Myeloma or Lymphoma: That is the Question? Myeloma Relapsing as an Angio-Immunoblastic T Cell Lymphoma

Case Report

Ann Hematol Oncol. 2019; 6(8): 1264.

Myeloma or Lymphoma: That is the Question? Myeloma Relapsing as an Angio-Immunoblastic T Cell Lymphoma

Chehade R1*, Mangel J1,2, Louzada M1,2, Shepherd J3 and Deotare U1,2

1Department of Medicine, Schulich School of Medicine and Dentistry, University of Western Ontario, Canada

2Division of Hematology, Department of Medicine, Schulich School of Medicine and Dentistry, University of Western Ontario, Canada

3Department of Pathology and Laboratory Medicine, Schulich School of Medicine and Dentistry, University of Western Ontario, Canada

*Corresponding author: Chehade R, Department of Medicine, Schulich School of Medicine and Dentistry, University of Western Ontario, Canada, 1151 Richmond St, London, ON N6A 5A5, Canada

Received: June 05, 2019; Accepted: July 17, 2019; Published: July 24, 2019


Angio Immunoblastic T Cell Lymphoma (AITL) is a clinically aggressive type of Peripheral T cell Lymphoma derived from T follicular helper cells. A rare malignancy with poor overall survival, AITL is often accompanied by polyclonal or monoclonal proliferation of B lymphocytes, however, AITL is rarely associated with plasma cell proliferation. Here we describe a new diagnosis of AITL developing in a patient previously treated for multiple myeloma. This highlights a role for circulating plasma cells and interaction with T follicular helper cells in AITL tumorigenesis. To the best of our knowledge, this is the first report of newly diagnosed AITL in a patient with a previous history of multiple myeloma.

Keywords: Angio immunoblastic T cell lymphoma; Multiple myeloma


AITL: Angio Immunoblastic T Cell Lymphoma; MM: Multiple Myeloma; TFH: T Follicular Helper Cell; VMP: Lite Bortezomib, Melphalan, Dexamethasone; CT: Computed Tomography; FISH: Fluorescence In Situ Hybridization; P53: Protein 53; LDH: Lactate Dehydrogenase; CD: Cluster of Differentiation; BCL2: B-Cell Lymphoma 2; TET2: Tet Methylcytosine Dioxygenase 2; DNMT3A: DNA Methyltransferase 3A; IDH2; Isocitrate Dehydrogenase 2; PLCG1: Phospholipase C Gamma 1; RHOA: Ras Homolog Family Member A; NOTCH1; Notch Homolog 1, Translocation-Associated; M: Male; F: Female; LN: Lymph Node; PB: Peripheral Blood; BM: Bone Marrow; H&E: Hematoxylin and Eosin; EBV: Epstein Bar Virus; EBER: EBV Encoded RNA

Case Report

A 76-year-old Caucasian man presented to the emergency department with confusion, on a background of several weeks of generalized fatigue with fevers and night sweats. He was noted to have significant cervical and supraclavicular lymphadenopathy, as well as hepatosplenomegaly. Serum LDH level was elevated at 248 U/L. Computed Tomography (CT) scans of chest, abdomen and pelvis revealed extensive axillary, thoracic, retroperitoneal and pelvic lymphadenopathy in addition to previously noted bony lytic lesions.

The patient had a past medical history of IgA lambda multiple myeloma with high-risk cytogenetics, including 17p deletion. He initially presented with mild anemia identified on routine blood work by his family doctor in October 2010. The original investigation involved a negative colonoscopy, and in later investigations, he had serum protein electrophoresis which showed significant IgA monoclonal peak. The patient then underwent a bone marrow aspiration and biopsy, which showed 40 to 50% plasma cell infiltration. The bone marrow flow cytometry identified a 6% plasma cell population that was lambda light chain restricted. FISH identified a P53 mutation, detected in 18.5% of the cells. In December 2010, the patient had noticed quite significant height loss associated with severe thoracic back pain. Skeletal survey identified multiple thoracic and lumbar compression fractures, as well as multiple areas of bony lytic lesions in the pubic rami, innominate bones, proximal femur, and proximal right humerus, as well as diffuse osteopenia.

He received nine cycles of bortezomib, melphalan and dexamethasone (VMP Lite Chemotherapy) as initial therapy, requiring dose reductions because of neuropathy in the toes, yet attaining near complete response. He showed evidence of biochemical progression in October 2013 as his IgA had increased up to 12.2 with a monoclonal peak measuring at 3.58 g/L and worsening kidney function. He received fifty-two cycles of bortezomib and dexamethasone until November 2016, as a part of a clinical trial and achieved complete hematological response. He was followed up regularly and was doing well until September 2017 when he was found to have elevated lambda light chain levels and creatinine; later in October, he presented to the hospital with confusion.

After admission to hospital, he underwent an open excisional biopsy of a cervical lymph node as well as a bone marrow biopsy. The lymph node (Figures 1D and 1E) was diffusely effaced by a mass-like proliferation of polymorphous lymphocytes, eosinophils, plasma cells, Hodgkin Reed Sternberg-like cells and occasional immunoblasts with proliferation of high endothelial cells toward the periphery. Immunohistochemistry showed the lymphoid infiltrate to be positive for CD3, CD5, CD7, with dominance of CD4 over CD8 expression, and CD10 (Figure 2) and BCL2 negative. There were scattered EBER positive CD20 positive B lymphocytes (Figure 2). The bone marrow biopsy (Figures 1A, 1B and 1C) showed increased cellularity of 60%, with two abnormal population groups noted: (a) a nodular interstitial and paratrabecular lymphoid infiltrate, composed predominantly of small lymphocytes, eosinophils and intermediate sized lymphocytes with atypical angulated nuclei and moderate amount of pale cytoplasm, as well as scattered large atypical lymphoid cells containing irregular nuclei with vesicular chromatin and prominent nucleoli; and (b) few paratrabecular plasma cell aggregates with atypical features, including large, binucleated, dutcher bodies. The plasma cells were less than 10%, CD56 positive and lambda light chain restricted. The diagnosis was peripheral T cell lymphoma, consistent with AITL. There were minor residual clonal plasma cells in the bone marrow.