Table 6 Comparison of selected clinical features of the 33 reported cases of ALK-DLBCL

Delsol¹

1

53/M

Systemic LA, splenomegaly

IVA

CTX plus intrathecal MTX, relapsed, BMT

Dead of disease after 26 months

2

15/M

NR

I

COPAD-Ara-C

Alive without disease after 156 months

3

37/M

Mediastinal LA (2)

II (1)

M-BACOD (2)

Cases 3, 4 , 7—dead of disease after 9–33 months

4

44/M

III–IV (4)

B-CHOP (1)

5

67/F

MOPP + XRT (1)

Lost to follow-up after 11 months

6

51/M

ACVBP (2)

Alive without disease after 14 months

7

60/M

See case 3

Gascoyne²

1

46/M

Supraclavicular and abdominal LA

III

CTX, relapse at 5 months, CTX and XRT

Alive without disease after 27 months

2

45/F

Inguinal tumor

NA

NA

NA

3

49/M

Systemic LA, epidural mass

IV

CHOP and XRT, partial response

Alive with progressive disease after 9 months

4

48/M

Axillary LA

IA

CTX

Alive without disease after 27 months

5

(Delsol case 1)

6

58/M

Supraclavicular LA and subarachnoid involvement

IV

CHOP+Rituximab

Dead of disease after 6 months

De Paepe³

1

10/M

Cervical mass

II

ALCL-99 HR followed by SFOP-LMB 96 two years later

Alive without disease after 6 months

2

13/F

Cervical LA, HSM, mediastinal mass (at relapse)

III

NHL-BFM ALCL99 with ALCL relapse, BMT

Dead of disease after 3 months

3

26/M

Cervical LA, base of tongue tumor

II

CHOP × 4/VIM × 1/DHAP × 1, neck and upper mediastinum XRT, progressive disease, development of skeletal lesions, hyper-C-VAD, and BMT after BEAM

Alive without disease after 44 months

Chikatsu⁴

1

36/F

Multiple intramuscular tumors, bilateral ovarian tumors, HSM

IV

Combination CTX

Dead of disease after 11 months

Onciu⁵

1

16/M

Scalp and parietal bone mass, cervical, axillary, and inguinal LA, multiple lytic skeletal lesions

IV

LMB 89, poor response, weekly vinblastine, intrathecal CTX, and palliative XRT

Dead of disease after 24 months

2

10/M

Laryngeal supraglottic mass, cervical and submandibular LA

II

POG8719, XRT to sites of persistent disease and DAHP × 3

Alive without disease after 156 months

Adam⁶

1

35/M

Right cervical and supraclavicular LA

IIA

CHOEP-21 × 5, disease progression, auto BMT, relapse, relapse CTX (adriamycin, bleomycin, vinblastine, decarbazine)

Dead of disease after 14 months

McManus⁷

1

21/M

Pyloric mass

IIE

CHOP × 6

Alive without disease after 2 years

Colomo⁸

1

34/M

Generalized LA

NR

Specifics NR

Dead of disease after 8 months

Ishii⁹ (abstract only)

1

33/M

Right neck LA, at relapse multiple paraaortic LA, and splenomegaly

NR

CR with chemotherapy and local XRT, relapse at 1 year, CR with CHOP biweekly, relapse allo-PBSCT

Dead of disease after 31 months

Rudzski¹⁰

1

48/M

Large upper neck mass

IIIB

CHOP × 3

Dead of disease after 3 months

(abstract only)

2

49/M

Abdominal LA, stomach infiltrate

IVB

On chemotherapy

Currently alive

Gesk¹¹

1

13/M

Cervical LA

II

ALCL99 SR: multiagent CTX

Partial remission

2

12/F

Mediastinal mass, cervical LA

II

Multiagent chemotherapy

Alive without disease at 4 years

3

16/M

Mediastinal mass, cervical LN, chest wall and left pleura involved

IV

Multiagent chemotherapy, BMT

Dead of disease after 1 year

Isimbaldi¹²

1

9/F

Left cervical mass

I

AIEOP LNH 97, CR, ICE at relapse followed by PVDA

Dead of disease after 9 months

Bubala¹³

1

9/M

Ollier disease, generalized LA, mediastinal mass, bony lesions

III

Initial induction with lymphoblastic lymphoma protocol, then LMB 89 protocol, intensification due to disease progression

Dead of disease after 5 months

This study

Four patients, please refer to Table 2