scholarly journals Recommendations for Managing Adults With Histiocytic Neoplasms: New NCCN Guidelines

2021 ◽  
Vol 19 (11.5) ◽  
pp. 1355-1357
Author(s):  
Ronald S. Go
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Clinical Suspicion ◽  
Evidence Based ◽  
Malignant Histiocytosis ◽  
Histologic Diagnosis ◽  
Nccn Guidelines ◽  
Cell Lineages ◽  
Rosai Dorfman Disease ◽  
Erdheim Chester

The histiocytoses, a group of clonal and reactive conditions, arise from monocytic macrophage or dendritic cell lineages. The current NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Histiocytic Neoplasms reflect the most up-to-date, evidence-based data relating to the evaluation and management of this disease. Specifically, the guidelines focus on adult Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Because these disorders are rare, challenges have arisen regarding clinical suspicion, histologic diagnosis, treatment, and molecular subtyping. Future versions of the NCCN Guidelines will address the diagnosis and management of pediatric patients, as well as malignant histiocytosis.

scholarly journals Histiocytic Neoplasms, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

2021 ◽  
Vol 19 (11) ◽  
pp. 1277-1303
Author(s):  
Ronald S. Go ◽  
Eric Jacobsen ◽  
Robert Baiocchi ◽  
Ilia Buhtoiarov ◽  
Erin B. Butler ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Optimal Management ◽  
Watch And Wait ◽  
Hematologic Disorders ◽  
Nccn Guidelines ◽  
Mild Disease ◽  
Rosai Dorfman Disease ◽  
Erdheim Chester ◽  
Chester Disease

Histiocytic neoplasms are rare hematologic disorders accounting for less than 1% of cancers of the soft tissue and lymph nodes. Clinical presentation and prognosis of these disorders can be highly variable, leading to challenges for diagnosis and optimal management of these patients. Treatment often consists of systemic therapy, and recent studies support use of targeted therapies for patients with these disorders. Observation (“watch and wait”) may be sufficient for select patients with mild disease. These NCCN Guidelines for Histiocytic Neoplasms include recommendations for diagnosis and treatment of adults with the most common histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease.

Fulminant Multisystem Non–Langerhans Cell Histiocytic Proliferation With Hemophagocytosis

2005 ◽  
Vol 129 (2) ◽  
pp. e39-e43 ◽  
Author(s):  
R. Nagarjun Rao ◽  
Chung-che Chang ◽  
Nevin Uysal ◽  
Kenneth Presberg ◽  
Vinod B. Shidham ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Course ◽  
Langerhans Cell ◽  
Variant Form ◽  
Malignant Histiocytosis ◽  
Unique Case ◽  
Multiple Organs ◽  
Biopsy Specimens ◽  
Extensive Involvement ◽  
Erdheim Chester

Abstract Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.

Association of Langerhans Cell Histiocytosis with Erdheim-Chester Disease: How Close Monocyte/Macrophage and Dendritic Cell Lineages Are?

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4716-4716
Author(s):  
Baptiste Hervier ◽  
Julien Haroche ◽  
Olivier Hermine ◽  
Jean Donadieu ◽  
Frederic Charlotte ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Langerhans Cell Histiocytosis ◽  
Conflicts Of Interest ◽  
Langerhans Cell ◽  
Bone Involvement ◽  
Time Interval ◽  
Cell Lineages ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

Abstract Abstract 4716 Objectives Histiocytoses are a heterogeneous group of diseases that can be classified into either Langerhans cell histiocytosis (LCH) or non-Langerhans cell histiocytosis. The latter includes Erdheim-Chester disease (ECD). This study investigated the clinical association between LCH and ECD. Methods This retrospective study included 16 patients (10 males, 6 females, median age 41 years) treated at twelve different university hospitals between 1970 and 2010. Inclusion criteria were biopsy-proven LCH in association with two or more diagnostic signs of ECD. Results LCH and ECD were diagnosed simultaneously in 4/16 cases, whereas LCH preceded ECD in 12/16 cases. The median time interval was 7.5 years (range 2–22) in these cases. Major organs involved in LCH were the bones (n=12), skin (n=8) and lungs (n=3). ECD mainly affected the large vessels (n=11), bones (n=11) and retroperitoneum (n=9). Non-biopsy proven central nervous system (n=6) and pituitary gland (n=6) involvement also occurred. No specific histologic features were identified in the 65 biopsies studied, including platelet-derived growth factor receptor β expression. Between one and four lines of treatment were required in nine patients diagnosed with LCH. Nine patients were treated with interferon α-2a after the diagnosis of ECD was made. A partial improvement occurred in all assessable patients concerning ECD (n=5) and/or LCH (n=2). These 16 patients were compared with a monocentric cohort of 48 ECD patients; the only difference between the groups was a lower frequency of bone involvement in ECD patients with concomitant LCH (9/13 vs 47/48, p<0.003). Conclusions This study suggests that a pathogenic link exists between LCH and ECD. Although the mechanisms responsible for both diseases remain unknown, the present association could argue for transitions between monocyte/macrophage and dendritic cell lineages. The patient characteristics of LCH in association with ECD were similar to those in patients with LCH alone, whereas bone involvement may have been less common in ECD when it was in association with LCH. Clinicians should be aware of this association and should consider the possibility of ECD in patients with LCH, especially in the case of treatment resistance. Disclosures: No relevant conflicts of interest to declare.

Isolated Langerhans Cell Histiocytosis Bone Lesion in Pediatric Patients

2015 ◽  
Vol 153 (5) ◽  
pp. 751-757 ◽  
Author(s):  
Aren Bezdjian ◽  
Abdullah A. Alarfaj ◽  
Namrata Varma ◽  
Sam J. Daniel
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Lesion ◽  
Langerhans Cell

Erdheim?Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate

2004 ◽  
Vol 445 (4) ◽  
pp. 405-409 ◽  
Author(s):  
V. P. Andrade ◽  
C. C. V. Nemer ◽  
A. N. L. Prezotti ◽  
W. S. L. Goulart
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Hard Palate ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease
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