T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

Table 1

Reported cases of hemophagocytic lymphohistiocytosis secondary to T cell histiocyte rich large B cell lymphoma.

Serial #	Age/sex [ref]	Presentation	Site of involvement	Immunophenotype	Treatment	Outcome
1	20/M [7]	Jaundice, malaise, abdominal pain, fever	LN, liver	CD3+ CD5+ CD7+ CD45+ T cell infiltrate; Scattered large CD20+ PAX5+ CD15− CD30− Alk-1− B-cells	R-CHOP × 6; IT-MTX	Alive
2	52/M [8]	Fever, DOE, weight loss	LN	Scattered large CD20+ cells; CD3+ T cells; CD30− CD15− EBV−	R-CHOP × 8, IT-MTX, cytarabine, MP	Recurrence at 10 m, salvage therapy
3	30/M [9]	Fever, jaundice, weight loss, ARF	LN, lung	Large CD20+ CD15− CD30− B cells; CD3+ CD5+ CD7+ CD8+ TIA-1+ T cells; CD68+ histiocytes	DA-R-EPOCH	Alive
4	30/F [10]	Pruritus, night sweats, fever, weight loss	LN, liver	CD79a+ Mib-1+ large cells	MOPP-ABV then high dose MTX, vincristine and etoposide, then AHSCT	Alive at 24 m
5	34/M [11]	Fever, abdominal pain, jaundice	BM	ND	ND	DOD
6	43/M [current case]	Fever	LN	Large atypical cell CD20+, PAX5+, BCL-6+, MUM1+, EMA (weak), Kappa (weak); CD3+, CD2+, CD5+, CD7+, CD43+ T cells	R-CHOP × 6	Alive

AHSCT: autologous hematopoietic stem cell transplant; BM: bone marrow; DA-R-EPOCH: dose adjusted Rituximab, Etoposide, Prednisone, Vincristine (Oncovin), Cyclophosphamide, and Doxorubicin; DOE: dyspnea on exertion; DOD: died of disease; IT-MTX: intrathecal methotrexate; LN: lymph node; M: months; MOPP-ABV: mechlorethamine, vincristine, procarbazine, prednisone/doxorubicin bleomycin, and vincristine; MP: methylprednisolone; ND: not described; R-CHOP: Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone.