Table 1 Clinical, histological, immunophenotypic, and molecular cytogenetic features of pediatric ALK-positive DLBCL
Feature | Case 1 | Case 2 | Case 3 |
|---|---|---|---|
Age/gender | 13/M | 12/F | 16/M |
Clinical presentation | Cervical lymph node | Mediastinal mass, biopsy of cervical lymph node was positive | Mediastinal mass, biopsy of cervical lymph node, left pleura and chest wall was positive |
Stage | II | II | IV (bone marrow positive) |
Treatment and outcome | ALCL99 protocol (standard risk group): multi-agent chemotherapy (course AM2), partial remission | Patient was treated with multiagent chemotherapy. Patient remains in complete remission more than 4 years after diagnosis. | Patient was treated with multiagent chemotherapy. Patient died 1 year after diagnosis despite bone marrow transplant. |
Histology | Immunoblasts, plasmablasts, sinusoidal growth | Lymphoma was of large-cell type with single prominent nucleoli. Some large cells had a plasmablastic appearance, and occasional Reed–Sternberg-like cells were noted | Lymphoma was of large cell type with mostly centroblasts. Occasional plasmablastic cells were noted. |
ALK staining | Granular cytoplasmic + | Granular cytoplasmic + | Granular cytoplasmic + |
B-cell markers: | |||
CD20 | − | − | − |
CD79a | − | − | − |
CD138 | + | + | + |
VS38c | + | + | + |
PAX5 | − | ND | ND |
MUM1 | + | + | + |
BCL2 | − | ND | ND |
CD45 | − | − | − |
CD30 | − | − | − |
EMA | + | + | + |
Ig staining: | |||
Light-chain lambda | + | − | − |
Light-chain kappa | − | + | + |
IgA | − | − | − |
T-cell markers: | |||
CD4 | + | − | − |
Perforin | + | ND | ND |
Molecular cytogenetics | |||
FISH LSI ALK dual color flanking probe | Split | Split | Split |
FISH CLTC dual color flanking probe | ND | Split | NE |
FISH triple color LSI ALK-CLTC | Fusion of ALK and CLTC | NE | NE |
