Mantle cell lymphoma: a great masquerader

A 78-year-old male presented with a 4-week history of fatigue, cervical and inguinal lymphadenopathy, weight loss, and abdominal distention. The laboratory evaluation revealed anemia (hemoglobin 11.7 g/dL), lymphocytosis (total white blood cell count 23.3 9 10/L; 77 % lymphocytes) and acute renal failure (creatinine 2 mg/dL). A CT scan of the abdomen and pelvis showed massive mesenteric and retroperitoneal lymphadenopathy; splenomegaly and bilateral hydronephrosis (Fig. 1a). Peripheral smear showed small lymphocytes with scant cytoplasm and irregular nuclei (Fig. 1b Wright Giemsa, 8009 magnification). A cervical lymph node biopsy and bone marrow biopsy demonstrated nodular and interstitial lymphoid infiltrates expressing CD20, CD5, cyclin D1, BCL-2, and kappa light chain, consistent with stage IVB, high-risk MCL international prognostic index diffuse-variant mantle cell lymphoma (MCL). The B cells were negative for CD3, CD10, CD23, and BCL-6. After bilateral internal ureteral stent placement, he received a total of five cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP) chemotherapy. Recurrent neutropenic fevers required premature discontinuation of chemotherapy. Postchemotherapy assessment indicated marked improvement in the retroperitoneal and mesenteric adenopathy, and minimal residual bone marrow involvement (5 % of total cellularity as compared to 70 % at diagnosis). Nine months later, the patient complained of right eye erythema and foreign body sensation. Examination revealed a red, palpable conjunctival mass (Fig. 1c). The biopsy findings again demonstrated CD20, CD5, CD10 and cyclin D1 cells due to MCL-related infiltration (Fig. 1d, Hematoxylin and Eosin, 2009 magnification). Restaging CT scan revealed soft-tissue thickening surrounding both kidneys, suggestive of lymphomatous dissemination. A course of six cycles of bendamustine and rituximab resulted in a partial response. Within a month, however, he presented with cognitive decline, gait instability, and a new rash (Fig. 1e) with multiple subcutaneous nodules and right inguinal lymphadenopathy. Progression of his disease was confirmed with a punch biopsy of a cutaneous lesion. A PET scan also suggested posterior nasopharyngeal involvement. His cerebrospinal fluid analysis was remarkable for significant pleocytosis with an abnormal CD5 (dim), CD10, kappa-restricted, large lymphoid cell population with moderately abundant cytoplasm and variably prominent nucleoli (Fig. 1f, Wright Giemsa, 8009 magnification). This immunophenotype was different from that noted at the time of initial presentation. Although a separate lymphoproliferative disorder could not be entirely excluded, alterations in immunophenotype, including loss of CD5 and gain of CD10 expression, have been previously reported in transformation to the blastoid variant of MCL [1]. Given his poor performance status with an increasingly aggressive disease course, the patient preferred to transition to home hospice, and shortly thereafter succumbed to his refractory disease. MCL is an uncommon, difficultto-treat, non-Hodgkin lymphoma with a strikingly male predominance, heterogeneous outcome and a variable presentation ranging from an asymptomatic disease to myriad clinical manifestations resulting from extranodal dissemination to sites including spleen, Waldeyer’s ring, stomach, intestines, skin, central J. Paludo P. Kapoor (&) J. L. Oliveira J. A. Garrity G. S. Nowakowski Mayo Clinic, Rochester, MN, USA e-mail: kapoor.prashant@mayo.edu