Table. 1 Summary of baseline characteristics of 22 patients with SPTCL included in the study
Characteristics | Statistical value |
|---|---|
Male sex (n, %) | 14 (63.6) |
Median age at diagnosis of SPTCL (y, range) | 11.5 (6.0–19.0) |
Past history of HLH/HLH-like systemic illnesses | 3 (13.6) |
Clinical manifestation at diagnosis of SPTCL | |
Presence of HLH/HLH-like systemic illnesses | 15 (68.2) |
Skin lesion | 22 (100) |
Fever | 21 (95.5) |
Hepatomegaly | 13 (59.1) |
Splenomegaly | 13 (59.1) |
Lymphadenopathy | 4 (18.2) |
Neck or submandibular mass | 3 (13.6) |
Periorbital swelling | 3 (13.6) |
Weight loss | 3 (13.6) |
Parotid gland enlargement | 1 (4.5) |
Morphology of skin lesion | |
Subcutaneous nodule | 19 (86.4) |
Patch or plaque | 5 (22.7) |
Macule or papule | 2 (9.1) |
Ulceration | 1 (4.5) |
>1 morphology | 5 (22.7) |
Localization of skin lesion | |
Head or face | 5 (22.7) |
Trunk | 13 (59.1) |
Upper extremities | 14 (63.6) |
Lower extremities | 13 (59.1) |
HAVCR2 mutational status | |
Wild-type | 3 (13.6) |
Homozygous HAVCR2Y82C | 17 (77.3) |
Heterozygous HAVCR2Y82C | 2 (9.1) |
First-line treatment | |
IST-based | 8 (36.4) |
Chemotherapy-based | 14 (63.6) |