Table 3 Clinical and laboratory features of 6 patients with MCD
From: The French paediatric cohort of Castleman disease: a retrospective report of 23 patients
Patient/Sex | P18 / F | P19 / F | P20 / M | P21 / F | P22 / M | P23 / M |
|---|---|---|---|---|---|---|
Age at first symptoms (years) | 13 | 7 | 6 | 11 | 10 | 2.8 |
Initial symptoms | Left jugular lymph node | Recurrent fever, arthralgia, hepatomegaly, splenomegaly, abdominal lymph nodes, failure to thrive, fatigue and facial edema | Fever, arthralgia, abdominal pain, abdominal lymph nodes and failure to thrive | Fever, abdominal lymph nodes | Recurrent fevers, hepatomegaly, splenomegaly, abdominal pain, abdominal lymph nodes, trunk rash, vascular hepatopathy and oesophageal varicose veins | Recurrent fevers, hepatomegaly, abdominal lymph nodes, failure to thrive, fatigue, diarrhea, cholestasis and Duchenne muscular dystrophy |
Haemoglobin level, g/dl | 13.6 | 9 | 9 | 8.8 | 10.1 | 10.9 |
Platelet count, x109/L | 261 | 328 | 270 | 250 | 141 | 665 |
IgG level, g/l | 16 | 15 | 18 | 22.4 | - | 36 |
CRP level, mg/l | 7.1 | 67 | 40 | 96 | 46 | 48 |
ESR, mm | 20 | 55 | 75 | - | - | 131 |
Leukocyte count, x109/L | 7.4 | 10 | - | 7 | 8.1 | 14.9 |
Initial diagnosis | - | Primary parvovirus infection | Still disease then familial Mediterranean fever | - | unclassified vasculitis | - |
Initial treatment | - | Colchicine | Aspirin, methotrexate, colchicine, corticosteroids | - | Corticosteroids, hydroxychloroquine, colchicine, NSAID, anakinra | - |
Diagnosis delay (years) | 0 | 3.5 | 7.5 | 1 | 17 | 2 |
Diagnostic investigations | PET scan, lymph node biopsy | Ultrasonography, CT scan, PET scan, liver biopsy, lymph node biopsy | CT scan, PET scan, lymph node biopsy | Ultrasonography, CT scan, lymph node biopsy | CT scan, PET scan, lymph node biopsy | Ultrasonography, CT scan, MRI, lymph node biopsy |
Histological type | Mixed pathology | Plasma cell variant | Mixed pathology | Mixed pathology | ND | Plasma cell variant |
Treatments | Tocilizumab | Tocilizumab | Chemotherapy (cyclophosphamide and vinblastine), rituximab, steroids, anakinra and tocilizumab | Steroids, splenectomy | Tocilizumab | Steroids, tocilizumab |
Follow-up time | 15 months | 6 years | 17 years | 23 years | 1 year | 1 year |
Evolution | Complete remission, no relapse at 3 months after the tocilizumab weaning | Patial remission, no relapse but persistence of hepatic hypermetabolic signals. Fluctuating lymphopenia and thrombocytopenia | Tocilizumab weaning after 4 years of treatment: increased inflammatory markers and headaches. Resumption of tocilizumab allowing for a disappearance of the symptoms. No relapse with tocilizumab | Complete remission, no relapse | Partial remission, no relapse | Partial remission, patient dependent on tocilizumab treatment. Appearance of non-specific inflammatory colitis. |
Genetic variant | ND | MEFV: WT/WT | MEFV: K695R/WT | ND | MEFV: WT/WT | TNFRSF1A: P75L/P75L; MEFV: WT/WT |