Composite angioimmunoblastic T-cell and diffuse large B-cell lymphoma

Abstract

A58-year-old female smoker with a history of hypertension presented to the emergency department with a recurrent episode of worsening shortness of breath over five days. Her exam was significant for sinus tachycardia, cervical lymphadenopathy, and a firm left supraclavicular node. Laboratory values demonstrated a normal white cell count and differential, normocytic anemia, thrombocytopenia, and a lactate dehydrogenase of 803 IU/L. She was HIV negative. Computerized tomography (CT) scan of the chest/abdomen/pelvis demonstrated extensive bilateral cervical, supraclavicular and axillary adenopathy, mediastinal and hilar lymphadenopathy with infiltrate and consolidation of the right lower and posterior upper lobe and encasement of the right pulmonary artery, as well as splenomegaly with paraaortic and retroperitoneal adenopathy. An echocardiogram revealed a normal left ventricle and a severe pericardial effusion. She underwent a pericardial window and an excisional supraclavicular lymph node biopsy, which revealed T-cells with features of angioimmunoblastic T-cell lymphoma (AITL) and large atypical B-cells consistent with diffuse large Bcell lymphoma (DLBCL) (Figure 1A). Destruction of the lymph node architecture was noted, with many medium-sized atypical lymphocytes with irregular nuclei and moderate amounts of pale cytoplasm. In addition, scattered and focally increased large atypical lymphocytes with prominent nucleoli were seen. Blood vessels were increased. Immunostains demonstrated atypical T-cells expressing CD3 (Figure 1B), CD4 (Figure 1C), CD5, CD2, CD7, CD4, CD10, PD-1 (Figure 1D), as well as clustered large B-cells positive for expression of CD20, Bcl-6 and BOB-1 (Figure 1E). Polymerase chain reaction (PCR) testing revealed a clonal TCR gamma gene rearrangement, as well as a clonal IgH gene rearrangement. Epstein-Barr virus-encoded RNA (EBER) was negative by in situ hybridization (ISH). Bone marrow biopsy and CSF analysis were negative for lymphoid or plasma cell infiltrates. The patient was initiated on R-CHOEP (rituximab, cyclophosphamide, doxorubicin, vincristine, etoposide, prednisone) and completed three cycles of chemotherapy. Her CT scan after three cycles was consistent with a partial response, with significantly diminished bilateral axillary lymphadenopathy, improved mediastinal, perihilar and right lower lobe infiltrates, decreased splenomegaly, and upper abdominal and retroperitoneal adenopathy. The patient eventually had progression of her disease during the course of subsequent R-CHOEP cycles. She then progressed through two cycles of gemcitabine-carboplatin and one course of romidepsin. Her performance status deteriorated, not enabling any further treatment and the patient finally expired, approximately 24 months after initial diagnosis. This case illustrates a rare hematologic malignancy presenting with a dual primary lymphoma population. The treatment of composite lymphoma with simultaneous Band T-cell components is challenging and the less favorable prognosis component determines