[Ruptured mycotic cerebral aneurysm in an adult T-cell leukemia/lymphoma patient undergoing allogeneic stem cell transplantation].

A 63-year-old man with adult T-cell leukemia-lymphoma underwent allogeneic bone marrow transplantation from an HLA-matched unrelated donor. On day 17 after transplantation, chest computed tomography (CT) showed nodules in the lower lobes of both lungs, and invasive pulmonary aspergillosis (IPA) was suspected. Treatment with liposomal amphotericin B was started, and improvement of infectious lesions was confirmed with CT on day 28. The antifungal agent was changed to voriconazole on day 52 because of progressive renal dysfunction. Disorders of consciousness and paralysis of the left upper and lower extremities developed on day 61. Brain CT showed subcortical hemorrhage in the right parietal and occipital lobes, and the patient died on day 62. An autopsy revealed filamentous fungi, suspected to be Aspergillus, in the pulmonary nodules and a ruptured cerebral aneurysm. Although IPA occurs in 10% of transplant recipients, vigilant monitoring for mycotic cerebral aneurysms is required to prevent hematogenous dissemination of Aspergillus, which is associated with a high mortality rate.

Human T-cell leukemia virus type 1 is associated with dysthyroidism in the French Amazon.

Background: Human T-cell leukemia virus type 1 (HTLV-1) is a retrovirus known to cause two major diseases: adult T-cell leukemia/lymphoma and a progressive neuromyelopathy-tropical spastic paraparesis. Many viruses may be involved in the pathogenesis of thyroiditis; however, few studies have focused on the role of HTLV-1. We aimed to investigate the association between HTLV-1 and biological thyroid dysfunction. Methods: We included 357 patients with a positive HTLV-1 serology and thyroid-stimulating hormone assay data between 2012 and 2021 in a hospital in French Guiana; we compared the prevalence of hypothyroidism and hyperthyroidism in this group with that in an HTLV-1-negative control group (722 persons) matched for sex and age. Results: The prevalence of hypothyroidism and hyperthyroidism in patients with HTLV-1 infection was significantly higher than that in the control group (11% versus 3.2% and 11.3% versus 2.3%, respectively; p < 0.001). Conclusion: Our study shows, for the first time, the association between HTLV-1 and dysthyroidism in a large sample, suggesting that thyroid function exploration should be systematically implemented in this population as this may have an impact on therapeutic management.

Impact of HLA-mismatched unrelated transplantation in patients with adult T-cell leukemia/lymphoma.

This Japanese nationwide retrospective study investigated the impact of HLA-mismatched unrelated transplantation for adult T-cell leukemia-lymphoma (ATL) patients who received transplantation between 2000 and 2018. We compared 6/6 antigen-matched related donor (MRD), 8/8 allele-matched unrelated donor (8/8MUD), and 1 allele-mismatched unrelated donor (7/8MMUD) in the graft-versus-host direction. We included 1191 patients; 449 (37.7%) were in the MRD group, 466 (39.1%) in the 8/8MUD group, and 276 (23.7%) in the 7/8MMUD group. In the 7/8MMUD group, 97.5% of patients received bone marrow transplantation, and no patients received post-transplant cyclophosphamide. The cumulative incidences of non-relapse mortality (NRM) and relapse at 4 years, and the probabilities of overall survival at 4 years in the MRD group were 24.7%, 44.4%, 37.5%, in the 8/8MUD group were 27.2%, 38.2%, and 37.9%, and in the 7/8MMUD group were 34.0%, 34.4%, and 35.3%, respectively. The 7/8MMUD group had a higher risk of NRM (hazard ratio (HR) 1.50 [95% CI, 1.13-1.98; P = 0.005]) and a lower risk of relapse (HR 0.68 [95% CI, 0.53-0.87; P = 0.003]) than the MRD group. The donor type was not a significant risk factor for overall mortality. These data suggest that 7/8MMUD is an acceptable alternative donor when an HLA-matched donor is unavailable.

Human T-cell Leukemia Virus Type 1 (HTLV-1)-induced Uveitis.

Human T-cell leukemia virus type 1 (HTLV-1) is a human retrovirus that causes T-cell malignant diseases (adult T-cell leukemia/lymphoma) and HTLV-1-related non-malignant inflammatory diseases, such as HTLV-1 uveitis. Although the symptoms and signs of HTLV-1 uveitis are nonspecific, intermediate uveitis with various degrees of vitreous opacity is the most common clinical presentation. It can occur in one or both eyes and its onset is acute or subacute. Intraocular inflammation can be managed with topical and/or systemic corticosteroids; however, recurrence of uveitis is common. The visual prognosis is generally favorable, but a certain proportion of patients have a poor visual prognosis. Systemic complications of patients with HTLV-1 uveitis include Graves' disease and HTLV-1-associated myelopathy/tropical spastic paraparesis. This review describes the clinical characteristics, diagnosis, ocular manifestations, management, and immunopathogenic mechanisms of HTLV-1 uveitis.

Isolated cardiac valve involvement in smoldering adult T-cell leukemia/lymphoma.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive mature T-cell neoplasm caused by infection with the Human T-cell Lymphotropic Virus Type 1 (HTLV-1). Cardiac involvement in patients with ATLL is infrequent, and when it happens it is usually seen in aggressive ATLL subtypes. However, ATLL presenting as isolated cardiac valve involvement is extremely rare. To date, only three histologically proven cases of ATLL with isolated cardiac valve involvement have been reported. Herein, we describe a 61-year-old Peruvian man who presented heart failure symptoms secondary to progressive cardiac valve infiltration. The patient underwent mitral valve replacement with a mechanical prosthesis. Histopathological evaluation of the resected valve revealed leaflet thickening with a nodular appearance due to fibrous tissue containing atypical T-lymphocytes with Foxp3 expression, infiltrating all layers of the resected valve. Interestingly, tumor cells were distributed around an incidental venous malformation (i.e., cavernous hemangioma). Postoperative evaluation demonstrated positive serology for HTLV-1, and a diagnosis of ATLL was established. Postoperative positron emission tomography/computed tomography did not show lesions outside the heart and cell blood counts were within normal range with low level of circulating CD4+ CD25+ lymphoma cell counts (7%); therefore, patient's disease was considered as smoldering ATLL and a "watch and wait" strategy was pursued. Currently, the patient is alive with no progression of disease after 18 months from diagnosis. Isolated cardiac valve involvement by ATLL should be considered in the differential diagnosis of HTLV-1 carriers with progressive heart failure, even when systemic lymphoma involvement is absent or not apparent.

Radiation Recall Dermatitis after Donor Lymphocyte Infusion for Adult T-Cell Leukemia Lymphoma Post-Allogeneic Stem Cell Transplant.

To our knowledge, this is the first case report describing radiation recall dermatitis (RRD) after donor lymphocyte infusion post-allogeneic stem cell transplant in a patient with acute T-cell leukemia lymphoma. Given its rare occurrence, unclear clinical characterization, and etiology, RRD remains poorly understood. In the setting of novel immunotherapies and recent development of COVID-19 mRNA vaccines, we aimed to better characterize RRD and its most likely pathogenesis in our patient's case.

[A Case of T-Cell Lymphoma Treated by Emergency Surgery Due to Bleeding of Jejunal Lesion].

A 78-year-old man was aware of lightheadedness and darkness at a routine outpatient visit, and his blood pressure was declined at 87/51 mmHg. Contrast-enhanced CT scan showed an extravascular leakage image at jejunum. We diagnosed as small intestinal hemorrhage. Because he was in hemorrhagic shock, emergency surgery was performed. A tumor was found coincident with the bleeding site, and partial resection of the small intestine including enlarged lymph nodes was performed. Based on the pathological findings of T-cell origin and positive for serum anti-HTLV-1 antibody, he was suspected as adult T-cell leukemia/lymphoma(ATLL). Endoscopic examination of the upper and lower gastrointestinal tracts, bone marrow examination, and PET-CT scan were performed, but no other lesions were found. We report a case of the T-cell lymphoma with suspected solitary ATLL of the jejunum.

Particularities of Neurological Manifestations in Adult T-Cell Leukemia/Lymphoma: Need for a Multidisciplinary Approach-A Narrative Review.

ATL is a rare but a highly aggressive T-cell neoplasm associated with human T-cell leukemia virus-1 (HTLV-1) infection. Human T-cell lymphotropic virus type-1 (HTLV-1) is a oncogenic retrovirus responsible for the development of adult T-cell leukemia (ATL), but also for other non-malignant diseases, such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). HTLV-1 has a higher prevalence in Japan, the Caribbean, South America, intertropical Africa, Romania, and northern Iran. ATL patients can have an extensive spectrum of neurological manifestations. Numerous factors can be implicated, such as central nervous system infiltrates, neurolymphomatosis, complications to medication or allogeneic stem cell transplantation, HAM/TSP, infections, metabolic disturbances. The neurological complications are not always easy to recognize and treat. Thus, this review underlines the necessity of a multidisciplinary approach in ATL patients with neurological symptomatology.

Effect of HTLV-1 Infection on the Clinical Course of Patients with Rheumatoid Arthritis.

Human T-cell leukemia virus type 1 (HTLV-1) is the causative agent of adult T-cell leukemia/lymphoma (ATL) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). The effects of HTLV-1 on health are not fully elucidated. Epidemiological studies have shown that the prevalence of HTLV-1 infection is high in patients with rheumatic diseases. The prevalence of comorbidities, such as Sjögren's syndrome and rheumatoid arthritis (RA), is higher in patients with HAM/TSP than the in general population. Studies have shown the effects of HTLV-1-infection on the clinical course of RA. Major questions on the association between HTLV-1 infection and RA: (1) Is it possible that HTLV-1 infection causes RA? (2) Do patients with RA who are infected with HTLV-1 have different clinical features? (3) Are immunosuppressants associated with an increased prevalence of HAM/TSP or ATL in RA patients with HTLV-1 infection? Is ATL an immunosuppressive therapy-associated lymphoproliferative disorder? No large-scale studies have investigated the incidence of ATL in patients with RA. However, several studies have reported the development of ATL in patients with RA who have HTLV-1 infection. This review aimed to shed light on the association between HTLV-1 infection and RA and summarize the unmet medical needs of RA patients with HTLV-1 infection.

Progression of Infective Dermatitis Associated with HTLV-1 to Adult T-Cell Leukemia/Lymphoma-Case Report and Literature Review.

ABSTRACT: The human T-cell lymphotropic virus type 1 is a retrovirus that may cause severe diseases such as infective dermatitis associated with HTLV-1 (IDH) and adult T-cell leukemia/lymphoma (ATL). IDH is a chronic relapsing infected eczema of childhood, and ATL is a distinct type of peripheral T-cell leukemia/lymphoma, which is classified into the following types: smoldering, primary cutaneous tumoral, chronic, lymphoma, and acute. Progression of IDH to ATL during the course of IDH has been previously reported in 3 young patients, two of them from Bahia (Brazil). We present the case of a 22-year-old man who had IDH since childhood and developed ATL 18 months ago. The lymphoma lesions were superimposed on previously existing IDH lesions (forehead, axillae, umbilical area, and neck) or in areas generally affected by IDH (external genitalia, hypogastrium, groin, and eyelid). Cutaneous lesions in ATL are very frequent, but in this patient, besides infiltrated plaques and papules presented vesicles on the skin corresponding histologically to dilated Pautrier abscesses. Vesicular ATL is a rare condition. This case constitutes a very demonstrative example of the close correlation between IDH and ATL.

Adult T Cell Leukemia/Lymphoma Becoming Apparent during Treatment of Pulmonary Abscess and Empyema Caused by Nocardia asiatica: A Case Report and Review of the Literature.

Nocardia is a Gram-positive bacterium that causes opportunistic infections. Nocardia asiatica was newly isolated in 2004, and there have been no case reports describing the empyema caused by N. asiatica. Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-cell malignancy caused by human T-cell leukemia virus type 1 (HTLV-1). We herein report a case in which immunosuppression attributable to ATL may have led to pulmonary abscess and empyema caused by N. asiatica. Our case demonstrates the need to investigate causes of immunosuppression, including ATL, in patients showing nocardiosis.

Cardiac Involvement of Adult T Cell Leukemia/Lymphoma.

Adult T-cell leukemia/lymphoma (ATL) is a refractory T-cell lymphoma with variable clinical profiles, commonly exhibiting extra-nodal involvement. The myocardial involvement of ATL is often detected at an autopsy; however, the development of a symptomatic cardiac mass due to ATL is extremely rare. We herein report a 65-year-old man with ATL who developed cardiac symptoms due to a rapidly enlarging left ventricular mass soon after the initiation of systemic chemotherapy. We also summarize previously reported cases of symptomatic ATL with cardiac involvement.

Assessing smoldering adult T cell leukemia/lymphoma: a case report.

Adult T cell leukemia/lymphoma (ATLL) is a rare, extremely aggressive malignancy with four different clinical variants, all of which are associated with human T cell lymphotropic virus type 1. Antiretrovirals have been recognized as an effective therapy after study in clinical trials around the world. However, oncologists traditionally wait for asymptomatic patients with ATLL to reach a more severe stage of disease before treatment is initiated. We describe a patient with Fitzpatrick Skin Type V who presented with asymptomatic dyschromia of her neck, breast, and jaw. Her clinical, laboratory, and histological findings were consistent with smoldering type-ATLL. We teamed up with her oncologist to successfully treat her cutaneous symptoms with interferon-alpha/zidovudine. This report demonstrates efficacy with the employment of antiretroviral therapy immediately following a diagnosis of smoldering type ATLL.

A refractory human T-cell leukemia virus type 1-associated myelopathy/tropical spastic paraparesis patient with lymphoma-type adult T-cell leukemia/lymphoma: A case report and review of the literature.

RATIONALE: Adult T-cell leukemia/lymphoma (ATL) and human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) are caused by HTLV-1, but the coexistence of both disorders is rare. The estimated incidence is approximately 3%. PATIENT CONCERNS: A 54-year-old man was unable to stand up because of spastic paraparesis 1 month after the onset. He developed lymphadenopathy in the left supraclavicular fossa 5 months after the onset. The spastic paraplegia and sensory symptoms below the thoracic spinal cord level worsened. DIAGNOSES: Both blood and cerebrospinal fluid (CSF) tests were positive for anti-HTLV-1 antibodies. The patient was diagnosed with rapidly progressive HAM/TSP. He was also diagnosed with lymphoma-type ATL by the biopsy specimen of the lymph node. CSF examination at the time of symptom exacerbation showed abnormal lymphocytes, suggesting central infiltration of the ATL in the central nervous system. INTERVENTIONS: Methylprednisolone pulse therapy and oral prednisolone maintenance therapy were administered for rapidly progressive HAM/TSP. Intrathecal injection of methotrexate was administered for the suggested central infiltration of the ATL. OUTCOMES: Methylprednisolone pulse therapy and intrathecal injection of methotrexate did not improve the patient's exacerbated symptoms. Five months later, clumsiness and mild muscle weakness of the fingers appeared, and magnetic resonance imaging showed swelling of the cervical spinal cord. Clonality analysis showed monoclonal proliferation only in the DNA of a lymph node lesion, but not in the CSF and peripheral blood cells. LESSONS: This was a case of rapidly progressive HAM/TSP associated with lymphoma-type ATL that was refractory to steroids and chemotherapy. The pathogenesis was presumed to involve ATL cells in the brain and spinal cord because of the presence of abnormal lymphocytes in the CSF, but DNA analysis could not prove direct invasion. This case suggests that when we encounter cases with refractory HAM/TSP, it should be needed to suspect the presence of ATL in the background.