Demographic, clinical, and laboratory features of iMCD phenotypes
| . | TAFRO n = 65 . | NOS n = 42 . | IPL n = 12 . |
|---|---|---|---|
| Age, mean (SD), y | 33.5 (17.8) | 37.6 (12.8) | 44.0 (10.4) |
| <18, n (%) | 17 (26.2) | 4 (9.5) | 0 (0) |
| Deceased, n (%) | 6 (9.2) | 3 (7.1) | 0 (0) |
| Sex, n (%) | |||
| Female | 25 (38.5) | 27 (64.3) | 8 (66.7) |
| Male | 40 (61.5) | 15 (35.7) | 4 (33.3) |
| Race, n (%) | |||
| American Indian/Alaska Native | 0 (0) | 0 (0) | 1 (8.3) |
| Asian | 7 (10.8) | 1 (2.4) | 6 (50) |
| Black | 6 (9.2) | 6 (14.3) | 0 (0) |
| Native Hawaiian/Pacific Islander | 1 (1.5) | 0 (0) | 0 (0) |
| White | 47 (72.3) | 29 (69) | 4 (33.3) |
| Not provided | 4 (6.2) | 6 (14.3) | 1 (8.3) |
| Histopathological subtype, n (%) | |||
| Hyaline vascular/hypervascular | 46 (70.8) | 33 (78.6) | 1 (8.3) |
| Mixed | 19 (29.2) | 9 (21.4) | 3 (25) |
| Plasmacytic | 0 (0) | 0 (0) | 8 (66.7) |
| Inflammatory disease, n (%) | |||
| Present | 63 (100) | 13 (44.8) | 8 (80) |
| Absent | 0 (0) | 16 (55.1) | 2 (20) |
| Insufficient information | 2 | 13 | 2 |
| Follow-up time, median (IQR), y | 3.3 (1.3-5.0) | 2.0 (1.0-4.6) | 5.7 (1.9-13.6) |
| Clinical symptoms, n/assessed (%) | |||
| Constitutional symptoms | 63/65 (96.9) | 37/41 (90.2) | 11/12 (91.7) |
| Organomegaly | 59/65 (90.8) | 16/40 (40) | 5/12 (41.7) |
| Cherry hemangioma/violaceous papules | 4/56 (7.1) | 0 (0) | 0 (0) |
| Lymphocytic interstitial pneumonitis | 0 (0) | 0 (0) | 0 (0) |
| Fluid retention | 64/65 (98.5) | 23/40 (57.5) | 6/11 (54.5) |
| Platelets, median (IQR), ×103/μL | 83 (51-136) | 283 (219-348) | 451 (373-489) |
| CRP, median (IQR), mg/L | 80.5 (22.7-185.0) | 14.0 (5.0-55.0) | 114.0 (73.0-165.1) |
| ESR, median (IQR), mm/h | 78 (47.5-109.3) | 43 (22-72.0) | 73 (32.5-113.5) |
| Hemoglobin, median (IQR), g/dL | 9.2 (8.0-10.9) | 11.9 (10.8-13.4) | 9.9 (8.0-11.3) |
| Albumin, median (IQR), g/dL | 2.5 (2.1-2.9) | 3.9 (3.3-4.3) | 2.8 (2.5-3.6) |
| Creatinine, median (IQR), mg/dL | 1.3 (1.0-1.8) | 0.8 (0.6-1.0) | 0.8 (0.7-1.0) |
| eGFR, mL/min per 1.73 m2 | |||
| ≤60, n (%) | 32 (68.1) | 18 (56.3) | 5 (62.5) |
| >60, n (%) | 15 (31.9) | 14 (43.8) | 3 (37.5) |
| Not documented, n | 18 | 10 | 4 |
| IgG, median (IQR), mg/dL | 985 (736-1528) | 1137 (988-1264) | 4270 (3190-6178) |
| γ-Globulin, median (IQR), g/dL | 1.1 (0.8-1.7) | 1.2 (0.9-1.5) | 4.4 (4.1-5.9) |
| . | TAFRO n = 65 . | NOS n = 42 . | IPL n = 12 . |
|---|---|---|---|
| Age, mean (SD), y | 33.5 (17.8) | 37.6 (12.8) | 44.0 (10.4) |
| <18, n (%) | 17 (26.2) | 4 (9.5) | 0 (0) |
| Deceased, n (%) | 6 (9.2) | 3 (7.1) | 0 (0) |
| Sex, n (%) | |||
| Female | 25 (38.5) | 27 (64.3) | 8 (66.7) |
| Male | 40 (61.5) | 15 (35.7) | 4 (33.3) |
| Race, n (%) | |||
| American Indian/Alaska Native | 0 (0) | 0 (0) | 1 (8.3) |
| Asian | 7 (10.8) | 1 (2.4) | 6 (50) |
| Black | 6 (9.2) | 6 (14.3) | 0 (0) |
| Native Hawaiian/Pacific Islander | 1 (1.5) | 0 (0) | 0 (0) |
| White | 47 (72.3) | 29 (69) | 4 (33.3) |
| Not provided | 4 (6.2) | 6 (14.3) | 1 (8.3) |
| Histopathological subtype, n (%) | |||
| Hyaline vascular/hypervascular | 46 (70.8) | 33 (78.6) | 1 (8.3) |
| Mixed | 19 (29.2) | 9 (21.4) | 3 (25) |
| Plasmacytic | 0 (0) | 0 (0) | 8 (66.7) |
| Inflammatory disease, n (%) | |||
| Present | 63 (100) | 13 (44.8) | 8 (80) |
| Absent | 0 (0) | 16 (55.1) | 2 (20) |
| Insufficient information | 2 | 13 | 2 |
| Follow-up time, median (IQR), y | 3.3 (1.3-5.0) | 2.0 (1.0-4.6) | 5.7 (1.9-13.6) |
| Clinical symptoms, n/assessed (%) | |||
| Constitutional symptoms | 63/65 (96.9) | 37/41 (90.2) | 11/12 (91.7) |
| Organomegaly | 59/65 (90.8) | 16/40 (40) | 5/12 (41.7) |
| Cherry hemangioma/violaceous papules | 4/56 (7.1) | 0 (0) | 0 (0) |
| Lymphocytic interstitial pneumonitis | 0 (0) | 0 (0) | 0 (0) |
| Fluid retention | 64/65 (98.5) | 23/40 (57.5) | 6/11 (54.5) |
| Platelets, median (IQR), ×103/μL | 83 (51-136) | 283 (219-348) | 451 (373-489) |
| CRP, median (IQR), mg/L | 80.5 (22.7-185.0) | 14.0 (5.0-55.0) | 114.0 (73.0-165.1) |
| ESR, median (IQR), mm/h | 78 (47.5-109.3) | 43 (22-72.0) | 73 (32.5-113.5) |
| Hemoglobin, median (IQR), g/dL | 9.2 (8.0-10.9) | 11.9 (10.8-13.4) | 9.9 (8.0-11.3) |
| Albumin, median (IQR), g/dL | 2.5 (2.1-2.9) | 3.9 (3.3-4.3) | 2.8 (2.5-3.6) |
| Creatinine, median (IQR), mg/dL | 1.3 (1.0-1.8) | 0.8 (0.6-1.0) | 0.8 (0.7-1.0) |
| eGFR, mL/min per 1.73 m2 | |||
| ≤60, n (%) | 32 (68.1) | 18 (56.3) | 5 (62.5) |
| >60, n (%) | 15 (31.9) | 14 (43.8) | 3 (37.5) |
| Not documented, n | 18 | 10 | 4 |
| IgG, median (IQR), mg/dL | 985 (736-1528) | 1137 (988-1264) | 4270 (3190-6178) |
| γ-Globulin, median (IQR), g/dL | 1.1 (0.8-1.7) | 1.2 (0.9-1.5) | 4.4 (4.1-5.9) |
ESR, erythrocyte sedimentation rate; eGFR, estimated glomerular filtration rate; IgG, immunoglobulin G; SD, standard deviation.