Most common histological types, PTCL-NOS Less Common types, AITL, SCPTCL, NKTCL; prognosis is poor in HLH group. High microglobulin, CA-125 are important biomarkers in T-NHL associated HLH.
Common histological subtypes with bone marrow involvement: AITCL, PTCL-NOS, ALCL, EN-NKTCL, EATCL
High ferritin and liver dysfunction: common
Less common
<0.05
Anemia: 51%
29%
0.001
1 year overall survival: 5%
49%
Ménard et al. [5] (, Hodgkin lymphoma associated HLH)
Males/females, 26/6, M : F = 3.3 : 1 Age = 43 years (19–84 years), 26, HIV nonreactive, 8, HIV +ve. 100%, stage IVB
9/20 (45%), lymphocyte depleted, 8/20 (40%), mixed cellularity, 3/20 (15%), nodular sclerosis subtype 32/34 (92%) cases +ve for EBER (ISH), LMP-1 (IHC), ZEBRA −ve in all
HL-HLH strongly associated with EBV. Prognosis not studied. High levels of LMP-1 expression may induce the large amount of Th1 cytokines production by Reed Sternberg cells leading to HLH.
B-NHL-HLH
NKTCL-HLH
Remark
Takahashi et al. [14], Japan, (, lymphoma associated HLH)
Number () Median age at diagnosis Cytopenia(s), coagulopathy, liver dysfunction Intravascular lymphomatosis Hepatosplenomegaly Lymphadenopathy EBV positivity (ISH) Median survival (days)
68 63.5 years less common 10/20 + Nil 3/24 242
74 49 year more common — + + 19/23 69
Prognosis is better in B-NHL associated HLH than NKTCL associated HLH.
HLH: hemophagocytic lymphohistiocytosis, PTCL-NOS: peripheral T cell lymphoma, not otherwise specified, AITL: angioimmunoblastic T cell lymphoma, SCPTCL: subcutaneous panniculitis-like T cell lymphoma, EN-NKTCL: extranodal natural killer/T cell lymphoma, T-NHL: T cell non-Hodgkin lymphoma, EATCL: enteropathy associated T cell lymphoma, ALCL: anaplastic large cell lymphoma, ALK: anaplastic lymphoma kinase, DLBCL: diffuse large B cell non-Hodgkin lymphoma, not otherwise specified, B-NHL-HLH: B cell non-Hodgkin lymphoma associated HLH, NKTCL-HLH: natural killer cell T cell lymphoma associated HLH, IPI: international prognostic index, LDH: lactate dehydrogenase, DIC: disseminated intravascular coagulation, EBV: Epstein Barr virus, EBER: Epstein Barr encoding region, ISH: in situ hybridization, LMP: latent membrane protein, and IHC: immunohistochemistry.