Table 4.
Applicability of the Present Definition of iMCD-TAFRO to the ACCELERATE Natural History Registry Cohort
| iMCD patients in ACCELERATE, who are confirmed by an expert panel as meeting iMCD diagnostic criteria (pathology consistent, multicentric lymphadenopathy, clinical criteria, and exclusionary criteria)* |
(N = 68) | ||
|---|---|---|---|
| TAFRO, N(%) |
NOS, N(%) | Combined | |
| Newly proposed TAFRO criteria met (TAFRO) versus not met (NOS), N(%) | 36 (52.9) | 32 (47.1) | 68 |
| Required TAFRO criteria that were met, N(%) | |||
| At least T (thrombocytopenia), N(%)† | 36 (100) | 4 (12.5)* | 40 (58.8) |
| At least A (anasarca/fluid accumulation), N(%) | 36 (100) | 23 (71.9) | 59 (86.8) |
| At least F (fever or elevated CRP), N(%) | 36 (100) | 26 (81.3) | 62 (91.2) |
| At least O (organomegaly: hepatomegaly, splenomegaly, or lymphadenopathy), N% | 36 (100) | 32 (100) | 68 (100) |
| At least T+A, N(%) | 36 (100) | 4 (12.5) | 40 (58.8) |
| At least T+F, N(%) | 36 (100) | 2 (6.3) | 41 (60.3) |
| At least A+F, N(%) | 36 (100) | 19 (59.4) | 55 (80.9) |
| Additional Clinical Criteria, N(%) | |||
| Renal dysfunction (elevated creatinine or decreased eGFR) | |||
| Present, N(%) | 34 (94.4) | 14 (43.8) | 48 (70.6) |
| Absent, N(%) | 2 (5.6) | 18 (56.3) | 20 (29.4) |
| Not recorded, N | 0 | 0 | 0 |
| Fibrosis of bone marrow ^ | |||
| Present, N(%) | 15 (45.5) | 5 (26.3) | 20 (38.5) |
| Absent, N(%) | 18 (54.5) | 14 (73.7) | 32 (61.5) |
| Not recorded, N | 3 | 13 | 16 |
| Megakaryocytic hyperplasia of bone marrow ^ | |||
| Present, N(%) | 27 (81.8) | 5 (26.3) | 32 (61.5) |
| Absent, N(%) | 6 (18.2) | 14 (73.7) | 20 (38.5) |
| Not recorded, N | 3 | 13 | 16 |
| At least one of R (renal dysfunction, fibrosis of bone marrow, megakaryocytic hyperplasia of bone marrow), N (%) | |||
| Present, N(%) | 36 (100.0) | 17 (53.1) | 53 (77.9) |
| Absent, N(%) | 0 (0.0) | 15 (46.9) | 15 (22.1) |
| Not recorded, N | 0 | 0 | 0 |
| Additional laboratory parameters ‡ | |||
| Gammaglobulin (g/dL, ref: <1.7) | |||
| N | 14 | 14 | 28 |
| Mean (SD) | 1.54 (0.94) | 2.87 (2.08) | 2.20 (1.72) |
| > Upper limits normal, N (%) | 4 (28.6) | 8 (57.1) | 12 (42.9) |
| Immunoglobulin G (mg/dL, ref: <1700) | |||
| N | 29 | 24 | 53 |
| Mean (SD) | 1285.7 (958.6) | 2898.6 (2015.5) | 2916.0 (1717.1) |
| > Upper limits normal, N (%) | 6 (20.7) | 16 (66.7) | 22 (41.5) |
| Alkaline phosphatase (U/L, ref: <147) | |||
| N | 36 | 30 | 66 |
| Mean (SD) | 171.1 (99.7) | 133.0 (93.1) | 153.8 (97.9) |
| > Upper limits normal, N (%) | 16 (44.4) | 7 (23.3) | 23 (34.8) |
| Lactate dehydrogenase (U/L, ref: <400) | |||
| N | 34 | 23 | 57 |
| Mean (SD) | 358.9 (327.2) | 200.7 (160.4) | 295.1 (281.6) |
| > Upper limits normal, N (%) | 8 (23.5) | 3 (13.0) | 11 (19.3) |
Abbreviations: ART, ACCELERATE Registry Team; CAS, Certification and Access Subcommittee; CRP, C-reactive protein; NOS, Not Otherwise Specified
ACCELERATE is an international natural history registry of Castleman disease (CD) patients of all subtypes who enroll directly via a web-based portal, in which they electronically provide consent and Health Insurance Portability and Accountability Act authorization to the ACCELERATE Registry Team (ART). The ART collects and extracts all medical records into the study database. Each case undergoes Certification and Access Subcommittee (CAS) review and grading. CAS-approved grade 3 or higher are those who are considered likely to have CD.
If a bone marrow biopsy was performed and myelofibrosis or megakaryocytic hyperplasia was not documented in the pathology report, then it is considered to be absent. It is possible that a reticulin stain was not performed or a description of megakaryocytic hyperplasia was not included though it may have been present.
Due to the nature of real-world data, thrombocytopenia required at least 2 platelet measurements of <100 k/μL to meet thrombocytopenia in this cohort
Closest value to date of diagnosis within +/− 90 days