Table 2 Clinical, biological, therapeutic and evolution features of previously reported cases of TCRBCL associated HLH
From: Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma
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 |