Angioimmunoblastic T-cell lymphoma induced hemophagocytic lymphohistiocytosis and disseminated intravascular coagulopathy: A case report

Table 2 Clinical features of angioimmunoblastic T-cell lymphoma patients with hemophagocytic lymphohistiocytosis or disseminated intravascular coagulopathy

	Patient 1	Patient 2	Patient 3	Patient 4 (the present case)
Sex/age (yr) at the time of diagnosis	53/male	62/female	72/female	83/male
Laboratory findings in peripheral blood
% atypical lymphocytes in the blood of all lymphocytes	9%	NA	NA	NA
Hypereosinophilia (%)	NA	NA	Yes (17%)	Yes (9%)
Autoantibodies	Antinuclear antibodies and anti-double-stranded DNA antibodies were negative	NA	NA	Anti-TIF-1γ antibodies and anti-Jo-1 antibodies were positive
Hypergammaglobulinaemia	Yes (polyclonal)	NA	Yes (polyclonal)	Yes (monoclonal)
EBV DNA copies (IU/mL)	8.42 × 104	NA, but positive in lymph node biopsy	NA	131
Immunophenotype/immunohistochemical staining	CD2+, CD3+, CD5+, CD7+, CD10+; CD20dim, PAX5dim, and telomerase Bdim (biopsies of the left cervical lymph node)	CD3+, CD4+, CD8,CD30, CD56 andCD20were negative (cervical lymph node biopsy)	CD4+, CD5+, CD10+ (lymph node)	CD3+, CD4+, CD10+, PD1+, CD7 and CD8 were negative- (lymph node)
Clinical manifestation
Generalized lymphadenopathy	Yes	Yes	Yes	Yes
Bone marrow involvement	Hemophagocytosis and abnormal lymphocytes	Hemophagocytosis, but withoutevidence of lymphoma involvement	No lymphoma infiltration and no evidence of hemophagocytosis, bone marrow infiltration two weeks later	Hemophagocytosis, but without evidence of lymphoma involvement
Hepatomegaly	Yes	Yes	Yes	No
Splenomegaly	Yes	Yes	Yes	Yes
Skin rash/purpura	Yes	NA	NA	Yes
Pleural effusion	NA	NA	NA	Yes
Severe complication
HLH	Yes	Yes	No	Yes
DIC	No	No	Yes	Yes
Therapy	Etoposide together with CHOP regimen	CHOP, mesna, ifosfamide, mitoxantrone, etoposide; allogeneic HSCT with RIC	Steroids	Anti-infection and other symptomatic treatment
Outcome	Successfully treated	Successfully treated	Succumbed to DIC and fatal gastrointestinal bleeding	Succumbed to gastrointestinal bleeding and septic shock

NA: Not available; CHOP: Cyclophosphamide, doxorubicin, vincristine, and prednisolone; EBV: Epstein-Barr virus; HLH: Hemophagocytic lymphohistiocytosis; ; DIC: Disseminated intravascular coagulopathy; HSCT: Hematopoietic stem cell transplant; RIC: Reduced-intensity conditioning.