From: The French paediatric cohort of Castleman disease: a retrospective report of 23 patients
Patient/Sex | Age at first symptoms (year) | Initial symptoms | Hb g/dl | platelets x109/L | IgG g/l | CRP mg/l | ESR mm/h | Diagnosis delay (year) | Diagnostic investigations | Lymph node localization | Pathological type | Treatments | Follow-up time (year) | Evolution | Genetic variant |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
P1 / M | 0.25 | Cervical lymph node | 11.9 | 371 | 8.12 | - | - | 0.9 | Ultrasonography, CT scan, lymph node biopsy | Cervical | HV | Tocilizumab, steroids, anakinra, surgical excision | 1.5 | No relapse | ND |
P2 / F | 14 | Right cervical lymph node | 13 | 246 | - | <4 | 10 | 0.5 | CT scan, PET scan, lymph node biopsy | Cervical | HV | Surgical excision | 1.5 | No relapse | ND |
P3 / F | 10 | Left cervical lymph node | 12.8 | 304 | - | 6 | 4 | 2.5 | Ultrasonography, CT scan, lymph node cytopunction, lymph node biopsy | Cervical | HV | Surgical excision | 7 | No relapse | ND |
P4 / M | 16.5 | Oral pemphigus | 14.9 | 248 | 14 | 3 | - | 0.5 | CT scan, PET scan, lymph node biopsy | Thoracic | HV | Rituximab, steroids, immunoglobulin, surgical excision | 3 | No relapse | ND |
P5 / F | 14 | Left cervical lymph node | 12.8 | 310 | - | 0.6 | - | 0 | Ultrasonography, CT scan, MRI, PET scan, lymph node cytopunction, lymph node biopsy | Cervical | HV | Steroids, Surgical excision | 5 | No relapse | ND |
P6 / F | 15 | Torsion of an ovarian cyst and hepatosplenomegaly | 7.1 | 791 | 21.2 | 89 | 95 | 0 | PET scan, lymph node cytopunction | Thoracic | ND | Steroids, anakinra, tocilizumab and radiotherapy | 8 | Reduction of pulmonary lymph node after radiotherapy, which remains persistent, no relapse | ND |
P7 / M | 15.5 | Fever and aseptic meningitis | - | - | - | 49 | 61 | 0 | CT scan, lymph node biopsy | Thoracic | MP | Surgical excision | 11 | No relapse but unexplained fever episodes, pericarditis | IL10RA: V406L/WT; IL36RN: S113L/W; MEFV: WT/WT |
P8 / M | 11.5 | Fever and sporadic rectal bleeding | 7.9 | 365 | 21 | 150 | 105 | 0.5 | CT scan, lymph node biopsy | Peritoneal | MP | Surgical excision | 8 | No relapse | ND |
P9 / F | 6 | Bronchopathy, 2 unilateral cervical lymph nodes | 13.7 | 285 | 8.4 | 1 | - | 0 | Ultrasonography, CT scan, lymph node biopsy | Cervical | HV | Surgical excision | 0.75 | No relapse | ND |
P10 / M | 13 | Thoracic lymph node | 14.3 | 237 | 9.2 | 2 | - | 0 | CT scan, lymph node biopsy | Thoracic | HV | No treatment | 18 | Lymph node stability | ND |
P11 / F | 9 | Cervical lymph node | 13.8 | 115 | 6.9 | 1 | - | 1 | Ultrasonography, CT scan, lymph node biopsy | Cervical | HV | Steroids | 16 | No relapse | ND |
P12 / F | 12 | 3 unilateral cervical lymph nodes | 13.5 | 230 | 8.6 | 1 | - | 0 | Ultrasonography, CT scan, lymph node biopsy | Cervical | HV | No treatment | 2.8 | Lymph nodes stability, coeliac disease | ND |
P13 / M | 15 | Cervical lymph node | 14.7 | 310 | 9.4 | 1 | - | 0 | Ultrasonography, lymph node biopsy | Cervical | HV | Surgical excision | 2.9 | No relapse | ND |
P14 / M | 5 | Fever, cervical lymph node evolving since 2 years | 13.5 | 358 | 8.7 | 1 | - | 3 | Ultrasonography, PET scan, lymph node biopsy | Cervical | HV | No treatment | 0.5 | Fever remission, lymph nodes stability | ND |
P15 / F | 10.3 | Cervical lymph node | 13 | 434 | 8.4 | 1 | - | 0.8 | MRI, PET-Scan, lymph node biopsy | Cervical | HV | Surgical excision | 0.7 | No relapse | ND |
P16 / M | 15 | Cervical lymph node | 15.7 | 207 | - | 0.5 | 2 | 1.2 | Ultrasonography, CT scan, lymph node cytopunction, lymph node biopsy, PET scan | Cervical | HV | Surgical excision | 1 | No relapse | ND |
P17 / F | 13 | Abdominal pain, fatigue | 7.9 | 536 | 29.7 | 65 | 61 | 0.7 | Ultrasonography, CT scan, MRI, digestive endoscopy with biopsies, myelogram, lymph node biopsies | Peritoneal | MP | Surgical excision | 3 | No relapse | ND |