A 68-Year-Old Man With Mantle Cell Lymphoma - Episode 1

Case Assessment: 68-Year-Old Patient With MCL

Jonathon B. Cohen, MD, MS

Jonathon Cohen, MD, MS: Today we’ll be discussing the case of a 68-year-old man who presented with generalized lymphadenopathy, fatigue, as well as about an 8-pound weight loss. He was otherwise fairly well. His past medical history revealed hypertension that was under control. On his initial exam, he did have some abdominal distension, some splenomegaly, and also some palpable adenopathy, especially in the cervical and axillary regions. On his lab work-up, he was found to have a normal absolute neutrophil count, but his LDL [low-density lipoprotein] was elevated—at 345 mg/dL—and his hemoglobin was low—at 9.6 g/dL. His white blood cell count was otherwise normal.

He underwent an excisional lymph node biopsy of an axillary lymph node. FISH [fluorescence in situ hybridization] confirmed that he had translocation 11;14. In addition, the immunohistochemical stains confirmed that he was cyclin D1+, as well as CD5+ and CD20+. As a result, the diagnosis of mantle cell lymphoma was confirmed.

He underwent a bone marrow biopsy, which was also positive for involvement with lymphoma. His PET [positron emission tomography] and CT [computed tomography] scans demonstrated widespread lymphadenopathy in a number of nodal regions. In addition to the cervical and axillary regions, there was lymphadenopathy in the abdomen. In addition, he did have splenomegaly consistent with his physical exam. Ultimately, he was diagnosed by the Ann Arbor staging system as having stage IV disease, and he had a high-risk MIPI [Mantle Cell Lymphoma International Prognostic Index] score. His performance status was 1. Otherwise, he looked well at the time of his visit, although you could certainly tell that he was impacted by his developing disease.

Given that he did have symptoms, and based on the extent of his disease, he was started on chemotherapy—bendamustine and rituximab. He completed 6 cycles of therapy. At the conclusion of treatment, he achieved a metabolic PR [pathologic response] and clearly had an excellent response to therapy. At that time was initiated on maintenance rituximab, which he received every 2 months.

He did well for several months, but about 9 months later he did present with recurrent lymphadenopathy. A biopsy confirmed relapsed mantle cell lymphoma. At that time, he was started on ibrutinib monotherapy at 560 mg per day. After the first 3 months of treatment, he completed follow-up PET and CT scans, which demonstrated no evidence of residual disease. At this point, he remains on ibrutinib.

Transcript edited for clarity.


Case: A 68-Year-Old Male with Mantle Cell Lymphoma

Initial presentation

  • A 68-year-old man presented with generalized lymphadenopathy, fatigue and an unintentional 8-lb weight loss
  • PMH: HTN, medically controlled
  • PE: abdominal distention, splenomegaly

Clinical workup

  • LDH 345 U/I, ANC 3500/mm3, beta-2-microglobulin 4.4 µg/L, leukocytes, 5.98 X 109/L, hemoglobin 9.6 gm/dL
  • FISH: t (11;14)
  • Immunocytochemistry: cyclin D1+, CD5+, CD20++
  • BMB positive for lymphoid cells with cyclin D1
  • PET/CT scan showed widespread lymphadenopathy in numerous nodal regions including left mesenteric (3.4 cm), bilateral axillary (3.9 cm, 5.3 cm), and cervical (5.1 cm)
  • Ann Arbor stage IV; MIPI score 6.3; ECOG PS 1

Treatment

  • Patient was started on bendamustine + rituximab
    • Achieved PR
    • Continued on maintenance rituximab q8W
  • At 9 months the patient had clinical disease relapse
    • He was started on ibrutinib 560 mg PO qDay
    • Imaging at 12-week follow-up showed no evidence of disease