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Table 2 Clinical and laboratory features of 17 patients with UCD

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

  1. Hb haemoglobin, CRP C-reactive protein, ESR erythrocyte sedimentation rate, HV hyaline vascular, MP mixed pathology, ND no data, NGS next-generation sequencing, WT wild type