Considerations in a Diagnosis of Follicular Lymphoma

Ajay K. Gopal, MD:This is a case of a 62-year-old gentleman who presented to the clinic with left axillary adenopathy. His past medical history is noted here with a history of a DVT (deep vein thrombosis) and, somewhat unusual, a history of CMV (cytomegalovirus) infection. It’s not clear what that is, but that’s a pretty unusual situation. Laboratory studies at baseline were normal. CBC (complete blood count) and LDH (lactate dehydrogenase) were normal. We’re not given the beta-2 microglobulin information. He underwent a left axillary lymph node biopsy, which showed a CD10-positive follicular lymphoma, grade 2. And further staging studies, including a PET/CT scan, showed multifocal disease, multiple sites of adenopathy, and the bone marrow biopsy showed about 30% involvement.

As part of the workup, you calculate the FLIPI score. The FLIPI score for this gentleman apparently is a 2, which would mean that we would expect about a 50% 10-year overall survival, at least based on the historical original FLIPI data. He is treated with bendamustine/rituximab and receives 6 cycles and achieves an unconfirmed complete remission.

The prognosis of this patient can be somewhat predicted by the FLIPI score. With a FLIPI score of 2, we would expect about 50% 10-year survival, at least based on the historical FLIPI data. We expect it’s probably better than that because that was based prior to many of the modern regimens pre-rituximab era. FLIPI score of 3 would be about a 30% to 35% 10-year survival. The first question we have when we have a patient with an indolent lymphoma is whether or not they need treatment. That’s the first treatment we ask ourselves and discuss with the patient. And we usually use either the NCCN or GELF criteria. It has to do with, does the patient have symptoms, are there cytopenias related, does the patient have high tumor burden? Officially by GELF, it has to be 3 sites over 3 cm or 1 over 7 cm. It’s not completely clear from this case history, but presumably one might be able to attribute this clot to the lymphoma. And it sounds like this patient may have had symptoms that justified treatment, so this was the reason for likely choosing bendamustine/rituximab.

There are no major differences between the NCCN and GELF guidelines. One thing that we do keep in mind, particularly in the NCCN guidelines, as pointed out, that patients who are eligible for a clinical trial should be considered for a clinical trial.

Transcript edited for clarity.

January 2016

• A 62-year-old male presented with left axillary lymphadenopathy
• PMH: DVT managed on warfarin, CMV infection
  • Laboratory findings: CBC count and LDH WNL
  • Excisional biopsy, IHC staining CD10+, grade 2 follicular lymphoma
  • PET/CT, multicompartmental adenopathy and splenomegaly consistent with stage IV disease
  • Bone marrow biopsy, 30% involvement
  • FLIPI 2 (intermediate-risk)
• The patient was started on bendamustine + rituximab (6 cycles)
• She achieved an unconfirmed complete response

June 2017

• 18 months later, he developed recurrent cervical adenopathy with weight loss and fatigue
• Imaging revealed adenopathy in 2 cervical lymph nodes (4.6 cm and 2.4 cm), a mediastinal node (2 cm) and left inguinal node (3.1 cm) with splenomegaly.
• The patient was treated with R-CHOP
• After 2 cycles he developed anemia and grade 2 fatigue
• He achieved a partial response

November 2017

• Five months later, the patient reports feeling tired and abdominal fullness
• Physical exam remarkable for palpable splenomegaly
• Laboratory evaluation showed marked anemia, thrombocytopenia
• PET imaging showed enlargement of pelvic and retroperitoneal nodes and development of several new mediastinal lesions
• Repeat biopsy showed grade 3 follicular lymphoma, 90% bone marrow involvement
• The patient was started on idelalisib therapy
• After 2 months on therapy, he developed grade 3 colitis which was managed
• After four months on therapy, the patient has stable disease