Case Reports in Hematology最新文献

英文中文

Systemic Relapse in a Young Adult Patient with Primary CNS Diffuse Large B-Cell Lymphoma. 一名原发性中枢神经系统弥漫大 B 细胞淋巴瘤年轻成人患者的全身复发

IF 0.7 Q4 HEMATOLOGY

Case Reports in Hematology

Pub Date : 2022-06-21 eCollection Date: 2022-01-01 DOI: 10.1155/2022/7139661

Adam Khorasanchi, Zachary Benson, Misty Hall, Nelya Ebadirad, Mohammad H Gharavi, Patrick Willard, Miranda Chimzar, John McKay, Gary Simmons, Victor Yazbeck

Primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL) is a rare form of non-Hodgkin's lymphoma, characterized by an aggressive disease course. While CNS relapse is common, systemic relapse is rare with no consensus on optimal treatment. This paper presents an unusual case of advanced PCNS-DLBCL with systemic relapse, including adrenal gland involvement. A review of the existing literature and a discussion on the management of systemic relapse in PCNS-DLBCL is also provided.

原发性中枢神经系统弥漫大 B 细胞淋巴瘤（PCNS-DLBCL）是一种罕见的非霍奇金淋巴瘤，病程凶险。中枢神经系统复发很常见，但全身复发却很罕见，而且最佳治疗方法尚未达成共识。本文介绍了一例罕见的晚期 PCNS-DLBCL 全身复发病例，包括肾上腺受累。本文还回顾了现有文献，并讨论了 PCNS-DLBCL 全身复发的治疗方法。

引用次数: 0

An Unusual Case of Breast Implant-Associated Anaplastic Large Cell Lymphoma. 乳房假体相关间变性大细胞淋巴瘤1例。

IF 0.7

Case Reports in Hematology

Pub Date : 2022-06-09 eCollection Date: 2022-01-01 DOI: 10.1155/2022/4700787

Sarah Premji, Andreia Barbieri, Christine Roth, Eric M Rohren, Gustavo Rivero, Sravanti P Teegavarapu

Introduction: Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare disease entity associated with textured breast implants. Though the clinical course is typically indolent, BIA-ALCL can occasionally invade through the capsule into the breast parenchyma with spread to the regional lymph nodes and beyond including chest wall invasive disease.

Case: We present the case of a 51-year-old female with a history of bilateral silicone breast implants placed approximately twenty years ago who presented with two months of progressively enlarging right breast mass. Ultrasound-guided biopsy of right breast mass and right axillary lymph node showed CD 30-positive ALK-negative anaplastic large cell lymphoma, and staging work up showed extension of the tumor to chest wall and ribs consistent with advanced disease. She received CHP-BV (cyclophosphamide, doxorubicin, prednisone, and brentuximab vedotin) for six cycles with complete metabolic response. This was followed by extensive surgical extirpation and reconstruction, radiation for residual disease and consolidation with autologous stem cell transplant. She is currently on maintenance brentuximab vedotin with no evidence of active disease post autologous stem cell transplant.

Conclusion: Treatment guidelines for advanced chest wall invasive BIA-ALCL are not well defined. Lack of predictive factors warrants mutation analysis and genetic sequencing to identify those at highest risk of progression to chest wall invasive disease. This rare case highlights the need for definitive consensus on the optimal management of chest wall invasive BIA-ALCL.

简介:乳房植入物相关间变性大细胞淋巴瘤(BIA-ALCL)是一种罕见的与有纹理的乳房植入物相关的疾病。虽然临床过程通常是惰性的，但BIA-ALCL偶尔可以通过被膜侵入乳腺实质并扩散到区域淋巴结并超越胸壁侵袭性疾病。病例:我们提出的情况下，51岁的女性与双侧硅胶乳房植入史放置大约20年前，谁提出了两个月的逐步扩大的右乳房肿块。超声引导下右乳腺肿块及右腋窝淋巴结活检显示cd30阳性alk阴性间变性大细胞淋巴瘤，分期显示肿瘤向胸壁和肋骨延伸，符合疾病晚期。她接受了CHP-BV(环磷酰胺、阿霉素、强的松和布伦妥昔单抗韦多汀)治疗6个周期，代谢完全缓解。随后是广泛的手术切除和重建，放射治疗残余疾病和自体干细胞移植巩固。她目前正在接受布伦妥昔单抗维多汀维持治疗，自体干细胞移植后无活动性疾病迹象。结论:晚期胸壁浸润性BIA-ALCL的治疗指南尚不明确。由于缺乏预测因素，需要进行突变分析和基因测序，以确定进展为胸壁侵袭性疾病的最高风险。这个罕见的病例强调了对胸壁浸润性BIA-ALCL的最佳治疗需要明确的共识。

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引用次数: 0

Tibial Plasmablastic Lymphoma in a HIV-Negative Child: A Novel Extraoral Localisation. hiv阴性儿童胫骨浆母细胞淋巴瘤:一种新的口外定位。

IF 0.7

Case Reports in Hematology

Pub Date : 2022-06-08 eCollection Date: 2022-01-01 DOI: 10.1155/2022/4353830

Ali Mala, Yameena Noman Khan, Akbar Ahmed, Naema Khayyam, Kanwal Aftab

Plasmablastic lymphoma is an aggressive, high-grade non-Hodgkin lymphoma predominantly seen in HIV-infected individuals. Alongside a strong correlation with HIV, PBL can manifest in immunocompromised HIV-negative patients. A rare case of PBL in an immunocompetent and otherwise healthy child presented to Indus Hospital & Health Network (IHHN), Karachi, Pakistan. The patient had complaints of swelling and pain in the right leg and was referred from a city in Interior Sindh. Histopathological analysis revealed sheets and aggregates of neoplasm replacing bone marrow interspersed with sclerotic bony fragments. Large, monomorphic, multinucleated neoplastic cells containing abundant cytoplasm and scattered pleomorphic cells were also noted, leading to the diagnosis of tibial plasmablastic lymphoma. A FAB/LMB96 group C chemotherapy regimen for aggressive and high-risk cancer was administered with a marked improvement in clinical symptoms.

浆母细胞淋巴瘤是一种侵袭性高级别非霍奇金淋巴瘤，主要见于hiv感染者。除了与HIV有很强的相关性外，PBL还可以在免疫功能低下的HIV阴性患者中表现出来。巴基斯坦卡拉奇的印度河医院和健康网络(IHHN)报道了一名免疫功能正常且其他方面健康的儿童患PBL的罕见病例。患者有右腿肿胀和疼痛的主诉，从信德省一个城市转诊过来。组织病理学分析显示肿瘤片和聚集体取代骨髓，穿插硬化骨碎片。大的，单形的，多核的肿瘤细胞含有丰富的细胞质和分散的多形性细胞，也被注意到，导致胫骨浆母细胞淋巴瘤的诊断。采用FAB/LMB96 C组化疗方案治疗侵袭性和高危癌症，临床症状明显改善。

引用次数: 1

A Case Report of Premalignant Plasma Cell Dyscrasia-Induced Renal Failure in a 31-Year-Old Female 31岁女性癌前浆细胞病变致肾功能衰竭1例报告

IF 0.7

Case Reports in Hematology

Pub Date : 2022-06-07 DOI: 10.1155/2022/2497380

Ayrton I Bangolo, MA Akhter, Amer Jarri, Manpreet Kaur, Ali Atoot, Parul Jandir, Mahmood Ibrahim, Lochana Manandhar, A. Atoot

Monoclonal gammopathy of renal significance (MGRS) is a rare disorder in which monoclonal immunoglobulin secreted by nonmalignant B cell or plasma cell clone causes kidney damage. Although MGRS is a premalignant condition, it can cause severe kidney disease and end-stage renal disease (ESRD) at any age. Herein, we present a 31-year-old female with past medical history of lupus nephritis who presented with signs of volume overload and worsening renal function despite adequate immunosuppressive therapy. Renal biopsy revealed heavy and light chain deposition consistent with MGRS. This case report demonstrates the importance of including MGRS in the differential diagnosis of worsening renal function despite adequate treatment, raising awareness of this premalignant yet morbid condition.

单克隆肾性伽玛病(MGRS)是一种罕见的疾病，由非恶性B细胞或浆细胞克隆分泌的单克隆免疫球蛋白引起肾脏损害。虽然MGRS是一种恶性前病变，但它可以在任何年龄引起严重的肾脏疾病和终末期肾脏疾病(ESRD)。在此，我们报告一位31岁的女性，既往有狼疮肾炎病史，尽管接受了适当的免疫抑制治疗，但仍表现出容量超载和肾功能恶化的迹象。肾活检显示轻重链沉积与核磁共振一致。本病例报告表明，在充分治疗后肾功能恶化的鉴别诊断中，包括MGRS的重要性，提高了对这种癌前病变的认识。

引用次数: 1

Transient Complete Recovery of Chronic Refractory Idiopathic Thrombocytopenic Purpura after Treatment with Monoclonal Antibody Targeting SARS-CoV-2 Spike Protein 靶向SARS-CoV-2刺突蛋白单克隆抗体治疗慢性难治性特发性血小板减少性紫癜后的短暂完全恢复

IF 0.7

Case Reports in Hematology

Pub Date : 2022-06-07 DOI: 10.1155/2022/8335541

Pooja Gogia, Yiqing Xu

Idiopathic thrombocytopenic purpura (ITP), also known as immune thrombocytopenic purpura, is an immune-mediated acquired disease characterized by transient or persistent decrease of the platelet count due to autoimmune-related destruction of platelets. Therapy for ITP relies on competing and inhibiting the autoantibody binding and destruction (intravenous immunoglobulin and anti-D immunoglobulin and spleen tyrosine kinase (Syk) inhibitor fostamatinib), augmenting platelet production (thrombopoietin receptor agonists), immunosuppression to reduce the autoantibody production, as well as splenectomy. Studies on autoantigens on the platelets suggested epitopes to be located predominantly on the GP IIb/IIIa receptor or integrin αIIbβ3, though the trigger for the development of ITP is unclear. We report a case here of a 37-year-old gentleman who has chronic ITP managed on eltrombopag, who after receiving monoclonal antibody against SARS-CoV-2 (mAb) i.e. casirivimab and imdevimab for his COVID-19 infection, demonstrated complete recovery of his platelet count for a short period of time. We discuss a few potential mechanisms of action and propose further studies to elucidate the therapeutic effect of COVID-19 mAb in ITP.

特发性血小板减少性紫癜(ITP)，也称为免疫性血小板减少性紫癜，是一种免疫介导的获得性疾病，其特征是由于自身免疫相关的血小板破坏导致血小板计数短暂或持续减少。ITP的治疗依赖于竞争和抑制自身抗体结合和破坏(静脉注射免疫球蛋白和抗d免疫球蛋白和脾酪氨酸激酶(Syk)抑制剂fostamatinib)，增加血小板产生(血小板生成素受体激动剂)，免疫抑制以减少自身抗体产生，以及脾切除术。对血小板自身抗原的研究表明，表位主要位于GP IIb/IIIa受体或整合素αIIbβ3上，但ITP发生的触发因素尚不清楚。我们在此报告了一例37岁的慢性ITP患者，他接受了针对SARS-CoV-2的单克隆抗体(mAb)，即卡西维单抗和imdevimab治疗他的COVID-19感染后，他的血小板计数在短时间内完全恢复。我们讨论了几种潜在的作用机制，并提出进一步的研究来阐明COVID-19单抗对ITP的治疗作用。

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引用次数: 0

A Rare Case of Extensive Cerebral Venous Sinus Thrombosis Complicated by Heparin-Induced Thrombocytopenia 广泛脑静脉窦血栓形成并发肝素性血小板减少症1例

IF 0.7

Case Reports in Hematology

Pub Date : 2022-06-06 DOI: 10.1155/2022/7845786

Noman Ahmed Jang Khan, A. Farooqi, Mohamed Alsharedi

Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening cause of stroke. Several risk factors have been identified including hypercoagulable state, malignancy, use of oral contraceptives, pregnancy, head injury, infection, and prothrombotic states such as heparin-induced thrombocytopenia (HIT). HIT is a prothrombotic state leading to thrombosis in several distinct locations including CVST requiring prompt discontinuation of heparin and initiation of nonheparin anticoagulation to prevent catastrophic consequences. Very rarely, HIT can complicate the ongoing CVST leading to worsening thrombosis and clinical deterioration. We here report an exceedingly rare case of CVST complicated by HIT in a 22-year-old female patient who showed remarkable clinical improvement after discontinuation of heparin and initiation of argatroban.

脑静脉窦血栓形成(CVST)是一种罕见但可能危及生命的中风原因。已确定的几个危险因素包括高凝状态、恶性肿瘤、口服避孕药的使用、妊娠、头部损伤、感染和血栓前状态，如肝素诱导的血小板减少症(HIT)。HIT是一种导致包括CVST在内的几个不同部位血栓形成的血栓形成前状态，需要立即停用肝素并开始使用非肝素抗凝以防止灾难性后果。在极少数情况下，HIT会使正在进行的CVST复杂化，导致血栓形成恶化和临床恶化。我们在此报告一例极其罕见的CVST合并HIT的病例，患者为22岁的女性，在停用肝素并开始使用阿加曲班后表现出显著的临床改善。

引用次数: 1

Spontaneous Regression of Plasmablastic Lymphoma in an Immunocompetent Patient: Case Report and Review of the Literature 免疫功能正常患者浆母细胞淋巴瘤的自发消退:病例报告和文献回顾

IF 0.7

Case Reports in Hematology

Pub Date : 2022-05-30 DOI: 10.1155/2022/1142049

Kee Tat Lee, Nurul Akmar Misron, N. Abdul Aziz, Chin Hau Wong, H. Liew

Plasmablastic lymphoma (PBL) is a rare and highly aggressive type of lymphoma, which is commonly associated with human immunodeficiency virus (HIV) infection. Spontaneous regression of aggressive lymphomas is rare as they typically require administration of chemotherapy and radiotherapy for treatment. Here, we describe a case of a spontaneous regression of PBL after nasal biopsy and computed tomography (CT) guided biopsy of paravertebral mass in an immunocompetent patient. We postulate that the patient's immune system may be activated as a result of the stress and physical trauma brought on by nasal and paravertebral mass biopsy. Our case highlights the rare phenomenon of spontaneous regression of lymphoma which needs to be further studied on to establish its underlying pathophysiology.

浆母细胞淋巴瘤(PBL)是一种罕见的高侵袭性淋巴瘤，通常与人类免疫缺陷病毒(HIV)感染有关。侵袭性淋巴瘤的自发消退是罕见的，因为他们通常需要给予化疗和放疗的治疗。在这里，我们描述了一个免疫功能正常的患者，在鼻活检和计算机断层扫描(CT)引导下椎旁肿块活检后，PBL自发消退的病例。我们假设患者的免疫系统可能是由于鼻和椎旁肿块活检带来的压力和身体创伤而激活的。我们的病例强调了淋巴瘤自发消退的罕见现象，需要进一步研究以确定其潜在的病理生理。

引用次数: 0

Monoclonal Gammopathy of Renal Significance with Progression to Multiple Myeloma in a Patient with ASIA-MO Syndrome 亚洲-莫综合征患者进展为多发性骨髓瘤时肾脏单克隆γ病变的意义

IF 0.7

Case Reports in Hematology

Pub Date : 2022-05-24 DOI: 10.1155/2022/8571536

David Alejandro Vargas Gutiérrez, K. I. Arias Callejas, Edwin Pavel Palacios Ruiz, Priscila Joseline Pérez Vinueza, Juan Diego Muñoz Vega, Ana Karen G. Mejía Geraldo, Ingrid Salinas Zaldívar

Background Autoimmune/inflammatory syndrome induced by adjuvants is a disease associated with an unregulated hyperactivity of the immune system and may also be associated with a high frequency of hematologic malignancies. Report. This is a case of a female with ASIA-MO syndrome secondary to infiltration of mineral oil for aesthetic purposes and presented with multiple episodes of urolithiasis resulting in renal impairment of her left kidney confirmed by scintigraphy and ending in unilateral nephrectomy. Retrospective renal piece analysis confirmed tubulointerstitial infiltration with light chains and plasma cells. Paraffin fixation prevented subsequent immunofluorescence analysis for better follow-up of the patient. Conclusion The presence of positive immunofixation kappa chains explained the sudden deterioration of renal function with monoclonal gammopathy of renal significance which concluded in an association between diseases, such as multiple light chain myeloma, as a final diagnosis.

背景:佐剂诱导的自身免疫/炎症综合征是一种与免疫系统不受调节的过度活跃相关的疾病，也可能与血液系统恶性肿瘤的高频率有关。报告。这是一例女性ASIA-MO综合征，继发于审美目的的矿物油浸润，并表现为多次尿石症发作，导致左肾肾损害，经显像证实，最终行单侧肾切除术。回顾性肾片分析证实小管间质浸润伴轻链和浆细胞。石蜡固定阻止了后续的免疫荧光分析，以更好地随访患者。结论免疫固定阳性kappa链的存在解释了肾脏意义的单克隆γ病的肾功能突然恶化，这与疾病(如多发性轻链骨髓瘤)的最终诊断有关。

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引用次数: 0

Pancytopenia with Development of Persistent Neutropenia Secondary to COVID-19 全血细胞减少伴新冠肺炎继发持续性中性粒细胞减少

IF 0.7

Case Reports in Hematology

Pub Date : 2022-05-11 DOI: 10.1155/2022/8739295

Kathie Wu, Yvonne Dansoa, Priyanka Pathak

Viral infections have long been linked to hematologic dysfunction. With the rapid spread of COVID-19, various hematologic manifestations have emerged. While there have been several reports of immune thrombocytopenic purpura from SARS-CoV-2, concurrent lymphopenia and anemia have sparse. We describe a case of COVID-induced pancytopenia that presented months after initial COVID infection that initially responded to IVIG and steroids, but now with persistent neutropenia.

长期以来，人们一直认为病毒感染与血液功能障碍有关。随着新冠肺炎疫情的快速传播，出现了多种血液学表现。虽然有几例由SARS-CoV-2引起的免疫性血小板减少性紫癜的报道，但并发淋巴细胞减少和贫血的报道很少。我们描述了一例COVID诱导的全血细胞减少症，该病例在最初感染COVID后几个月出现，最初对IVIG和类固醇有反应，但现在出现了持续性中性粒细胞减少症。

引用次数: 3

Diagnosis and Management of Atypical Chronic Myeloid Leukemia with a t(2;13)(q33;q12) Translocation t(2;13)(q33;q12)易位的非典型慢性髓系白血病的诊断和治疗

IF 0.7

Case Reports in Hematology

Pub Date : 2022-05-04 DOI: 10.1155/2022/4628183

John S. Wang, O. Elghawy, Brett Kurpiel, Michael G. Douvas

Atypical chronic myeloid leukemia (aCML) is a rare myeloproliferative disorder that shares clinical features with chronic myeloid leukemia but lacks the classic t(9;22) BCR-ABL1 translocation and features prominent dysgranulopoiesis and granulocytic dysplasia. Challenges of this diagnosis include clinical and biologic heterogeneity, the high risk of transformation to acute myeloid leukemia, and the lack of standard treatment options. Allogeneic hematopoietic stem cell transplant is likely the preferred treatment, but this can be limited by patient psychosocial support, age, concomitant medical conditions, and availability of an appropriate donor. We report the case of a 61-year-old male with no significant past medical history diagnosed with aCML with a rare t(2;13)(q33;q12). He presented with weight loss, night sweats, splenomegaly, hyperleukocytosis, a leukoerythroblastic differential with a predominant neutrophilia, anemia, and thrombocytopenia. Subsequent peripheral blood and bone marrow studies lead to the diagnosis of aCML. He was recommended to undergo an allogeneic stem cell transplant evaluation and declined. He was initially treated with hydroxyurea and imatinib to which he responded for approximately three years. After clinical progression, he was treated with sorafenib, a multiprotein kinase inhibitor more commonly used in the treatment of hepatocellular and renal cell carcinoma due to its off target FLT3 inhibition. The patient achieved complete hematologic response which has been sustained for 7 years with tolerable side effects.

非典型慢性髓系白血病(aCML)是一种罕见的骨髓增生性疾病，与慢性髓系白血病具有相同的临床特征，但缺乏经典的t(9;22) BCR-ABL1易位，并具有明显的粒细胞生成异常和粒细胞发育不良。这种诊断的挑战包括临床和生物学的异质性，转化为急性髓性白血病的高风险，以及缺乏标准的治疗选择。同种异体造血干细胞移植可能是首选的治疗方法，但这可能受到患者心理社会支持、年龄、伴随医疗条件和合适供体的可用性的限制。我们报告一例61岁男性，无明显既往病史，诊断为aCML伴罕见t(2;13)(q33;q12)。患者表现为体重减轻、盗汗、脾肿大、白细胞增多、白细胞增生与嗜中性粒细胞增多、贫血和血小板减少。随后的外周血和骨髓检查导致aCML的诊断。医生建议他接受同种异体干细胞移植评估，但他拒绝了。他最初接受羟基脲和伊马替尼治疗，大约3年有反应。临床进展后，他接受索拉非尼治疗，索拉非尼是一种多蛋白激酶抑制剂，由于其脱靶FLT3抑制，更常用于治疗肝细胞癌和肾癌。患者获得了完全的血液学反应，持续了7年，副作用可耐受。

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