Hematopathology Diagnosis

皮塔卢加博士和我在反应性和肿瘤性淋巴增殖性疾病、免疫缺陷状态和各种血液恶性肿瘤的诊断和分类方面提供帮助。我们为NCI的医生以及其他研究所的研究血液淋巴疾病患者的医生提供咨询和协作服务，特别是NIAID、NHLBI、NHGRI和NIAMSD。我们定期出席由NCI和其他研究所(NIAID、NHLBI和NHGRI)的临床分支机构主办的会议。我们每年都会收到2000多个咨询案例。我们试图将协商限制在困难或具有挑战性的案件上，不接受“例行公事的第二意见”的案件。很大一部分(约20%)的案例是由其他学术机构基于诊断的不确定性或由于几个机构之间的意见分歧而提交的。在具有挑战性的诊断问题上，我们往往是最终仲裁者。此外，我们在临床实践的基础上进行了新的观察，并根据最初咨询后审查的病例材料发表了一些出版物。我们的临床咨询实践还与NIH的其他临床小组协同工作，加强了对NIH临床方案的转介。我们的工作改进了几种罕见疾病的诊断和分类，包括多中心Castleman病、Erdheim-Chester病、自身免疫淋巴增殖性综合征、KSHV和EBV相关的增殖，以及罕见的组织细胞疾病，如Rosai-Dorfman病。Castleman病的研究涉及大型的多中心合作努力，我们在研究的设计和实施中都发挥了重要作用。作为专家审查小组的成员，我审查提交给登记处的所有病例，以验证诊断，并与生物学和翻译研究相关联。另一个有争议和困难的领域是淋巴增生性疾病和淋巴瘤的诊断和分类，这些疾病与各种形式的免疫缺陷有关，包括先天性、移植后、其他医源性原因，以及继发于艾滋病毒的获得性疾病，或高龄伴随相关的免疫衰老。我们在《血液》杂志上发表了一篇透视文章，讨论了与免疫缺陷相关的淋巴增生性疾病分类的关键问题。我还在一个专家评审小组任职，以验证Burkitt淋巴瘤的诊断，以便对这种肿瘤进行全面的TCGA分析。我是世卫组织皮肤肿瘤分类蓝皮书的主要撰稿人，一篇综述皮肤淋巴瘤分类最新进展的综述文章发表在《血液》杂志上。文献中发表的一些报告强调了与非典型树突状细胞增殖、血管内大B细胞淋巴瘤和滤泡性淋巴瘤的组织学进展有关的诊断问题。

Dr. Pittaluga and I provide assistance in the diagnosis and classification of reactive and neoplastic lymphoproliferative disorders, immunodeficiency states, and diverse hematological malignancies. We provide consultative and collaborative services to physicians in the NCI, as well as to physicians studying patients with hematolymphoid disorders in other institutes, in particular NIAID, NHLBI, NHGRI, and NIAMSD. We regularly present at conferences sponsored by clinical branches in the NCI and other Institutes (NIAID, NHLBI, and NHGRI.) We receive more than 2000 cases in consultation each year. We try to restrict consultations to difficult or challenging cases, and do not accept cases for "routine second opinions". A significant proportion (approximately 20%) of the cases are submitted by other academic institutions based on diagnostic uncertainty, or because of differences of opinion among several institutions. We are often the final arbiter on challenging diagnostic problems. Additionally, we have made novel observations based on our clinical practice, and a number of publications have emanated from case material originally reviewed in consultation. Our clinical consultation practice also synergizes with other NIH clinical groups, enhancing referral to NIH clinical protocols. Our work has led to improvement in the diagnosis and classification of several rare diseases including multicentric Castleman disease, Erdheim-Chester disease, autoimmune lymphoproliferative syndrome, KSHV- and EBV-associated proliferations, and rare histiocytic disorders, such as Rosai-Dorfman disease. The studies of Castleman disease involve large multi-center collaborative efforts, in which we play a major role, both in the design and conduct of the studies. As a member of an expert review panel, I review all cases submitted to a registry, to validate the diagnosis, and correlate with biological and translational studies. Another controversial and difficult area is the diagnosis and classification of lymphoproliferative disorders and lymphomas associated with diverse forms of immunodeficiency, including congenital, post-transplant, other iatrogenic causes, and acquired secondary to HIV, or advanced age with associated immune senescence. We published a Perspective article in Blood, discussing key issues related to the classification of lymphoproliferative disorders associated with immune deficiencies. I also serve on an expert review panel to validate the diagnosis of Burkitt lymphoma for a comprehensive TCGA analysis of this tumor. I was a key contributor to the WHO Bluebook on the classification of cutaneous neoplasms, and a review article summarizing updates to the classification of cutaneous lymphomas was published in Blood. Several reports published in the literature highlighted diagnostic problems related to atypical dendritic cell proliferations, intravascular large B-cell lymphoma, and histological progression of follicular lymphoma.