P046 A rare case of peripheral T cell lymphoma presenting as peripheral neuropathy & myositis
Authors: Ashraf Madiha , David Joel 
Published: April 2022

Background/Aims

Peripheral T cell Lymphomas are a heterogeneous group of very aggressive and rare lymphomas in the western world accounting for 5% to 15% of non-Hodgkin lymphomas. Inflammatory myositis and nephropathy associated with hematological malignancies are extremely rare.

Methods

A 64-year-old gentleman presented with progressive neuropathy with pain and numbness in feet, clawing in both hands, muscle weakness and wasting. He also noticed a rash in his groin and scrotum (erythematous) and new erectile dysfunction along with Raynaud’s, dry eyes, and unintentional weight loss over a year. His paternal uncle suffered from peripheral neuropathy. He had splinter hemorrhage and telangiectasia on general examination. Neurological examination revealed clawing of hands, muscle wasting in his hands and legs in distribution of right median, ulnar, left radial, bilateral sural and bilateral peroneal nerve.

Results

He had normal routine blood tests (FBC, U&Es and LFTS and CRP). Rheumatology work up revealed CK of 204, LDH 317, ANA 1: 640 titre, negative ENA, dsDNA, ANCA, RF, anti CCP antibodies, myositis blot, normal C3, C4, protein electrophoresis, IgG sub classes and negative para neoplastic screen including anti Ri, anti Yo and anti Hu and a negative urine dip for blood or proteins. Echocardiogram revealed small pericardial effusion. PET scan showed markedly avid lesion in ascending colon proven to be low-grade tubulovillous adenoma on biopsy. MRI of thighs showed inflammatory change consistent with myositis and nerve conduction studies has revealed mono neuritis multiplex. Peroneal nerve biopsy showed small vessel lymphocytic vasculitis followed by skin biopsy of thigh and shin consistent with features of lymphocytic vasculitis and angiocentric NK cell lymphomatous changes. On the bases of his presenting symptoms and investigations, he was treated with 60mg of oral prednisolone and pulsed cyclophosphamide at dose of 15mg /kg for 6 cycles for multisystemic inflammatory condition causing mono neuritis multiplex and myositis. His cyclophosphamide was stopped after 4 cycles as there was no response to treatment and MDT review confirmed that skin and nerve biopsies features were consistent with peripheral T cell lymphoma. Further treatment was with CH(O)P (minus vincristine) under the care of oncologists.

Conclusion

Peripheral T cell lymphoma (PTCL) is a rare and aggressive heterogeneous group of lymphomas. Risk factors include family history of hematological malignancies and smoking. Rashes may be the antecedent finding in about 20% to 50% of angioimmunoblastic T cell lymphoma patients, with skin manifestations ranging from urticarial lesions to nodular tumor. Patients with cutaneous T cell lymphomas often present with “chronic dermatitis” which is resistant to therapy, early-stage having a predilection for folds and non-exposed sun areas. Cancer associated inflammatory myopathies are rare syndromes occurring in 10%-30% of all malignancies and inflammatory myopathies associated with hematological malignancies are even rarer entities.

Disclosure

M. Ashraf: None. J. David: None.