Clinical, histologic, immunophenotypic, and genotypic features of ALK+ DLBCL
Feature . | Case 1 . | Case 2 . | Case 3 . | Case 4 . | Case 5 . | Case 6 . |
---|---|---|---|---|---|---|
Age, y/sex | 46/M | 45/F | 49/M | 48/M | 51/M | 58/M |
Clinical presentation | Supraclavicular and abdominal LN | Rapidly growing inguinal tumor | Systemic LN and epidural mass | Axillary LN | Systemic LN and splenomegaly | Supraclavicular LN and subarachnoid involvement |
Stage | III | NA | IV | IA | IVA | IV |
Treatment and outcome | Multiagent CT, relapsed after 5 mo, retreated with CT and RT, CR for 27 mo | NA | CHOP + RT PR, relapsed after 9 mo and has progressive disease | CT alone, CR following 27 mo | CT plus intrathecal MTX, relapsed, BMT, DOD 26 mo after diagnosis | CHOP + rituximab DOD 6 mo after diagnosis |
Histology | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth with necrosis |
ALK staining | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic |
B-cell markers | CD20−, CD79a−, CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ |
Ig staining | Not interpretable | λ+ | IgAλ | IgAλ | IgAλ | Not interpretable |
T-cell markers | CD4+, CD57+ | CD4−/+ | CD4−, CD43− | CD4−/+ | CD4+, CD43− | CD4− |
Others | EMA+, CD30−, Bcl-2− | EMA+, CD30−, Bcl-2− | EMA+, CD30−, Bcl-2− | EMA+, CD30− | EMA+, CD30− | EMA+, CD30− Bcl-2− |
Karyotype | t(2;17;7)(p23;q23;q?22) | NA | NA | NA | NA | NA |
FISH | CLTC-ALK | CLTC-ALK | CLTC-ALK | CLTC-ALK | NA | CLTC-ALK |
RT-PCR | CLTC-ALK and full-length ALK | NA | NA | NA | CLTC-ALK and full-length ALK | NA |
Feature . | Case 1 . | Case 2 . | Case 3 . | Case 4 . | Case 5 . | Case 6 . |
---|---|---|---|---|---|---|
Age, y/sex | 46/M | 45/F | 49/M | 48/M | 51/M | 58/M |
Clinical presentation | Supraclavicular and abdominal LN | Rapidly growing inguinal tumor | Systemic LN and epidural mass | Axillary LN | Systemic LN and splenomegaly | Supraclavicular LN and subarachnoid involvement |
Stage | III | NA | IV | IA | IVA | IV |
Treatment and outcome | Multiagent CT, relapsed after 5 mo, retreated with CT and RT, CR for 27 mo | NA | CHOP + RT PR, relapsed after 9 mo and has progressive disease | CT alone, CR following 27 mo | CT plus intrathecal MTX, relapsed, BMT, DOD 26 mo after diagnosis | CHOP + rituximab DOD 6 mo after diagnosis |
Histology | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth | Immunoblasts, plasmablasts, sinusoidal growth with necrosis |
ALK staining | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic | Granular cytoplasmic |
B-cell markers | CD20−, CD79a−, CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ | CD20−, CD79a− CD138+ |
Ig staining | Not interpretable | λ+ | IgAλ | IgAλ | IgAλ | Not interpretable |
T-cell markers | CD4+, CD57+ | CD4−/+ | CD4−, CD43− | CD4−/+ | CD4+, CD43− | CD4− |
Others | EMA+, CD30−, Bcl-2− | EMA+, CD30−, Bcl-2− | EMA+, CD30−, Bcl-2− | EMA+, CD30− | EMA+, CD30− | EMA+, CD30− Bcl-2− |
Karyotype | t(2;17;7)(p23;q23;q?22) | NA | NA | NA | NA | NA |
FISH | CLTC-ALK | CLTC-ALK | CLTC-ALK | CLTC-ALK | NA | CLTC-ALK |
RT-PCR | CLTC-ALK and full-length ALK | NA | NA | NA | CLTC-ALK and full-length ALK | NA |
LN indicates lymph nodes; M, male; F, female; NA, not available; CT, chemotherapy; RT, radiotherapy; Ig, immunoglobulin; PR, partial remission; DOD, dead of disease; CR, complete remission; MTX, methotrexate; and BMT, bone marrow transplantation.