Abstract

Introduction: Angioimmunoblastic T Cell Lymphoma (AITL) is a well-recognized subtype of peripheral T cell lymphoma. It occurs predominantly in the lymph nodes with presence of systemic symptoms and carries a dismal prognosis. AITL accounts for about 1% - 2% of all cases of non-Hodgkin lymphoma. Case presentation: A 59-year-old gentleman of Chinese ethnicity with no prior medical illness presented to Tengku Ampuan Afzan Hospital with a two-month history of fever, anorexia, unintentional weight loss and generalized lymphadenopathies. Physical examination revealed diffuse lymphadenopathies involving the cervical, axillary and inguinal regions bilaterally. He had hepatosplenomegaly. An excisional biopsy of the cervical and inguinal lymph nodes was compatible with AITL. The bone marrow biopsy demonstrated disease infiltration. He was treated with 6 cycles of Etoposide-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) induction chemotherapy followed by consolidation high dose therapy-autologous stem cell transplant (HDT-ASCT). He achieved complete remission on 18-Fluoro- deoxyglucose Positron Emission Tomography (18-FDG-PET) imaging. His bone marrow biopsy showed disease clearance. Conclusion: The diagnosis of AITL remains challenging and often poses a dilemma to clinicians and lymphoma pathologists. The natural history of AITL remains very variable with many of them relapsing subsequently despite achieving prior complete remission.

Highlights

  • Angioimmunoblastic T Cell Lymphoma (AITL) is a well-recognized subtype of peripheral T cell lymphoma

  • The bone marrow biopsy demonstrated disease infiltration. He was treated with 6 cycles of Etoposide-CHOP induction chemotherapy followed by consolidation high dose therapy-autologous stem cell transplant (HDT-ASCT)

  • AITL occurs predominantly in the lymph nodes with the presence of systemic symptoms and carries a dismal prognosis

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Summary

Introduction

AITL accounts for about 1% - 2% of all cases of non Hodgkin lymphoma [2]. 20% of cases of Peripheral T Cell Lymphoma (PTCL) are attributed to AITL annually [2]. The median age of diagnosis is 65 and AITL does not have a clear gender predisposition. Almost 70% of patients with AITL have bone marrow involvement at diagnosis and most of them present at advanced stage [4]. The main objective of reporting this case is to share with the medical fraternity of a rare presentation of Angioimmunoblastic T Cell Lymphoma which has a variable natural history

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