Table 1.
Clinicopathologic features of iMCD-TAFRO patients.
| Patient 1 | Patient 2 | Patient 3 | |
|---|---|---|---|
| Age (years) | 32 | 3 | 20 |
| Sex | M | M | M |
| Fever | Yes | Yes | Yes |
| Anasarca | Large pleural effusion and ascites | Pleural effusion, ascites, pericardial effusion | Pleural effusion, ascites, pericardial effusion |
| Hb (g/dL) | 10.8 | 7.8 | 5.1 |
| PLT (x103/dL) | 32 | 14 | 100 |
| ALP (U/L) | Not available | 108 | 300 |
| LDH (U/L) | Not available | 481 | 409 |
| Viral workup (EBV/HHV-8/adenovirus/rhinovirus) |
Negative | Negative | Negative |
| Autoantibodies | Negative | Negative | Negative |
| Renal insufficiency | Yes | Yes | Yes (Hemodialysis) |
| Organomegaly | Yes | Yes | Yes |
| Lymphadenopathy | Diffuse | Diffuse | Diffuse |
| Castleman like changes in node | Yes | Yes | Yes |
| Flow cytometry in node | Negative | Negative | Negative |
| Bone marrow atypical megakaryocytes | Yes | Yes | Yes |
| Bone marrow fibrosis | Yes (MF1-2/3) | Yes (MF1/3) | Yes (MF1-2/3) |
| Bone marrow flow cytometry | Negative | Negative | Negative |
| JAK2/MPL/CALR mutations | Negative | Negative | Negative |
| Serum IL-6 level | 43.6 pg/mL | Significantly increased | 138.2 pg/mL |
| Anti-IL-6 treatment | Tocilizumab | Tocilizumab | Siltuximab |
| Steroids treatment | Yes | Yes | Yes |
| Follow-up | Recovered, but relapsed | Recovered, no relapse for 3 years | Recovered |