Current Criteria Used to Help Diagnose Follicular Lymphoma
Important considerations when diagnosing follicular lymphoma, including the rationale for assessing patients’ FLIPI score and looking for any signs of histologic transformation.
John P. Leonard, MD: This is a 71-year-old patient with newly diagnosed follicular lymphoma who has indications for therapy. She has significant disease. She has splenomegaly. She has cytopenias. We want to be clear that she has follicular lymphoma and her diagnosis appears to be accurately made.
We want to think about if she has evidence of transformation, because that would change our treatment approach. She had a PET [positron emission tomography] scan that showed no evidence of transformation, at least to the extent that her SUV [standard uptake value], in the range of 8 to 10, is more consistent with follicular lymphoma. However, she does have a slight elevation in LDH [lactate dehydrogenase], so we do have to think about transformation, and that’s something that one needs to think about in any patient, particularly one starting on therapy.
Overall, once we’ve established the diagnosis, staged the patient, and sorted out her extent of disease, a crucial issue is, does she need treatment at all? We’ll get into these details in a minute, but it appears that she does. She’s anemic. She has symptoms. So this is a patient who would need treatment.
We have a couple of different possibilities to think about in this case.
When we look at a newly diagnosed patient with follicular lymphoma, there are some prognostic issues that are useful. In particular, the IPI [International Prognostic Index] has been used over many years. More recently, the FLIPI [Follicular Lymphoma International Prognostic Index] is helpful. The FLIPI includes nodes. No-LASH is a way to remember it: number of nodal regions, LDH elevation, age over 60, stage, and hemoglobin.
There are specific cutoff values. This patient has a number of adverse FLIPI factors, including sites of disease, age over 60, elevated LDH, anemia, and stage of disease. She would fall into an adverse-risk group with respect to her FLIPI score.
That being said, some would argue that patients with an adverse or high-risk FLIPI score should be treated more aggressively. This is a patient who clearly does need treatment. One might argue that a more aggressive treatment might make sense, although to be clear, the use of the FLIPI score is quite limited in choosing therapy. We don’t know that we necessarily should treat higher-risk patients differently from lower-risk patients. One would argue that this is a patient who has a higher-risk score, so she should probably be treated with chemoimmunotherapy, vs rituximab alone as an alternative.
Transcript edited for clarity.
Case: A 72-Year-Old Woman With Follicular Lymphoma
Initial Presentation
- A 71-year-old woman complains of a 5-month history of occasional fevers, decreased appetite, fatigue, and an unintentional 7-lbs. weight loss
- PMH: unremarkable
- PE: palpable left axillary lymph nodes ~ 4 cm and bilateral cervical lymph nodes ~ 3 cm; spleen palpable 4.5 cm below costal margin
Clinical Work-Up
- Labs: ANC 1.5 x 109/L, WBC 11.8 x 109/L, 42% lymphocytes, Hb 9.6 g/dL, plt 100 x 109/L, LDH 325 U/L, B2M 3.7 µg/mL; HBV negative
- Excisional biopsy of the axillary lymph node on IHC showed CD 20+, CD 3+, CD5+, CD 10+, BCL2+; follicular lymphoma grade 2
- Bone marrow biopsy showed paratrabecular lymphoid aggregates, 45% involvement
- Molecular genetics: t(14;18) (q32;q21)
- PET/CT showed enlargement of left axillary, mediastinal, and bilateral para-aortic lymphadenopathy (4.2 cm, 5.3 cm, 3.6 cm, and 3.5 cm respectively)
- Ann Arbor Stage IV; ECOG 0
Treatment
- She was treated with R-CHOP for 6 cycles; continued rituximab maintenance 375 mg/m3; achieved partial response
- 6 months later, she complained of increasing frequency of fevers and chills
- Repeat PET/CT revealed progression of disease
- She was started on bendamustine + obinutuzumab for 6 cycles and continued maintenance on obinutuzumab
- Repeat lymph node biopsy grade 2 follicular lymphoma
- 12 months later, she complained of increased weight loss and fatigue
- She was started on idelalisib 150 mg PO BID and achieved partial response
- She experienced grade 2 diarrhea, which was successfully managed
