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Morphological Clues of Acute Monocytic Leukemia in COVID-19-Induced Transient Leukoerythroblastic Reaction with Monocytosis. COVID-19 诱导的伴有单核细胞增多的一过性红细胞白血病反应中急性单核细胞白血病的形态学线索
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-05-28 DOI: 10.3390/hematolrep16020033
Ingrid S Tam, Mohamed Elemary, John DeCoteau, Anna Porwit, Emina E Torlakovic

Viral infections, including those caused by COVID-19, can produce striking morphologic changes in peripheral blood. Distinguishing between reactive changes and abnormal morphology of monocytes remains particularly difficult, with low consensus rates reported amongst hematopathologists. Here, we report a patient who developed transient monocytosis of 11.06 × 109/L with 32% promonocytes and 1% blasts during hospitalization that was secondary to severe COVID-19 infection. Three days later, the clinical status of the patient improved and the WBC had decreased to 8.47 × 109/L with 2.2 × 109/L monocytes. Flow cytometry studies did not reveal immunophenotypic findings specific for an overt malignant population. At no time during admission did the patient develop cytopenia(s), and she was discharged upon clinical improvement. However, the peripheral blood sample containing promonocytes was sent for molecular testing with an extended next-generation sequencing myeloid panel and was positive for pathogenic NPM1 Type A and DNMT3A R882H mutations. Subsequently, despite an essentially normal complete blood count, the patient underwent a bone marrow assessment that showed acute myeloid leukemia with 77% promonocytes. This case emphasizes the critical importance of a full work up to exclude acute leukemia when classical promonocyte morphology is encountered in the peripheral blood. Promonocytes are not a part of the reactive changes associated with COVID-19 and remain specific to myeloid neoplasia.

病毒感染(包括由 COVID-19 引起的感染)可在外周血中产生显著的形态变化。区分单核细胞的反应性变化和异常形态仍然特别困难,血液病理学家之间的共识率很低。在此,我们报告了一名因严重 COVID-19 感染而在住院期间出现一过性单核细胞增多症的患者,其单核细胞为 11.06 × 109/L,原核细胞占 32%,1% 为血泡。三天后,患者的临床状况有所改善,白细胞降至 8.47 × 109/L,其中单核细胞为 2.2 × 109/L。流式细胞术研究没有发现明显恶性肿瘤的特异免疫表型。患者在入院期间从未出现细胞减少症,临床症状好转后便出院了。然而,含有原核细胞的外周血样本被送去进行分子检测,经扩展的新一代测序骨髓细胞面板检测,发现致病性 NPM1 A 型和 DNMT3A R882H 突变呈阳性。随后,尽管全血细胞计数基本正常,但患者接受了骨髓评估,结果显示其患有急性髓性白血病,原核细胞占 77%。该病例强调,当外周血中出现典型的原核细胞形态时,全面检查以排除急性白血病至关重要。原核细胞不属于与 COVID-19 相关的反应性变化,仍然是髓细胞瘤的特异性表现。
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引用次数: 0
Phase II Trial of Romidepsin as Consolidation Therapy after Gemcitabine, Dexamethasone, and Cisplatin in Elderly Transplant-Ineligible Patients with Relapsed/Refractory Peripheral T-Cell Lymphoma. 罗米地平作为吉西他滨、地塞米松和顺铂治疗后的巩固疗法,用于不符合移植条件的老年复发性/难治性外周T细胞淋巴瘤患者的II期试验。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-05-28 DOI: 10.3390/hematolrep16020034
Satoshi Yamasaki, Hiroatsu Iida, Akio Saito, Morio Matsumoto, Yoshiaki Kuroda, Tohru Izumi, Akiko M Saito, Hiroaki Miyoshi, Koichi Ohshima, Hirokazu Nagai, Hiromi Iwasaki

Romidepsin is an important therapeutic option for patients with peripheral T-cell lymphoma (PTCL). However, the timing of romidepsin administration remains controversial. The objective of this study was to characterize the safety and efficacy of romidepsin as consolidation therapy after gemcitabine, dexamethasone, and cisplatin (GDP) therapy (GDPR). This study of patients treated between March 2019 and March 2021 was registered with the Japan Registry of Clinical Trials (registration number: jRCT0000000519). If complete response, partial response, or stable disease was confirmed after 2-4 GDP cycles, romidepsin was administered every 4 weeks for 1 year. Seven patients with relapsed/refractory (R/R) PTCL (T-follicular helper phenotype [n = 1] and angioimmunoblastic T-cell lymphoma [n = 6]) were included in this prospective study (PTCL-GDPR). After a median follow-up of 34 months of patients in PTCL-GDPR, the 2-year overall survival rate was 71%, and the overall response rate after treatment was 57%. Common adverse events in patients with PTCL-GDPR included hematological toxicities such as neutropenia, which improved with supportive treatment. There were no treatment-related mortalities. GDPR might be safe and effective in elderly transplant-ineligible patients with R/R PTCL; however, further investigation is required.

罗米地辛是外周T细胞淋巴瘤(PTCL)患者的重要治疗选择。然而,罗米地平的用药时机仍存在争议。本研究旨在确定罗米地平作为吉西他滨、地塞米松和顺铂(GDP)治疗(GDPR)后巩固治疗的安全性和有效性。这项针对 2019 年 3 月至 2021 年 3 月期间接受治疗的患者的研究已在日本临床试验注册中心注册(注册号:jRCT0000000519)。如果2-4个GDP周期后确认完全应答、部分应答或病情稳定,则每4周给予一次罗米地平,持续1年。这项前瞻性研究(PTCL-GDPR)共纳入了七名复发/难治性(R/R)PTCL(T-滤泡辅助表型[n = 1]和血管免疫母细胞T细胞淋巴瘤[n = 6])患者。PTCL-GDPR患者的中位随访时间为34个月,2年总生存率为71%,治疗后总反应率为57%。PTCL-GDPR患者常见的不良反应包括中性粒细胞减少等血液学毒性反应,这些反应在接受支持性治疗后有所改善。没有出现与治疗相关的死亡病例。GDPR可能对不符合移植条件的老年R/R PTCL患者安全有效,但仍需进一步研究。
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引用次数: 0
Appropriate Treatment Intensity for Diffuse Large B-Cell Lymphoma in the Older Population: A Review of the Literature. 老年弥漫性大 B 细胞淋巴瘤的适当治疗强度:文献综述。
IF 1.1 Q4 HEMATOLOGY Pub Date : 2024-05-24 DOI: 10.3390/hematolrep16020032
Satoshi Yamasaki

Most patients with diffuse large B-cell lymphoma (DLBCL) are >65 years of age, with the number of patients expected to increase in the coming years. A comprehensive geriatric assessment that carefully evaluates fitness status and comorbidities is essential for selecting the appropriate treatment intensity. Although generally healthy patients or those <80 years of age may benefit from standard immunochemotherapy, unfit/frail patients or patients >80 years old may require reduced-intensity chemotherapy or less-toxic drugs. Some new drugs are currently being tested as single or combined agents for first-line treatment, aiming to improve the outcomes of conventional chemotherapy. This review systematically collates and discusses the outcomes associated with the use of immunochemotherapy in older patients with DLBCL, as well as considering the impact of full-dose immunochemotherapy on quality of life in older and frail patients, summarizing the rationale for reduced dosing in the older population, and presenting recommendations for selecting patients likely to benefit from reduced dosing. If preliminary efficacy and safety data are confirmed in future clinical trials, non-chemotherapy-based immunotherapy approaches could become an alternative potentially curative option in frail patients and those >80 years of age with DLBCL.

大多数弥漫大 B 细胞淋巴瘤(DLBCL)患者年龄大于 65 岁,预计未来几年患者人数还会增加。要选择适当的治疗强度,必须进行全面的老年病评估,仔细评估身体状况和合并症。虽然一般健康的患者或 80 岁的患者可能需要降低化疗强度或使用毒性较低的药物。目前,一些新药正作为单药或联合用药进行一线治疗试验,旨在改善传统化疗的疗效。本综述系统地整理和讨论了老年DLBCL患者使用免疫化疗的相关结果,并考虑了全剂量免疫化疗对老年和体弱患者生活质量的影响,总结了在老年人群中减少剂量的理由,并提出了选择可能从减少剂量中获益的患者的建议。如果初步的疗效和安全性数据在未来的临床试验中得到证实,非化疗免疫疗法可能成为体弱患者和年龄大于80岁的DLBCL患者的另一种潜在治疗选择。
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引用次数: 0
EBV-Positive Nodal T- and NK-Cell Lymphoma Mimicking Anaplastic Large Cell Lymphoma: A Case Report EBV阳性结节性T细胞和NK细胞淋巴瘤模仿无弹性大细胞淋巴瘤:病例报告
IF 0.9 Q4 Medicine Pub Date : 2024-05-23 DOI: 10.3390/hematolrep16020031
B. Abro, Pamela Allen, Saja Asakrah, Kyle Bradley, Linsheng Zhang
EBV-positive nodal T- and NK-cell lymphoma (EBV+ NT/NKCL) is a recently recognized entity in the 5th edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Notably, CD30 positivity is frequently observed in (EBV+ NT/NKCL), creating diagnostic challenges to distinguish it from ALK-negative anaplastic large cell lymphoma (ALCL). Furthermore, cases of EBV+ ALCL have been documented in the literature, predating the inclusion of EBV+ nodal cytotoxic T-cell lymphoma as a variant of peripheral T-cell lymphoma. We present a case of a 47-year-old male presenting with multiple lymphadenopathies. The histomorphologic and immunophenotypic features of the lymph node closely resemble ALK-negative ALCL, characterized by uniform CD30 expression and a subcapsular distribution of lymphoma cells. However, the lymphoma cells exhibit diffuse positivity for EBV, consistent with EBV+ NT/NKCL. A case of ALK-negative ALCL with an immunophenotype identical to the EBV-positive case is included for comparison. Given that EBV+ NT/NKCL represents an aggressive neoplasm requiring unique clinical management compared to ALK-negative ALCL, it is critical to accurately differentiate EBV+ NT/NKCL from ALK-negative ALCL with a cytotoxic T-cell immunophenotype.
EBV 阳性结节性 T- 和 NK 细胞淋巴瘤(EBV+ NT/NKCL)是最近在第五版《世界卫生组织造血和淋巴组织肿瘤分类》(WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues)中被确认的一种实体。值得注意的是,在(EBV+ NT/NKCL)中经常观察到 CD30 阳性,这给将其与 ALK 阴性的无性大细胞淋巴瘤(ALCL)区分开来带来了诊断上的挑战。此外,EBV+ ALCL 的病例在文献中也有记载,这比将 EBV+结节细胞毒性 T 细胞淋巴瘤作为外周 T 细胞淋巴瘤的一种变体要早。我们报告了一例 47 岁男性多发淋巴结病例。淋巴结的组织形态学和免疫表型特征与 ALK 阴性 ALCL 非常相似,其特点是 CD30 表达一致,淋巴瘤细胞呈囊下分布。然而,淋巴瘤细胞表现出弥漫的EBV阳性,与EBV+ NT/NKCL一致。此外,还有一例ALK阴性ALCL,其免疫表型与EBV阳性病例完全相同,以作比较。与 ALK 阴性 ALCL 相比,EBV+ NT/NKCL 是一种侵袭性肿瘤,需要独特的临床治疗,因此准确区分 EBV+ NT/NKCL 和具有细胞毒性 T 细胞免疫表型的 ALK 阴性 ALCL 至关重要。
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引用次数: 0
Complications of Brentuximab Therapy in Patients with Hodgkin’s Lymphoma and Concurrent Autoimmune Pathology—A Case Series 霍奇金淋巴瘤合并自身免疫病理患者接受布伦妥昔单抗治疗的并发症--病例系列
IF 0.9 Q4 Medicine Pub Date : 2024-05-20 DOI: 10.3390/hematolrep16020030
Oana Diana Preda, S. Badelita, I. Ursuleac, R. Irimia, Sonia Balanica, Monica Cojocaru, Cristina Cotruta, C. Dobrea, Daniel Coriu
Background: Brentuximab Vedotin (BV) has revolutionized the treatment landscape for Hodgkin’s lymphoma, yet its effects on pre-existing autoimmune disorders remain elusive. Methods: Here, we present four cases of patients with concurrent autoimmune conditions—Crohn’s disease, vitiligo, type I diabetes, and minimal change disease—undergoing BV therapy for Hodgkin’s lymphoma. The patients were treated with A-AVD instead of ABVD due to advanced-stage disease with high IPI scores. Results: Our findings reveal the surprising and complex interplay between BV exposure and autoimmune manifestations, highlighting the need for multidisciplinary collaboration in patient management. Notably, the exacerbation of autoimmune symptoms was observed in the first three cases where T-cell-mediated autoimmunity predominated. Additionally, BV exposure precipitated autoimmune thrombocytopenia in the vitiligo patient, underscoring the profound disruptions in immune regulation. Conversely, in the minimal change disease case, a disease characterized by a blend of B- and T-cell-mediated immunity, the outcome was favorable. Conclusions: This paper underscores the critical importance of vigilance toward autoimmune flare-ups induced by BV in patients with concurrent autoimmune conditions, offering insights for tailored patient care.
背景:布伦妥昔单抗韦多汀(BV)彻底改变了霍奇金淋巴瘤的治疗格局,但它对原有自身免疫性疾病的影响仍然难以捉摸。方法:在此,我们介绍了四例同时患有自身免疫性疾病--克罗恩病、白癜风、I型糖尿病和微小病变--并接受BV治疗的霍奇金淋巴瘤患者。这些患者由于疾病处于晚期且 IPI 评分较高,因此接受了 A-AVD 而非 ABVD 治疗。结果:我们的研究结果揭示了 BV 暴露与自身免疫表现之间惊人而复杂的相互作用,强调了多学科合作管理患者的必要性。值得注意的是,在前三个病例中观察到了自身免疫症状的加重,其中以 T 细胞介导的自身免疫为主。此外,白癜风患者接触 BV 会诱发自身免疫性血小板减少症,这突出表明免疫调节功能受到严重破坏。相反,在以 B 细胞和 T 细胞介导的免疫混合为特征的微小变化病例中,结果却很好。结论:本文强调了在同时患有自身免疫性疾病的患者中警惕BV诱发自身免疫性疾病发作的极端重要性,为量身定制的患者护理提供了启示。
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引用次数: 0
Anti-PF4 ELISA-Negative, SRA-Positive Heparin-Induced Thrombocytopenia 抗 PF4 酶联免疫吸附试验阴性、SRA 阳性肝素诱导的血小板减少症
IF 0.9 Q4 Medicine Pub Date : 2024-05-09 DOI: 10.3390/hematolrep16020029
A. Attah, Chelsea Peterson, Max W. Jacobs, Rama Bhagavatula, Deep Shah, Robert Kaplan, Y. Samhouri
Heparin products are frequently used in the inpatient setting to prevent and treat venous thromboembolism, but they simultaneously put patients at risk of developing heparin-induced thrombocytopenia (HIT). The 4Ts score determines the pretest probability of HIT. Diagnosis is made with a screening antiplatelet factor (PF4) immunoassay and the serotonin-release assay (SRA) as a confirmatory test. Anti-PF4 assays have high sensitivity (98%) but lower specificity (50%) and result in frequent false-positive tests. We present a rare case from our institution of a patient with anti-PF4–Polyanion ELISA-negative, SRA-positive HIT and describe the challenges in making a timely diagnosis in this case.
肝素产品常用于住院病人预防和治疗静脉血栓栓塞,但同时也会给患者带来肝素诱发血小板减少症(HIT)的风险。4Ts 评分可确定 HIT 的检测前概率。通过抗血小板因子(PF4)免疫测定筛查和血清素释放测定(SRA)确诊。抗血小板因子检测的灵敏度较高(98%),但特异性较低(50%),而且经常出现假阳性检测结果。我们介绍了本院一例罕见的抗-PF4-多聚酶联免疫吸附试验阴性、血清素释放试验阳性的 HIT 患者,并描述了及时诊断该病例所面临的挑战。
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引用次数: 0
Treatment Patterns and Clinical Outcomes of Patients with Moderate to Severe Acute Graft-Versus-Host Disease: A Multicenter Chart Review Study 中重度急性移植物抗宿主疾病患者的治疗模式和临床结果:多中心病历回顾研究
IF 0.9 Q4 Medicine Pub Date : 2024-05-06 DOI: 10.3390/hematolrep16020028
D. Michonneau, Raynier Devillier, Mikko Keränen, M. Rubio, Malin Nicklasson, H. Labussière-Wallet, Martin Carre, A. Huynh, Elisabet Viayna, Montserrat Roset, Jonathan Finzi, Minja Pfeiffer, D. Thunström, Núria Lara, Lorenzo Sabatelli, P. Chevallier, M. Itälä-Remes
Acute graft-versus-host disease (aGVHD) remains a barrier to successful allogeneic hematopoietic stem cell transplantation (HSCT) outcomes. Contemporary comprehensive analyses of real-world clinical outcomes among patients who develop aGVHD post-HSCT are needed to better understand the unmet needs of this patient population. This multicenter, retrospective chart review describes treatment patterns and clinical outcomes among patients (≥18 years old) from Finland, Sweden, and France who developed grades II–IV aGVHD after their first HSCT (January 2016–June 2017). From 13 participating centers, 151 patients were included. The median (Q1, Q3) age at HSCT was 56 (45, 62) years old. One line of aGVHD treatment was received by 47.7%, and the most common first-line treatment was methylprednisolone (alone or in a combination regimen, 74.2%; monotherapy, 25.8%). Among patients treated with methylprednisolone, 79.5% achieved a complete or partial response. The median (Q1, Q3) number of treatment lines was 2.0 (1.0, 3.0). The median (Q1, Q3) time to obtain an aGVHD diagnosis from transplant was 29.5 (21.0, 44.0) days, and 14.5 (7.0, 34.0) days to achieve the best response for 110 evaluable patients. At 6 and 12 months, 53.6% and 49.0%, respectively, achieved a complete response. Chronic GVHD occurred in 37.7% of patients, and aGVHD reoccurred in 26.5%. Following aGVHD diagnosis, mortality rates were 30.0% at 6 months and 37.3% at 12 months. Findings from this study demonstrate a continuing unmet need for new therapies that control aGVHD and improve mortality.
急性移植物抗宿主疾病(aGVHD)仍然是异基因造血干细胞移植(HSCT)成功的障碍。我们需要对造血干细胞移植后发生移植物抗宿主疾病的患者的真实临床结果进行当代全面分析,以更好地了解这一患者群体尚未得到满足的需求。这项多中心、回顾性病历回顾描述了芬兰、瑞典和法国首次造血干细胞移植后(2016 年 1 月至 2017 年 6 月)出现 II-IV 级 aGVHD 患者(≥18 岁)的治疗模式和临床结果。13个参与中心共纳入151名患者。造血干细胞移植时的中位(Q1,Q3)年龄为 56(45,62)岁。47.7%的患者接受了一线aGVHD治疗,最常见的一线治疗是甲基强的松龙(单独或联合治疗,74.2%;单药治疗,25.8%)。在接受甲基强的松龙治疗的患者中,79.5%的患者获得了完全或部分应答。治疗次数的中位数(第一季度,第三季度)为 2.0(1.0,3.0)次。在110名可评估的患者中,从移植到确诊AGVHD的中位(Q1,Q3)时间为29.5(21.0,44.0)天,达到最佳反应的中位(Q1,Q3)时间为14.5(7.0,34.0)天。在6个月和12个月时,分别有53.6%和49.0%的患者获得完全应答。37.7%的患者出现慢性GVHD,26.5%的患者再次出现AGVHD。在确诊为 aGVHD 后,6 个月和 12 个月的死亡率分别为 30.0% 和 37.3%。这项研究的结果表明,控制 aGVHD 和改善死亡率的新疗法仍有需求未得到满足。
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引用次数: 0
Current Therapeutic Sequencing in Chronic Lymphocytic Leukemia. 当前慢性淋巴细胞白血病的治疗排序。
IF 0.9 Q4 Medicine Pub Date : 2024-04-30 DOI: 10.3390/hematolrep16020027
Samir Mouhssine, Nawar Maher, Sreekar Kogila, Claudio Cerchione, Giovanni Martinelli, Gianluca Gaidano

The treatment landscape of chronic lymphocytic leukemia (CLL), the most frequent leukemia in adults, is constantly changing. CLL patients can be divided into three risk categories, based on their IGHV mutational status and the occurrence of TP53 disruption and/or complex karyotype. For the first-line treatment of low- and intermediate-risk CLL, both the BCL2 inhibitor venetoclax plus obinutuzumab and the second generation BTK inhibitors (BTKi), namely acalabrutinib and zanubrutinib, are valuable and effective options. Conversely, venetoclax-based fixed duration therapies have not shown remarkable results in high-risk CLL patients, while continuous treatment with acalabrutinib and zanubrutinib displayed favorable outcomes, similar to those obtained in TP53 wild-type patients. The development of acquired resistance to pathway inhibitors is still a clinical challenge, and the optimal treatment sequencing of relapsed/refractory CLL is not completely established. Covalent BTKi-refractory patients should be treated with venetoclax plus rituximab, whereas venetoclax-refractory CLL may be treated with second generation BTKi in the case of early relapse, while venetoclax plus rituximab might be used if late relapse has occurred. On these grounds, here we provide an overview of the current state-of-the-art therapeutic algorithms for treatment-naïve patients, as well as for relapsed/refractory disease.

慢性淋巴细胞白血病(CLL)是成人中最常见的白血病,其治疗方法也在不断变化。根据患者的 IGHV 突变状态、TP53 干扰和/或复杂核型,CLL 患者可分为三种风险类别。对于低危和中危CLL的一线治疗,BCL2抑制剂venetoclax加obinutuzumab和第二代BTK抑制剂(BTKi),即acalabrutinib和zanubrutinib,都是有价值和有效的选择。相反,以venetoclax 为基础的固定疗程疗法在高风险 CLL 患者中并未显示出显著疗效,而以 acalabrutinib 和 zanubrutinib 为基础的持续治疗则显示出良好疗效,与 TP53 野生型患者的疗效相似。对通路抑制剂产生获得性耐药性仍是一项临床挑战,复发/难治性CLL的最佳治疗顺序尚未完全确定。共价BTKi难治性患者应使用文尼考昔(venetoclax)加利妥昔单抗治疗,而文尼考昔(venetoclax)难治性CLL在早期复发时可使用第二代BTKi治疗,如果晚期复发,则可使用文尼考昔(venetoclax)加利妥昔单抗治疗。有鉴于此,我们在此概述了目前针对治疗无效患者以及复发/难治性疾病的最先进治疗算法。
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引用次数: 0
Is There an Association between a Tonsillar Diffuse Large B-Cell Lymphoma Arising after a Neck Squamous Cell Carcinoma of Occult Primary? A Case Report and Extensive Literature Review. 颈部隐匿原发鳞状细胞癌后出现扁桃体弥漫大 B 细胞淋巴瘤之间有关联吗?病例报告和广泛的文献综述。
IF 0.9 Q4 Medicine Pub Date : 2024-04-29 DOI: 10.3390/hematolrep16020026
Dimitris Tatsis, Athena Niakou, Konstantinos Paraskevopoulos, Stavroula Papadopoulou, Konstantinos Vahtsevanos

Objectives: The aim of this review is to focus on the possibility of patients with squamous cell carcinoma to develop a second primary disease such as DLBCL, perhaps because of the irradiation of the head and neck area.

Materials and methods: A case of an 89-year-old man is reported, who initially underwent surgical and complementary treatment for neck squamous cell carcinoma of occult primary and later for tonsillar diffuse large B-cell non-Hodgkin lymphoma.

Results: The second primary was considered a recurrence in the neck of the original cancer of unknown primary, so a new surgical management was decided. The final pathology report described a diffuse large B-cell non-Hodgkin lymphoma.

Conclusions: The importance of maintaining follow-ups for patients with occult primary cancers who are at an elevated risk of developing a metastasis or a second primary carcinoma outbreak is highlighted.

目的:本综述旨在关注鳞状细胞癌患者可能因头颈部受到照射而发展为第二种原发性疾病(如 DLBCL)的可能性:报告了一例 89 岁的男性患者,他最初因颈部隐匿原发鳞状细胞癌接受了手术和辅助治疗,后来又因扁桃体弥漫大 B 细胞非霍奇金淋巴瘤接受了手术和辅助治疗:结果:第二原发癌被认为是原发灶不明的颈部癌症复发,因此决定采取新的手术治疗。最终病理报告显示为弥漫大 B 细胞非霍奇金淋巴瘤:结论:对于原发癌隐匿、发生转移或爆发第二原发癌风险较高的患者,坚持随访非常重要。
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引用次数: 0
Eculizumab Treatment of Massive Hemolysis Occurring in a Rare Co-Existence of Paroxysmal Nocturnal Hemoglobinuria and Myasthenia Gravis 依库珠单抗治疗阵发性夜间血红蛋白尿和重症肌无力罕见并发症中发生的大量溶血
IF 0.9 Q4 Medicine Pub Date : 2024-04-19 DOI: 10.3390/hematolrep16020025
Ráhel Réka Bicskó, Árpád Illés, Zsuzsanna Hevessy, G. Ivády, György Kerekes, Gábor Méhes, T. Csépány, L. Gergely
The co-occurrence of myasthenia gravis (MG) and paroxysmal nocturnal hemoglobinuria (PNH) is rare; only one case has been published so far. We report a 63-year-old Caucasian female patient who was diagnosed with MG at the age of 43. Thymoma was also detected, and so it was surgically resected, which resulted in reasonable disease control for nearly 20 years. Slight hemolysis began to emerge, and then myasthenia symptoms progressed, so immunosuppressive therapy was started. Due to progressive disease and respiratory failure, the patient underwent plasmapheresis, and ventilatory support was stopped. Marked hemolysis was present, and diagnostic tests confirmed PNH with type III PNH cells. Her myasthenia symptoms aggravated, mechanical ventilation had to be started again, and due to the respiratory acidosis, massive hemolysis occurred. After two plasmapheresis sessions, the patient received eculizumab at 600 mg, resulting in prompt hemolysis control. After the second dose of the treatment, the patient was extubated. Still, due to their inability to cough, she developed another respiratory failure and pneumonia–sepsis, resulting in the patient’s death. This case highlights the rare association between these two serious diseases and similar immune-mediated pathophysiology mechanisms involving the complement system.
重症肌无力(MG)和阵发性夜间血红蛋白尿(PNH)同时出现的情况非常罕见,迄今为止只发表过一例。我们报告了一名 63 岁的白种女性患者,她在 43 岁时被诊断出患有重症肌无力。同时还发现了胸腺瘤,因此进行了手术切除,结果近 20 年来病情得到了合理控制。患者开始出现轻微溶血,随后肌无力症状进展,因此开始接受免疫抑制治疗。由于病情进展和呼吸衰竭,患者接受了浆液吸出术,并停止了呼吸机支持。患者出现了明显的溶血,诊断性检查证实其患有 III 型 PNH 细胞。她的肌无力症状加重,不得不再次开始机械通气,由于呼吸性酸中毒,发生了大量溶血。经过两次血浆置换治疗后,患者接受了 600 毫克的依库珠单抗治疗,溶血很快得到控制。第二剂治疗后,患者被拔掉了气管。但由于无法咳嗽,她再次出现呼吸衰竭和肺炎-败血症,导致患者死亡。本病例强调了这两种严重疾病之间罕见的关联,以及涉及补体系统的类似免疫介导病理生理学机制。
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引用次数: 0
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