更新于：2024-11-01

Histiocytic and Dendritic Cell Neoplasm

组织细胞和树突状细胞肿瘤

更新于：2024-11-01

基本信息

别名

Histiocytic and Dendritic Cell Neoplasm、Histiocytic and Dendritic Cell Neoplasms、Histiocytic and Dendritic Cell Tumors

+ [1]

简介

A group of rare tumors that affect the hematopoietic and lymphoid tissues. The cells of origin are the histiocytes and accessory cells. This category includes the following: Langerhans cell histiocytosis, Langerhans cell sarcoma, indeterminate dendritic cell histiocytosis, interdigitating dendritic cell sarcoma, histiocytic sarcoma, disseminated juvenile xanthogranuloma, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, ALK-positive histiocytosis, and follicular dendritic cell sarcoma.

关联

项与组织细胞和树突状细胞肿瘤相关的药物

Selinexor

塞利尼索

靶点

XPO1

作用机制

XPO1抑制剂

在研机构

Karyopharm Therapeutics, Inc. [+7]

原研机构

Karyopharm Therapeutics, Inc.

在研适应症

浆细胞骨髓瘤难治 [+69]

非在研适应症

急性早幼粒细胞白血病 [+52]

最高研发阶段批准上市

首次获批国家/地区

美国

首次获批日期2019-07-03

Luvometinib

复迈替尼

靶点

MEK1 x MEK2

作用机制

MEK1抑制剂 [+1]

在研机构

凯莱英生命科学技术（天津）有限公司 [+2]

原研机构

重庆复创医药研究有限公司

在研适应症

NF1突变型丛状神经纤维瘤 [+7]

非在研适应症

黑色素瘤 [+1]

最高研发阶段申请上市

首次获批国家/地区-

首次获批日期1800-01-20

Choline Salicylate

水杨酸胆碱

靶点-

作用机制-

在研机构

National Cancer Institute

原研机构

Mayo Clinic

在研适应症

组织细胞和树突状细胞肿瘤 [+9]

非在研适应症

弥漫性大B细胞淋巴瘤 [+1]

最高研发阶段临床1期

首次获批国家/地区-

首次获批日期1800-01-20

项与组织细胞和树突状细胞肿瘤相关的临床试验

NCT06508463 / Recruiting临床1期IIT

Phase I Trial of Systemic Administration of Vesicular Stomatitis Virus Genetically Engineered to Express NIS and Human Interferon, in Patients With Relapsed or Refractory Multiple Myeloma, Acute Myeloid Leukemia, Lymphomas, or Histiocytic/Dendritic Cell Neoplasms

This phase I trial studies the best dose and side effects of recombinant vesicular stomatitis virus (VSV) carrying the human (h) sodium iodide symporter (NIS) and Interferon (IFN) beta (β) genes (VSV-hIFNβ-NIS) in combination with ipilimumab and cemiplimab in patients with T-cell lymphoma. A virus, called VSV-hIFNβ-NIS, which has been changed in a certain way, may be able to kill cancer cells without damaging normal cells. Immunotherapy with ipilmumab and cemiplimab may induce changes in body's immune system and may interfere with the ability of tumor cells to grow and spread.

开始日期2024-01-05

申办/合作机构

Mayo Clinic

[+1]

NCT04640779 / Recruiting临床1期IIT

Phase Ib Trial of Low-Dose Selinexor (KPT-330) in Combination with Choline Salicylate (CS) for the Treatment of Patients with Non-Hodgkin Lymphoma (NHL), Hodgkin Lymphoma, Histiocytic/Dendritic Cell Neoplasms, or Multiple Myeloma

This phase Ib trial evaluates the side effects and best dose of choline salicylate given together with a low dose of selinexor in treating patients with non-Hodgkin or Hodgkin lymphoma, or multiple myeloma whose prior treatment did not help their cancer (refractory) or for patients with histiocytic/dendritic cell neoplasm. Anti-inflammatory drugs, such as choline salicylate lower the body's immune response and are used with other drugs in the treatment of some types of cancer. Selinexor may stop the growth of cancer cells by blocking a protein called CRM1 that is needed for cell growth. This trial may help doctors learn more about selinexor and choline salicylate as a treatment for with non-Hodgkin or Hodgkin lymphoma, histiocytic/dendritic cell neoplasm, multiple myeloma.

开始日期2021-02-08

申办/合作机构

Mayo Clinic

[+1]

NCT03017820 / Recruiting临床1期IIT

This phase I trial studies the best dose and side effects of recombinant vesicular stomatitis virus carrying the human NIS and IFN beta genes (VSV-hIFNbeta-sodium iodide symporter [NIS]) with or without cyclophosphamide or ipilimumab and nivolumab or cemiplimab in treating patients with multiple myeloma, acute myeloid leukemia (AML) or lymphoma that has come back or does not respond to treatment. A virus, called VSV-hIFNbeta-NIS, which has been changed in a certain way, may be able to kill cancer cells without damaging normal cells. Cyclophosphamide is in a class of medications called alkylating agents. It works by damaging the cell's DNA and may kill cancer cells. It may also lower the body's immune response. Immunotherapy with ipilmumab and nivolumab or cemiplimab may induce changes in body's immune system and may interfere with the ability of tumor cells to grow and spread. Giving VSV-hIFNbeta-NIS and ruxolitinib phosphate may work better at treating multiple myeloma, acute myeloid leukemia and T-cell lymphoma.

开始日期2017-04-04

申办/合作机构

Mayo Clinic

[+1]

100 项与组织细胞和树突状细胞肿瘤相关的临床结果

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100 项与组织细胞和树突状细胞肿瘤相关的转化医学

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0 项与组织细胞和树突状细胞肿瘤相关的专利（医药）

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项与组织细胞和树突状细胞肿瘤相关的文献（医药）

2024-01-01·Modern Pathology

Cutaneous Manifestations of Myeloid Neoplasms Exhibit Broad and Divergent Morphologic and Immunophenotypic Features but Share Ancestral Clonal Mutations With Bone Marrow

Article

作者: Luskin, Marlise R ; Kim, Annette S ; Rodig, Scott J ; Pinkus, Geraldine S ; Shanmugam, Vignesh ; Lane, Andrew A ; Pozdnyakova, Olga ; Garcia, Jacqueline S ; Zemmour, David ; Lucas, Fabienne ; Hergott, Christopher B ; Sadigh, Sam ; Morgan, Elizabeth A ; Tsai, Harrison K ; Winer, Eric S ; DeAngelo, Daniel J ; Lovitch, Scott B ; Hasserjian, Robert P

In this study, we performed a comprehensive molecular analysis of paired skin and peripheral blood/bone marrow (BM) samples from 17 patients with cutaneous myeloid or cutaneous histiocytic-dendritic neoplasms. The cutaneous manifestations included 10 patients with cutaneous acute myeloid leukemia (c-AML), 2 patients with full or partial Langerhans cell differentiation, 2 patients with blastic plasmacytoid dendritic cell neoplasms (BPDCN), 1 patient with both Langerhans cell differentiation and BPDCN, and 2 patients with full or partial indeterminate dendritic cell differentiation. Seven of the 10 c-AML patients (70%) exhibited concurrent or subsequent marrow involvement by acute myeloid leukemia, with all 7 cases (100%) demonstrating shared clonal mutations in both the skin and BM. However, clonal relatedness was documented in one additional case that never had any BM involvement. Nevertheless, NPM1 mutations were identified in 7 of the 10 (70%) of these c-AML cases while one had KMT2A rearrangement and one showed inv(16). All 3 patients (100%) with Langerhans cell neoplasms, 2 patients with BPDCN (100%), and one of the 2 patients (50%) with other cutaneous dendritic cell neoplasms also demonstrated shared mutations between the skin and concurrent or subsequent myeloid neoplasms. Both BM and c-AML shared identical founding drivers, with a predominance of NPM1, DNMT3A, and translocations associated with monocytic differentiation, with common cutaneous-only mutations involving genes in the signal transduction and epigenetic pathways. Cutaneous histiocytic-dendritic neoplasms shared founding drivers in ASXL1, TET2, and/or SRSF2. However, in the Langerhans cell histiocytosis or histiocytic sarcoma cases, there exist recurrent secondary RAS pathway hits, whereas cutaneous BPDCN cases exhibit copy number or structural variants. These results enrich and broaden our understanding of clonally related cutaneous manifestations of myeloid neoplasms and further illuminate the highly diverse spectrum of morphologic and immunophenotypic features they exhibit.

2019-09-01·Surgical Pathology Clinics

Histiocytic and Dendritic Cell Neoplasms

Review

作者: Pan, Zenggang ; Xu, Mina L

Histiocytic and dendritic cell neoplasms are very rare, belonging to a group that share morphologic, immunophenotypic, and ultrastructural characteristics of mature histiocytic/dendritic neoplasms. Histiocytic and dendritic cell neoplasms may arise de novo or in association with B-cell, T-cell, or myeloid neoplasms. Recent molecular findings, particularly the discoveries of the mutations in the RAS-RAF-MEK-ERK pathway, have greatly advanced the diagnosis and treatment options. Histiocytic and dendritic cell neoplasms may closely resemble each other, non-hematopoietic neoplasms, and even reactive processes. Therefore, it is essential to understand the clinicopathologic characteristics, differential diagnoses, and pitfalls of each entity.

2018-11-01·Annals of Hematology3区 · 医学

Histiocytic cell neoplasms involving the bone marrow: summary of the workshop cases submitted to the 18th Meeting of the European Association for Haematopathology (EAHP) organized by the European Bone Marrow Working Group, Basel 2016

3区 · 医学

Article

作者: Kremer, Markus ; Tzankov, Alexandar ; Gianelli, Umberto ; Leguit, Roos ; van der Walt, Jon ; Orazi, Attilio ; Hebeda, Konnie M

The bone marrow is a preferential site for both reactive and neoplastic histiocytic proliferations. The differential diagnosis ranges from reactive histiocyte hyperplasia in systemic infections, vaccinations, storage diseases, post myeloablative therapy, due to increased cell turnover, and in hemophagocytic lymphohistiocytosis, through extranodal Rosai-Dorfman disease to neoplasms derived from histiocytes, including histiocytic sarcomas (HS), Langerhans cell histiocytoses (LCH), Erdheim-Chester disease (ECD), and disseminated juvenile xanthogranuloma (JXG). One of the most important recent developments in understanding the biology of histiocytic neoplasms and in contributing to diagnosis was the detection of recurrent mutations of genes of the Ras/Raf/MEK/ERK signaling pathway, in particular the BRAF^V600E mutation, in LCH and ECD. Here, we summarize clinical and pathological findings of 17 histiocytic neoplasms that were presented during the bone marrow symposium and workshop of the 18th European Association for Haematopathology (EAHP) meeting held in Basel, Switzerland, in 2016. A substantial proportion of these histiocytic neoplasms was combined with clonally related lymphoid (n = 2) or myeloid diseases (n = 5, all ECD). Based on the latter observation, we suggest excluding co-existent myeloid neoplasms at initial staging of elderly ECD patients. The recurrent nature of Ras/Raf/MEK/ERK signaling pathway mutations in histiocytic neoplasms was confirmed in 6 of the 17 workshop cases, illustrating their diagnostic significance and suggesting apotential target for tailored treatments.