Case Reports in Oncological Medicine / 2017 / Article / Tab 1

Case Report

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]PresentationSite of involvementImmunophenotypeTreatmentOutcome

120/M [7]Jaundice, malaise, abdominal pain, feverLN, liverCD3+ CD5+ CD7+ CD45+ T cell infiltrate
Scattered large CD20+ PAX5+ CD15− CD30− Alk-1− B-cells
R-CHOP × 6
IT-MTX
Alive

252/M [8]Fever, DOE, weight lossLNScattered large CD20+ cells
CD3+ T cells
CD30− CD15− EBV−
R-CHOP × 8, IT-MTX, cytarabine, MPRecurrence at 10 m, salvage therapy

330/M [9]Fever, jaundice, weight loss, ARFLN, lungLarge CD20+ CD15− CD30− B cells
CD3+ CD5+ CD7+ CD8+ TIA-1+ T cells
CD68+ histiocytes
DA-R-EPOCHAlive

430/F [10]Pruritus, night sweats, fever, weight lossLN, liverCD79a+ Mib-1+ large cellsMOPP-ABV then high dose MTX, vincristine and etoposide, then AHSCTAlive at 24 m

534/M [11]Fever, abdominal pain, jaundiceBMNDNDDOD

643/M [current case]FeverLNLarge atypical cell CD20+, PAX5+, BCL-6+, MUM1+, EMA (weak), Kappa (weak)
CD3+, CD2+, CD5+, CD7+, CD43+ T cells
R-CHOP × 6Alive

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.

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