Case 134: Summary

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We discussed a 61-year old man who presented to haematology with a 6-month history of weight loss, night sweats and a left axillary lump. He was diagnosed with peripheral T-cell lymphoma, NOS. CT imaging showed stage 3 disease with splenic involvement. He was refractory to first line CHOP therapy and was escalated to IVE/MTX. His treatment was complicated by ifosfamide-induced encephalopathy but he made a good recovery and successfully underwent auto-HSCT.

Peripheral T-cell lymphoma, not otherwise specified (NOS)

PTCL-NOS is a rare (10-15% of all non-Hodgkin lymphomas) lymphoproliferative disorder originating from peripheral T-cells or mature natural killer (NK) cells. It is an aggressive disease which can have nodal and/or extranodal involvement.

It is twice as common in males and tends to present in the sixth and seventh decades. The majority of patients present with advanced stage disease, and it can be associated with eosinophilia, pruritus, and haemophagocytosis.

Diagnosis is by lymph node histology and staging via CT imaging (the role of PET-CT is debated). Other relevant tests include full blood count, renal/hepatic function, lactate dehydrogenase (LDH), uric acid and viral screen (HIV, hepatitis B/C).

Treatment is in the form of chemotherapy. First line standard therapy is usually with CHOP, with some evidence for the addition of etoposide (CHOEP) for patients under 60 years.
Treatment for refractory/relapsed disease varies among centres. Prognosis is generally poor.

IVE/MTX with auto-HSCT is a treatment option for relapsed/refractory disease, but it is important to be aware of toxicities. In this case we discussed ifosfamide-induced encephalopathy (IIE), which can present with symptoms ranging from mild confusion, somnolence, agitation, to hallucinations, extrapyramidal symptoms, and seizures. Hepatic impairment (particularly low albumin) is a recognised risk factor. Although there is a lack of convincing evidence, the use of methylene blue is usually advised for treatment, and sometimes for prophylaxis.

Dearden C, et al. Guidelines for the management of mature T‐cell and NK‐cell neoplasms (excluding cutaneous T‐cell lymphoma). Br J Haematol . 2011 May;153(4):451-85

D’Amore F, et al. Peripheral T-cell lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol . 2015 Sep;26 Suppl 5:v108-15

Click to access London-Cancer-Methylene-Blue-Guideline-v1.pdf

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