Spontaneous Complete Remission of Angioimmunoblastic T-Cell Lymphoma

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a rare hematologic neoplasm that typically presents with B symptoms, anemia, and lymphadenopathy. Its overall prognosis is poor, with a 5-year survival rate of 30%. We present a case of AITL that went into spontaneous remission, an uncommon occurrence.A ngioimmunoblastic T-cell lymphoma (AITL) is a rare hematologic neoplasm that classically presents with lymphadenopathy, hepatosplenomegaly, anemia, and B-type symptoms. Although the disease has a poor prognosis, we present a patient with a rare spontaneous remission. CASE REPORTA 63-year-old man with a congenitally bicuspid aortic valve and benign prostate hypertrophy presented to the emergency department complaining of cyclical fevers and 20-pound unintentional weight loss over the previous 3 weeks and was admitted to the hospital for workup of fever of unknown origin. Th ese fevers reached 40°C and occurred regularly at 6-to 12-hour intervals, lasting 90 minutes each time before resolving completely. Review of systems was also positive for urinary retention, frequency, and perineal burning sensation.Th e patient had spent large amounts of time in many countries in Africa, Eastern Asia, and South and Central America. While in Egypt 2 years earlier, he was bitten by a spider and developed signifi cant left cervical lymphadenopathy. A cervical lymph node biopsy in Poland was reportedly benign. Th e procedure was complicated by left spinal accessory nerve damage, and 6 months prior to onset of his presenting symptoms he underwent surgical repair of the nerve at our institution. At that time another cervical lymph node was biopsied, which was benign, and his lactate dehydrogenase (LDH) level was 183 U/L (normal, 122-222 U/L).Physical examination revealed bilateral tender cervical and submandibular lymphadenopathy, but no axillary or inguinal lymphadenopathy. A harsh precordial systolic murmur consistent with aortic stenosis was audible. No hepatosplenomegaly was found. Except for the presence of IgG antibodies to EpsteinBarr virus (EBV) viral capsid antigen and Epstein-Barr nuclear antigen, serologic testing for multiple infections was negative ( Table 1). Results of a peripheral smear, blood cultures, and urinalysis were all negative, and the chest x-ray showed no infi ltrates. Computed tomography scan of the abdomen revealed periaortic and periportal lymphadenopathy and splenomegaly. Flow cytometry revealed no increase in aberrant CD3/CD16-positive T cells or increase in natural killer cells. T-cell receptor gene rearrangement showed clonal T cells. Due to the clinical presentation and elevated infl ammatory markers (Table 2), he was diagnosed with acute prostatitis. Ciprofl oxacin led to prompt resolution of fevers. He was discharged home on day 5 with antibiotics but returned within a week due to fever recurrence. At this time, magnetic resonance imaging of the prostate

The Incremental Prognostic Value of Baseline 18F‐FDG PET/CT Imaging in Angioimmunoblastic T‐Cell Lymphoma

“…The clinical course of AITL is complex and the treatment response is different. Humeniuk et al [2] reported a case of AITL that went into spontaneous remission, an uncommon occurrence. Besides, the majority of AITL patients showed an aggressive course and dismal outcome with current therapies [1,3].…”