AML) |
| ISM (n = 159) | ASM (n = 41) | SM-AHN (n = 138) |
|
| Demographic data | | | | |
| Age, years | 49 (19–84) | 65 (32–85) | 65 (20–87) | 41 |
| Gender, males/females | 69 (43)/90 (57) | 19 (46)/22 (54) | 97 (70)/41 (30) | Female |
| Clinical features | | | | |
| Time from symptoms to diagnosis, months | 72 (0–516) | 18 (1–372) | 15 (1–360) | 1 month |
| Maculopapular skin lesions | 100 (63) | 15 (37) | 25 (18) | Yes |
| Cutaneous symptoms | 20 (71) | 16 (62) | 10 (83) | Yes |
| Constitutional symptoms | 3 (19) | 24 (59) | 85 (62) | Yes |
| MC mediators-related symptoms | 110 (69) | 9 (22) | 39 (28) | Yes |
| Anaphylactoid reactions | 53 (33) | 2 (5) | 2 (1) | No |
| Musculoskeletal symptoms | 48 (30) | 17 (41) | 41 (30) | No |
| Gastrointestinal symptoms | 113 (71) | 26 (63) | 79 (57) | Yes |
| Hepatomegaly | 22 (14) | 16 (39) | 53 (38) | Yes |
| Splenomegaly | 26 (17) | 18 (44) | 76 (57) | Yes |
| Lymphadenopathy | 22 (14) | 11 (27) | 40 (29) | Yes |
| C-findings | NA | 41 (100) | 36 (26) | Yes |
| BM dysfunction with cytopenia(s) | NA | 13 (32) | NA | Yes |
| Hepatomegaly with functional impairment | NA | 11 (27) | 20 (14) | Yes |
| Splenomegaly with hypersplenism | NA | 9 (22) | 16 (12) | Yes |
| Osteolysis/pathological fractures | NA | 18 (44) | 5 (4) | No |
| Malabsorption with weight loss | NA | 2 (5) | 1 (1) | No |
| Peripheral blood findings | | | | |
| Hemoglobin, g/dl | 13.9 (8.1–16.7) | 11.3 (5.1–16.5) | 10.9 (6.4–17.4) | 11.0 |
| Hemoglobin, <10.0 g/dl | 4 (3) | 10 (24) | 48 (45) | No |
| Platelets, ×109/L | 260 (39–570) | 179 (20–561) | 129 (2–1625) | 16 |
| Platelets, <100 × 109/L | 2 (1) | 11 (27) | 50 (37) | Yes |
| Neutrophils, ×109/L | 4.2 (0.6–12.4) | 4.2 (0.9–17.8) | 4.8 (0.2–42.5) | 0.3 |
| Neutrophils, <100 × 109/L | 2 (1) | 2 (5) | 11 (8) | Yes |
| Serum tryptase, μg/L | 53.6 (11.4–1410) | 145 (10–2000) | 75.4 (3.7–1360) | 184 |
| Increased tryptase: >11.5/>200 μg/L | 89 (99)/11 (12) | 14 (93)/6 (40) | 49 (92)/15 (28) | >11.5/<200 |
| Decreased albumin (<35 g/L) | 10 (9) | 10 (26) | 29 (27) | Yes |
| Increased AP (>115 U/L) | 36 (25) | 24 (60) | 65 (50) | Yes |
| Increased AST (>48)/ALT (>55 U/L) | 10 (7)/4 (7) | 5 (13)/1 (9) | 22 (17)/5 (16) | Yes |
| Increased total bilirubin | 3 (11) | 10 (28) | 42 (32) | Yes |
| Increased LDH (>222 U/L) | 2 (4) | 1 (9) | 25 (25) | Yes |
| Bone marrow findings | | | | |
| BM cellularity: increased/decreased | 49 (32)/15 (10) | 24 (67)/3 (8) | 123 (91)/1 (1) | Increased |
| MC in BM biopsy: <10% | 58 (41) | 9 (26) | 42 (35) | NA |
| MC in BM biopsy: 10–30%/>30% | 68 (48)/15 (11) | 17 (50)/8 (24) | 66 (55)/12 (10) | NA |
| Fibrosis grade 2 or more | 5 (14) | 8 (47) | 31 (46) | Grade 2 |
| MC phenotype, FCM: CD2+/CD25+ | 27 (66)/39 (95) | 4 (50)/8 (100) | 7 (33)/18 (86) | No/Yes |
| MC nuclear morphology: oval/elongated | 87 (78)/24 (22) | 21 (66)/7 (22) | 82 (73)/17 (15) | Mixed |
| MC nuclear morphology: indented/round | 0 (0)/0 (0) | 3 (9)/1 (3) | 10 (9)/3 (3) | |
| Blasts in BM smears: 5–10%/>10% | 0 (0)/0 (0) | 0 (0)/0 (0) | 16 (12)/11 (8) | 24% |
| Molecular and chromosomal aberrancies | | | | |
| KIT D816V mutation | (78) | (82) | (60) | Yes |
| FIP1L1-PDGFRA rearrangement | (52) | NA | NA | No |
| JAK2 V617F mutation | (4) | NA | NA | NA |
| Abnormal karyotype | (5) | (20) | (31) | No |
| Survival and leukemic transformation | | | | |
| Transformation into AML or MCL | 1 (<1) | 2 (5) | 18 (13) | MCL + AML |
| Median survival time from diagnosis, mo | 198 | 41 | 24 | Alive, 24 mo |
| Deaths after median follow-up of 21 mo | 26 (16) | 25 (61) | 99 (72) | NA |
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