Case Presentation: A 68-year-old Jamaican woman presented with generalized fatigue of one-week duration. On presentation, she also had dizziness and constipation. She was tachycardic and lethargic on examination. Laboratory values were remarkable for hypercalcemia of 17.3 mg/dl. She had lymphocytosis as high as 77000 (ALC 42.6). Serum lactate dehydrogenase (LDH) was 7000 U/L. Peripheral flow cytometry revealed 38.57% T cells, most of which were atypical, comprising 36.67% of the total cellularity. Human T-lymphotropic virus type 1 (HTLV-1) antibody was positive. CT chest revealed bilateral axillary lymphadenopathy. She developed spontaneous tumour lysis syndrome (TLS) with severe hyperkalemia, metabolic acidosis and acute kidney injury (AKI). Ferritin and LDH levels were significantly elevated, consistent with Hemophagocytic Lymphohistiocytosis (HLH) in the setting of leukemic adult T-cell leukemia/lymphoma (ATLL). Following treatment for TLS, which improved, treatment for ATLL was initiated. Despite initial improvement in her clinical condition, she developed non-ST segment elevation myocardial infarction and died from multiorgan failure.
Discussion: Approximately 95% of individuals infected with HTLV-1 are asymptomatic (1). Only 3-5% of patients manifest HTLV-1-associated diseases.(2) ATLL is a rare peripheral T lymphocyte cell neoplasm associated with HTLV-1. (4,5)Based on the Shimoyama classification, ATLL can present in four ways; acute, chronic, lymphoma and smoldering (6). Our patient’s presentation was acute. ATLL diagnosis is based on clinical manifestation, morphologic and immunophenotypic changes of the malignant cells. A lymph node biopsy can also be done to confirm the diagnosis. Treatment depends on subtype, specific country and institution guidelines, and available therapy plans. However, the most common treatment options include chemotherapy with multiple agents, antiretrovirals, interferon alpha and stem cell transplantation (7).There are no treatment options to prevent HTLV-1 replication in asymptomatic patients. In patients who developed HTLV-1-associated disease, there is no standard treatment for the lymphoproliferative effects of ATLL (8).The current emerging treatment regimens include combination chemotherapy agents and antiretroviral therapies, which are mostly found to be helpful in patients with a leukemic subtype of ATLL. There is a critical need for the development and discovery of treatments in providing virologic control in patients with HTLV-1-associated diseases.
Conclusions: This case highlights the clinical challenges and complexities associated with ATLL in a patient with HTLV-1 infection, presenting with severe hypercalcemia and tumour lysis syndrome. Despite HTLV-1’s global prevalence, symptomatic cases remain rare, and patients who do develop ATLL often present with advanced and multifaceted disease manifestations. The patient in our report faced a complicated hospital course, reflecting the aggressive nature of ATLL and the limitations of current treatment options. While therapeutic strategies, including multi-agent chemotherapy and antiretroviral therapy, offer some benefits, the need for effective treatment for HTLV-1-associated diseases remains unmet. This case underscores the importance of further research to improve treatment modalities and develop virologic control measures for HTLV-1, particularly in managing ATLL and preventing disease progression in infected individuals.