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.

Case Details

Disease Location

Angioimmunoblastic t-cell lymphoma (aitl)

Personal Characteristics

63 -year-old male congenitally bicuspid aortic valve benign prostate hypertrophy the patient had spent large amounts of time in many countries in africa, eastern asia, and south and central america. When in egypatient 2 years earlier he was bitten by a spider and developed a significant left cervical lymphadenopathy. He had a biopsy in poland and it was reportedly benign. The 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 the reporting institution. At that time, another cervical ln was biopsied which was benign and his ldh was elevated (183u/l)

Clinical Characteristics

Aitl is a rare hematologic neoplasm that classically presents with lymphadenopathy, hepatosplenomegaly, anemia and b-type symptoms patient 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. The fevers reached 40 degrees c and occurred regularly at 6- to 12-hour intervals, lasting 90 minutes each before resolving completely. There was positive urinary retention, frequency, and perineal burning sensation physical exam revealed bilateral tender cervical and submandibular lymphadenopathy a harsh precordial systolic murmur consistent with aortic stenosis was audible IGG antibodies to ebv viral capsid antigen and epstein-barr nuclear antigen were positive CT scan of the abdomen revealed periaortic and periportal lymphadenopathy and splenomegaly t-cell receptor gene rearrangement showed clonal t cells he was diagnosed with acute prostatitis, ciproflaxacin led to prompting resolution of fevers. He was discharged on day 5 with antibiotics but returned within a week due to fever recurrence when returned, MRI of the prostate ruled out prostatic abscess, ekg revealed bicuspid aortic valve stenosis, he was discharged and continued antibiotics for presumed prostatitis 2 weeks after this second discharge, he was hospitalized due to recurrent fevers and urinary symptoms. He now had progressive anemia and splenomegaly palpable to 2cm below the costal margin. Biopsies of a hypervascular cervical ln revealed an atypical lymphoid infiltrate with clonal t-cell receptor gene rearrangements suspicious for t-cell lymphoma versus reactive paracortical t-cell hyperplasia PET/CT revealed significant fdg-avid splenomegaly and lymphadenopathy both above and below the diaphragm, suggesting lymphoma bone marrow biospy revealed atypical t-cell infiltrate suggestive of peripheral t-cell lymphoma, with no cytogenetic abnormalities excisional biopsy of two fdg-avid cervical ln confirmed a diagnosis of aitl with patchy ebv uptake, he was discharged with oral ciprofloxacin a second opinion confirmed findings were most consistent with aitl and recommended chop chemotherapy prior to chemo, he underwent successful aortic valve replacement with a bioprosthesis and two-vessel coronary artery bypass grafting 6 months after noted sr, a 2nd PET/CT showed there was no ln fdg avidity but there was a marrow avidity. Bone marrow biopsy showed atypical lymphocytes and persistently decreased hematopoiesis but no lymphoma signs

Remission Characteristics

His fevers improved and due to the clinical improvement he sought a second opinion at his hematology follow-up 3 weeks after cardiac surgery, he was asymptomatic and denied recurrence of fevers, night sweats, and lymphadenopathy prechemotherapy PET/CT showed no evidence of fdg-avid lymphadenopathy, suggesting completely sr. He received no therapy between the two PET/CT scans, thus no chemo was started he returned 6 months later for a follow up and a repeat PET was performed showing no fdg-avid lymph nodes but there was an increased marrow fdg avidity with elevated ldh. After the bone marrow biopsy, 2 weeks later without therapy, the ldh declined to 235 u/l and continued to decline from this point forward he had no further lymphoma recurrence he started transfusions and prednisone therapy for his hypoproliferative anemia he responded well and after 1 month was no longer dependent on transfusion. Prednisone was tapered and discontinued 3 months later 12 months after being off corticosteroid, his lymphoma remains in remission

Treatment & Mechanisms

Proposed Remission Mechanisms

Literature regarding aitl showed that having an extra third chromosome was associated with a higher chance of sr the current patient didn't have any extrachromosomes, so the possible mechanism for sr is unknown

Clinical Treatment

Ciprofloxacin and antibiotics excisional biopsy of two cervcial lymph nodes oral ciprofloxacin transfusions and predinsone therapy

Non-Clinical Treatment

None reported