Castleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key

Table 2 Basic clinical data of 39 patients with Castleman disease (n = 39)

	UCD (n = 18)	iMCD-NOS (n = 13)	TAFRO (n = 8)	P value
Age (yr)	41.17 ± 18.23	47.23 ± 22.09	40.50 ± 9.04	0.530
Male/female	10/8	7/6	4/4	1.000
Rural/urban	5/13a	6/7	6/2a	0.085
Time of diagnosis (median, months)	NA	12	1	0.000
Systemic manifestations
Fever	2	10	8
Splenomegaly	0	5	3
Edema/polyserous cavity effusion	0	0	8
Bronchiolitis obliterans	2	0	0
Rash	0	5	3
Paraneoplastic pemphigus	1	1	0
Abnormal renal function	0	1	7
Laboratory examination
White blood cell1
Decreased	0	2	0
Raise	1	4	3
Hemoglobin1				0.000
Decrease (n)	2
Average (g/L)	132.00 ± 19.985	99.31 ± 27.41	79.57 ± 21.08
Platelet1
Decreased	0	3	8t
Raise	2	6	0
CRP (mg/L)	NA	91.09 ± 59.14	141.55 ± 64.31	0.000
Albumin (g/L)1	39.61 ± 5.52	31.65 ± 7.39	22.79 ± 9.26	0.000
Direct anti-human ball test was positive (n, %)	NA	9/13	6/6
IL-6 (median, pg/mL) (n)	NA	47.35 (8)	12.65 (8)	0.040
VGEF (median, pg/mL) (n)	NA	NA	> 800 (5)
Ferritin > five times normal value	NA	4/13	1/8
Elevated LDH	0	2	2
Elevated ALP	1/18	3/13	6/8
ANA positive (titer > 1:40)	2/18	8/13	2/8
Elevated polyclonal immunoglobulin	0	9/13	1/8
Hemophilia syndrome	0	0	1/8
Pathological type
Hyaline vascular type	13	0	5/8
Plasma cell type	3	4	0
Mixed type	2	9	2/8
No evidence of lymph node biopsy	0	0	1/8
Treatment
Biopsy only, treatment unknown	0	5	0
Symptomatic treatment only	0	0	1
Simple surgical resection	18	0	0
Glucocorticoid alone	0	2	3
Hormones combined with chemotherapy2	0	51	2
Hormone combined with rituximab	0	0	1
Hormone combined with tozumab	0	1	1
Prognosis
Loss of contact	0	3	0
Improved	16	6	7
Relapse	0	0	1
Solid cancer occurred during the follow-up	2b	1c	0
Transformation into lymphoma	0	1d	0
Death	0	2	0

^aA statistically significant difference between the two groups.

^bTwo cases with pancreatic head cancer and thyroid.

^cOne case with pancreatic cancer.

^dOne case with non-Hodgkin lymphoma.

¹Normal reference value: white blood cell count 3.5–9.5 × 10⁹/L; hemoglobin 130–175 g/L (male), 115–150 g/L (female); platelet count 125–350 × 10⁹/L; 40–55 g/L albumin.

²Chemotherapy regimens included CP (cyclophosphamide + prednisone), COP (cyclophosphamide + ciac/vincristine + prednisone), and CHOP (cyclophosphamide + doxorubicin + vincristine + prednisone).

iMCD-NOS: Idiopathic multicentric Castleman disease-not otherwise specified; UCD: Unicentric Castleman disease; ALP: Alkaline phosphatase; ANA: Antinuclear antibody; CRP: C-reactive protein; IL-6: Interleukin 6; LDH: Lactate dehydrogenase; VGEF: Vascular endothelial growth factor.