Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma: An Adjunctive Diagnostic Role for Monitoring EBV Viremia?

Table 1

Clinical and laboratory profiles of ALPS patients with EBV lymphoma and/or EBV viremia.

Patient (reference; pedigree if available)

Country

Sex

Age of ALPS manifestations

Adeno-pathy

Spleno-megaly

Anemia

Thrombo-cytopenia

% DNTC*

EBV^+ Lymphoma

Lymphoma onset (years after ALPS onset)

EBV viremia

FAS mutation

Outcome

1^†

Canada

M

4 years

+

+

+

−

15

Composite lymphoma (follicular lymphoma, diffuse large B-cell lymphoma, and classic Hodgkin’s lymphoma)

30

+ (progressively increasing)

784A→T

Treated with chemotherapy. Alive.

2 [3]; (26-II-4)

USA

M

2 years

+

+

+

+

0.7

Omental Burkitt’s lymphoma

48

NR

973A→T

Treated with chemotherapy. Survived.

3 [3]; (26-IV-5)

USA

M

10 months

+

+

+

NR

2.1

Classic Hodgkin’s lymphoma (mixed cellularity)

7

NR

973A→T

Treated with chemotherapy; survived. Diagnosed with histiocytic sarcoma (at age 13); died from pulmonary hemorrhage after bone marrow transplant

4 [3]; (45-III-2)

USA

M

5 years

+

+

+

+

11.7

Hodgkin’s lymphoma

6

NR

779del11

Treated with chemotherapy. Outcome NR.

5 [3]; (G3-III-4)

Germany

F

NR

+

+

−

+

75

T-cell lymphoma

2

NR

1009A→G

Treated with chemotherapy. Survived

6 [8]

Japan

M

3 months

+

+

−

+

10.4

NR

NR

+

IVS8 + 5G→T

Alive at 15 years old.

7 [8]

Japan

M

1

+

+

+

−

20.3

NR

NR

+

1020C→T

Alive at 14 years old.

8 [8]

Japan

M

2

+

+

−

+

12.5

NR

NR

+

1020C→T

NR

“+”: presence. “−”: absence. NR: not reported. *DNTC: double negative T cells = TCR α/β CD4- CD8-T cells.
^†Current case; [3] Straus et al. Blood, 2001. [8] Nomura et al. Int J Hematol, 2011.