<b><i>Introduction:</i></b> Plasmablastic lymphoma (PBL) is a rare variant of diffuse large B-cell lymphoma for which no optimal treatment has been established and prognosis remains poor. Here, we describe a human immunodeficiency virus-uninfected patient with PBL that was refractory to conventional chemotherapies but was successfully controlled with a bortezomib-based regimen followed by a lenalidomide-based regimen. <b><i>Case Presentation:</i></b> A 64-year-old man suffered from nasal bleeding and occlusion. Whole-body computed tomography results revealed a large lesion occupying his nasal cavity. He was diagnosed with PBL based on a tumor biopsy and was treated with two lines of conventional chemotherapy. A dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (EPOCH) regimen and an ifosfamide, carboplatin, and etoposide (ICE) regimen were ineffective, but the bortezomib-based regimen CyBorD (bortezomib, cyclophosphamide orally, and dexamethasone orally) provided a clinical response. Due to peripheral neuropathy, the patient was then treated with a lenalidomide-based regimen (Ld; lenalidomide and dexamethasone). Although a complete response was not achieved, the Ld regimen was tolerated and was continued with a partial response (PR) for over 2 years. <b><i>Discussion/Conclusion:</i></b> In the present case, PBL that was refractory to conventional chemotherapies responded to the CyBorD regimen and a long-term Ld-based regimen without severe adverse effects. This strategy provided and maintained a PR for over 2 years. Despite not resulting in tumor reduction and only maintaining a PR, continued Ld treatment contributed to long-term survival of the present patient with PBL.
Adult T-cell Leukemia/Lymphoma (ATL) is a rare disease, related to human T-lymphotropic virus-1 (HTLV-1) and presented mainly in adulthood by generalised lymphadenopathy, hepatosplenomegaly, skin lesions and hypercalcaemia, with rare gastrointestinal and/or oral manifestations. We reported this case to raise awareness and demonstrate the therapeutic challenges of this rare disease. A 49-year-old Japanese female presented with skin papules on both forearms, painful mouth ulcers and multiple neck swellings since early February 2017. Initial clinical examination and laboratory investigations were misleading and her condition was diagnosed as candidiasis. Because of un-improvement of the case, a screening upper endoscopy was requested 2 months later and revealed characteristic oropharyngeal ulcers which were biopsied, and its pathologic examination confirmed smouldering type ATL. This case report should raise awareness of doctors and endoscopists about this disease especially in HTLV-1 endemic areas to avoid late diagnosis and help achieve earlier therapeutic decisions.
Genetic evaluation showed ETV6/NTRK3 fusion but no BRAF mutations or RET/PTC rearrangements. RET/PTC rearrangement and BRAF mutations often contribute to the pathogenesis of PTC; however, rearrangements of NTRK genes are relatively rare in pediatric PTC. Although NTRK rearrangement has been shown to often present unique pathological types and infiltrative architectures in the western population, such findings were not observed in this patient. Thus, the present case of classic PTC with ETV6/NTRK3 rearrangement highlights the disparate collection of clinic-pathological features compared to the trend in the western population. We therefore emphasize the need to further accumulate clinical as well as genetic data in pediatric PTCs.
The development of multiple histologic types of lymphoma in a single patient has been sporadically reported as sequential or composite lymphoma. However, the incidence pattern of such patients has been rarely evaluated in a large population-based setting. We investigated the incidence of sequential or composite lymphoma based on 11,174 lymphoma records from a population-based cancer registry between 1985 -2012 in Nagasaki Prefecture, Japan. We identified 99 lymphoma records were of 49 independent patients other than relapse. The prevalence of the sequential or composite lymphomas in a single patient was 0.44% (95% confidence interval [95% CI], 0.32-0.56%) without sex difference. Among the 49 patients, five (10.2%) were composite/discordant lymphoma. The most frequent "composite lymphoma" was a combination of diffuse large B-cell lymphomas (DLBCL) and adult T-cell leukemia (n = 3). A case of "discordant lymphoma" was a combination of follicular lymphoma on spleen and Waldenström macroglobulinemia on bone marrow. The rest of the patients (n = 44, 89.8% of all composite lymphoma) were "sequential lymphoma" with various combination of lymphoma subtypes on different dates. The major combination of the sequential lymphoma was DLBCL after marginal zone lymphomas (n = 4). In the era of improved survival of lymphoma patients, hematologists should be aware of the development of additional lymphomas.
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