A small case series of patients with peripheral T-cell lymphoma (PTCL) arising in patients with CLL and review of the literature reinforces the knowledge gaps regarding such a rare event.
The three cases from Perth swell the total number of reported cases to just 37.
The review found patients diagnosed with the T-cell lymphoma had a median age of 65 years (range 38-86 years) and the median time from CLL diagnosis to PTCL diagnosis was two years (range 0-14 years).
In 31 cases where the outcomes were known, 81.2% of patients had died of the disease within a year. The estimated median overall survival was 10 months.
In the three WA cases, presentation included non-specific nausea, vomiting weight loss and abdominal pain with left inguinal lymphadenopathy, new-onset bilateral axillary lymphadenopathy with raised cutaneous lesions on the upper limb, and a right anterior chest wall mass.
Lead investigator Associate Professor Chan Cheah told the limbic PET-CT was not particularly useful in the three cases.
“In Richter’s transformation, which is the more common event in CLL, PET-CT is often useful because the SUV max is often >10 and you can use it to target which node to biopsy.”
“We looked at the PET-CT parameters in our small series of patients with T cell lymphoma and it often wasn’t that high.”
Biopsies of lymph nodes and cutaneous lesions revealed a proliferation of anaplastic cells.
Patients were variously treated with mini-CHOP, CHOP then R-CHOP and/or radiotherapy.
Associate Professor Cheah said treatment was directed at the aggressive lymphoma while “the CLL takes a back seat at that point”.
He said the poor survival with PTCL parallels what happens in Richter’s transformation.
“The bottom line is that if someone develops an aggressive lymphoma in the context of someone who already has CLL, the outcomes seem to be poorer than if they just develop the T cell lymphoma without any prior CLL.”
He said the case series was a reminder to not always assume that apparent clinical progression of CLL was necessarily going to be the same histology.
Whether PTCL is the result of decreased immunosurveillance, a malignant transformation in a stem cell, a second cancer related to CLL treatment, or an underlying immune deficiency remains to be determined.
“I don’t think we know the answers to that without doing more detailed genomic analyses. It would be difficult to answer that question as it’s quite a rare problem,” he said.