Diffuse Large B-Cell Lymphoma Trans-differentiating into CD30 Positive Anaplastic Large Cell-like Lymphoma

Case Report

Ann Hematol Oncol. 2017; 4(7): 1162.

Diffuse Large B-Cell Lymphoma Trans-differentiating into CD30 Positive Anaplastic Large Cell-like Lymphoma

Qiu JJ, Bhattacharyya S, Grant M and Bains A*

Department of Pathology, Temple University Hospital, USA

*Corresponding author: Bains A, Department of Pathology, Temple University Hospital, Philadelphia, PA 19140, USA

Received: May 11, 2017; Accepted: June 12, 2017; Published: July 06, 2017


Post-Transplant Lymphoproliferative Disorders (PTLD) is mostly of B-cell origin. “Composite” PTLD with diffuse large B-cell lymphoma (DLBCL) and T/ anaplastic large cell lymphoma component are extremely rare. We report a case of PTLD after heart transplant with two distinct components. The patient was initially diagnosed with monomorphic PTLD/DLBCL, EBV negative involving the intestinal wall, occurring nine years after heart transplantation. Sixteen years post-transplant, the lymphoma recurred involving the intestine and showed two distinct components: DLBCL and anaplastic large cell lymphoma with strong homogeneous expression of CD45 and CD30 while lacking B- and T-cell lineage markers mimicking Anaplastic Large Cell Lymphoma (ALCL), ALK negative with a null-cell phenotype and TIA-1 expression. At this time, diffuse lymphadenopathy was also present and a jugular lymph node biopsy prior to the intestinal resection, purely demonstrated only the anaplastic large lymphoma cells with a null-cell phenotype leading to the diagnosis of ALCL, ALK negative. However, molecular studies performed on the anaplastic large cell component identified strongly positive clonal IgH gene rearrangement, identical to the prior lymphoma. Extensive sampling from the intestinal resection specimen identified a transitioning phase between two morphologically and immunohistochemically separate components. This is a unique case of PTLD which demonstrates an immunophenotypic lineage plasticity of the neoplastic cells over an extended post-transplant period.

Keywords: Post-transplant lymphoproliferative disorder; Diffuse large B-cell lymphoma; Anaplastic large cell lymphoma

Case Presentation

Clinical features

A 66-year-old male underwent heart transplant secondary to dilated cardiomyopathy and received tacrolimus for immunosuppressive therapy. Nine years post-transplant, he developed monomorphic PTLD/DLBCL (stage 1E with no evidence of bone marrow involvement) causing small intestinal obstruction which was treated with segmental resection and reduction of immunosuppressive medication. Subsequently the patient received complete remission with no evidence of lymphomatous involvement on PET/CT scan which lasted for two years until he presented with abdominal pain. The lymphoma recurred at the anastomotic site two years from the initial diagnosis, for which the patient underwent a hemicolectomy and four cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone with rituximab (CHOP/R-CHOP). Sixteen years post-transplant, lymphoma recurred again, this time with extensive lymphadenopathy in addition to small intestinal involvement (Figure 1A and 1B). The complete blood count was unremarkable except for a low hemoglobin level (5,700 K/μL white blood count with normal differential, -10.8 gm/dL hemoglobin, and 247 K/μL platelets). Given the diffuse lymphadenopathy, initially a core biopsy of the most accessible jugular lymph node was performed which was diagnosed as ALCL, ALK negative with a null-cell phenotype. Subsequent to the diagnosis, the patient underwent partial small intestine resection to prevent potential perforation during treatment. A bone marrow biopsy was negative for lymphoma involvement. He then received salvage chemotherapy with six cycles of modified R-CHOP with addition of etoposide and achieved a complete response. It was followed by autologous stem cell transplant. At the time of this report, the patient has shown no signs of recurrent lymphoma.