Angioimmunoblastic T Cell Lymphoma Occurring as a Synchronous Malignancy with Chronic Myeloid Leukemia

Introduction:
Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon subtype of peripheral T-cell lymphoma (PTCL). We encountered a rare case of a patient who presented with bilateral neck masses. Biopsy of the lymph nodes and flow cytometry led to the diagnosis of AITL. Blood work raised suspicion of CML. To our knowledge, this may be the first reported case of coexistence of a newly diagnosed AITL and CML.
Methods:
A 68-year-old male presented with gradually enlarging bilateral neck masses over 5 months, involving the left side initially and then progressing to the right. On examination, a left-sided mass (5 to 6 cm) and a right-sided mass (2 cm) were noted. Blood work was significant for leukocytosis (38,700 with 77% neutrophilic predominance) and thrombocytosis (643,000). Ultrasound revealed multiple enlarged lymph nodes in the neck. An excisional biopsy showed lymph node effacement by small to medium-sized lymphoid cells with pale cytoplasm and increased vasculature flow cytometry showed 97% lymphocytes with 36% immunophenotypically abnormal T cells and molecular studies were positive for T-cell receptor beta and gamma gene rearrangements, suggestive of AITL. PET scan revealed supradiaphragmatic fluorodeoxyglucose (FDG) avid lymphadenopathy on the left involving extranodal tissue in the subcutaneous fat overlying the left infraspinatus, corresponding to stage II E disease. The leukocytosis and thrombocytosis were contrary to AITL, as cytopenias are classically seen. This raised suspicion of a coexisting malignancy. A bone marrow biopsy and FISH- t(9;22) confirmed the diagnosis of CML..
Results:
After six cycles including the CHOP regimen (cyclophosphamide, doxorubicin, vincristine plus prednisone every 21‚Äâdays) for AITL and 400 mg of imatinib for CML, a PET scan showed resolution and the patient remained in remission, at 1 year from diagnosis.
Discussion:
AITL arises from the follicular helper T cells. It accounts for 1-2% of all non-Hodgkin’s Lymphoma (NHL), with an incidence of only 0.05 cases/100,000 in the US. Ras-homology family member A (RHOA) RHOA is the disease-defining mutation in AITL in ~80% of the cases, followed by ET2, IDH2, DNMT3A, CD28, and phospholipase C gamma 1 (PLCG1). Overexpression of chemokine CXCL13 suggests its derivation from follicular helper T cells.