HUMAN T-CELL LYMPHOTROPIC VIRUS TYPE 1-ASSOCIATED ADULT T-CELL LEUKEMIA/LYMPHOMA AND PROGRESSION TO HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A CASE REPORT

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Human T-cell lymphotropic virus type 1 (HTLV-1) was the first human retrovirus to be discovered [1]. Its infection can lead to adult T-cell leukemia/lymphoma (ATLL). Hemophagocytic lymphohistiocytosis (HLH) is a syndrome with a mortality of 95% caused by overstimulation of the immune system [2,3,4]. This case report presents a male with HTLV-1—associated ATLL who developed HLH. CASE PRESENTATION: A 75 year-old Haitian male with a 22 pack-year smoking history presented to our hospital after a pre-syncopal episode with a 10 day history of pleuritic chest pain. Labs were significant for leukocytosis of 26.2 *10^9/L with lymphocyte predominance. Chest x-ray showed bilateral pleural effusions. Cytometry of the pleural fluid showed an aberrant population of CD4(+) T lymphocytes consistent with post-thymic T-cell lymphoma and atypical T lymphocytes, positive for CD45RO, CD25, and TCRab and negative for CD26 and CD56, consistent with ATLL. Bronchoscopy with endobronchial ultrasound-guided fine needle aspiration of lymph node stations 4R, 7, and 11L revealed aberrant T-cells. TCR gene rearrangement and HTLV-1 western blot confirmed ATLL due to HTLV-1. He was started on CHOP and brentuximab, but was readmitted weeks later with encephalopathy and acute hypoxic respiratory failure. CMV viremia was confirmed via serum PCR and ganciclovir was initiated. Labs were significant for elevated ferritin 9,464 ng/mL, triglycerides 162 mg/dL, D-dimer 549 ng/mL, low fibrinogen 150 mg/dL, and worsening cytopenia with hematocrit 27.1% and platelets 58*10^9/L. Bone marrow biopsy demonstrated histiocytes with engulfed erythroid cells, confirming HLH. The patient was treated with dexamethasone, but was too unstable for etoposide and died 12 days after readmission. DISCUSSION: Lymphoma is the third most common cause of malignant pleural effusion (MPE) and patients have an overall survival of 3-12 months after diagnosis [5]. Diagnostic tests for ATLL are usually done with serum and rarely from MPE, as was done in this case [6]. Signs of HTLV-1—associated ATLL include marked leukocytosis with atypical lymphocytes, rash, and lymphadenopathy [1]. Our patient did not have a rash, but had other signs and symptoms consistent with the presentation of HTLV-1—associated ATLL. HLH likely occurred from progression of HTLV-1—associated ATLL, but CMV viremia or chemotherapy could have contributed. The diagnosis of HLH is difficult because symptoms are nonspecific, requiring a high index of suspicion [7]. The optimal treatment for HLH is still unknown and a multidisciplinary approach is essential. CONCLUSIONS: This case demonstrates HLTV-1—associated ATLL that initially presented as MPE, and was a subsequent trigger for HLH. REFERENCE #1: Rodriguez-Zuniga MJM, Corte-Franco F, Qujiano-Gomero E. Adult T-Cell Leukemia/Lymphoma. Review of the Literature. Actas Dermosifiliogr. 2018;109(5):399-407 REFERENCE #2: Atteritano M, David A, Bagnato G, et al. Haemophagocytic syndrome in rheumatic patients. A systematic review. Eur Rev Med Pharmacol Sci. 2012;16(10):1414-1424 REFERENCE #3: Bindra BS, Garcia de de Jesus K, Cisneros O, Jorge VM, Kaur H. Hemophagocytic Lymphohistiocytosis: Management and Special Consideration in Human Immunodeficiency Virus Positive Patients with Immune Reconstitution Syndrome. Cureus. 2019;11(8):e5402 DISCLOSURES: No relevant relationships by Michael Bender, source=Web Response No relevant relationships by Liane Emerson, source=Web Response No relevant relationships by Chaim Rube, source=Web Response