Table 2.

Clinical, biological, therapeutic and evolution features of previously reported cases of TCRBCL associated HLH

Case	Sex	Age	Clinical features	Laboratory findings	Pathology	EBV	Treatment	Outcome
Mitterrer et al. [9]	Female	30	B symptoms, splenomegaly	Moderate pancytopenia, high LDH	Spleen: hemophagocytosis Hepatic nodules: TCRBCL	Reactivated EBV infection serological profile	MOPP-ABV then high dose methotrexate, vincristine and etoposide followed by AHSCT	Sustained CR for 2 years
Devitt et al. [12]	Male	30	Fever, jaundice, B symptoms, splenomegaly, repiratory failure	Hyperferritinemia Pancytopenia Hyperbilirubinemia Elevated liver enzymes High LDH	Bone marrow: Hemophagocytosis and lymphomatous infiltration	Negative (in situ hybridization)	HLH 2004, R-EPOCH	CR followed by AHSCT
Aljitawi et Boone [13]	Male	34	Relapse of previously treated TCRBCL Fever, jaundice, hepatosplenomegaly, ascites	Hyperferritinemia Pancytopenia Hyperbilirubinemia High soluble IL2-R	Bone marrow: Hemphagocytosis, relapsed TCRBCL	NA	Salvage therapy (NA)	Relapse after months and death
Jiang et al. [14]	Male	20	Jaundice Fatigue Abdominal disconfort Fever	Acute hepatitis Pancytopenia Hyperferritinemia High soluble IL2-R	Bone marrow and lymph node: TCRBCL	NA	R-CHOP	CR
Our case	Male	52	B symptoms, splenomegaly Lymph nodes	Pancytopenia High LDH Hyperferritinemia	Bone marrow: hemophagocytosis Lymph node: TCRBCL	Negative (biopsy and peripheral blood PCR)	R-CHOP	Relapse after 10 months

LDH lacticodeshydrogenase, R-EPOCH Rituximab, etoposide, prednisone, vincristine, cyclophosphamide and doxorubicine, CR complete remission, AHSCT autologous hematopoietic stem cell transplantation, TCRBCL T-cell rich B-cell lymphoma, MOPP-ABV mechlorethamine, vincristine, procarbazine, prednisone/doxorubicin, bleomycin, vincristine, PCR polymerase chain reaction