Clinical Lymphoma, Myeloma & Leukemia最新文献

Background: Immunoglobulin light-chain (AL) amyloidosis is a rare plasma cell disorder with multiorgan involvement. Delayed diagnosis remains prevalent, contributing to high early mortality, particularly among patients with cardiac involvement.

Objectives: This study aimed to evaluate the diagnostic journey of AL amyloidosis patients, including presenting symptoms, diagnostic pathways, and perspectives on delays in diagnosis.

Methods: A cross-sectional survey was conducted through the HealthTree® CureHub among individuals with AL amyloidosis.

Results: Eighty-seven patients completed the survey between 03/12/2023 to 07/10/2024. Median age was 66 years. Initial signs/symptoms were most often self-identified. Most individuals experienced 1 to 3 symptoms, most commonly fatigue, weakness, and swelling. Most patients first sought care from primary care physicians (37%) or organ specialists (27%). A median of 3 providers were seen before diagnosis. Cardiologists and nephrologists established the diagnosis in only 16% and 14% of cases, respectively. A diagnostic delay was reported by 59%, with 35% experiencing delays >12 months; approximately 50% attributed the delay to lack of provider awareness, and 65% felt the diagnostic process required significant improvement.

Conclusions: Diagnostic delays remain a major challenge in AL amyloidosis. Since general practitioners and organ specialists are often the first point of contact, increasing awareness among these providers may improve diagnostic timelines.

Mantle cell lymphoma (MCL) is a rare non-Hodgkin lymphoma that, despite advances in treatment, remains incurable. Clinical courses can range from indolent to aggressive. As understanding of the pathophysiology improves and more targeted therapies shift to the frontline setting, treatment of relapsed and refractory MCL is becoming less defined. This review discusses the evidence of current and emerging treatment strategies for patients with relapsed and refractory MCL, including Bruton tyrosine kinase inhibitor (BTKi) therapies and immunotherapies such as bispecific T-cell engagers and chimeric antigen receptor T-cells.

Background: Lymphomas involving ocular structures are well documented, particularly in the conjunctiva, vitreoretina, and choroid. In contrast, no primary lymphoma of the cornea has previously been reported in humans, whereas it has only been described in animals, essentially in rare, small series in horses. This study aimed to determine whether corneal lymphoma exists in humans and to describe its epidemiologic and clinical characteristics.

Methods: A retrospective, population-based analysis was conducted using the SEER 17 registries (November 2024 submission). All lymphoma cases coded as C69.1 (cornea) between 2000 and 2022 were included. Demographic, pathological, treatment, and survival data were extracted using SEER*Stat version 9.0.42.0. Incidence rates were age-adjusted to the 2000 U.S. standard population. Survival was calculated from diagnosis to death or last follow-up.

Results: Five cases of corneal lymphoma were identified, all strictly localized to the cornea, corresponding to an incidence of 0.0028 cases per million persons per year. The mean age at diagnosis was 75 years, with a 4:1 male predominance. Histologic subtype was available in 3 cases, all of which were mucosa-associated lymphoid tissue (MALT) lymphomas. All patients underwent local surgical management, and radiotherapy was administered in unilateral cases. No systemic therapy was reported. Median follow-up was 99 months. Two deaths occurred (1 unrelated to lymphoma and 1 lymphoma-related) while 3 patients remained alive, including 2 long-term survivors with follow-up exceeding 14 years.

Conclusion: Corneal lymphoma does occur in humans, is exceptionally rare, and appears to share the favorable prognosis of other ocular MALT lymphomas. Radiotherapy may provide durable control in localized disease. These findings represent the first evidence of this entity and support heightened diagnostic awareness in ocular oncology.

Aims: We sought to evaluate the pattern of changes in cardiac magnetic resonance parameters among light-chain (AL) cardiac amyloidosis patients receiving anticlonal therapy.

Methods and results: This study included 35 consecutive patients with AL cardiac amyloidosis (AL CA) from a single amyloidosis center, surviving at least 1 year after diagnosis and undergoing 3T CMR at diagnosis and at 12-months follow-up. Structural, functional parameters, native T1, T2, myocardial and spleen extracellular volume (ECV), and peak left atrial strain (PALS) were recorded. Hematologic response was assessed per current criteria. Mean age was 64.1 years, 57% males and per Mayo staging system 10% were stage I, 32% stage II, and 58% stage III. During follow-up, 54.3% achieved complete hematologic response (CR), 34.3% very good partial response, and 11.4% partial response. Significant changes at 12-month landmark included a reduction in native T1 (1420 ms vs. 1393 ms, P = .001), ECV (46% vs. 43%, P = .004), left ventricular maximum wall thickness (15 mm vs. 13 mm, P = .02), and an increase in stroke volume (76 mL vs. 85 mL, P = .016). Patients with complete hematologic or cardiac response demonstrated improvements in T1 native and ECV. In a subset of n = 12 patients with a repeated follow-up CMR study at a median of 31.2 ± 5.04 months after treatment initiation, a trend towards further reduction in T1 and ECV was noted.

Conclusions: In AL-CA patients treated with anticlonal therapy, significant improvement in CMR parameters, correlate with hematologic and organ response and suggest potential cardiac amyloid regression and functional improvement.