Adult disseminated Langerhans cell histiocytosis: incidence, racial disparities and long‐term outcomes

Goyal, Gaurav; Shah, Mithun Vinod; Hook, C. Christopher; Wolanskyj, Alexandra P.; Call, Timothy G.; Rech, Karen L.; Go, Ronald S.

doi:10.1111/bjh.14818

Cited by 52 publications

(60 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The prognosis of histiocytic neoplasms is variable, and the natural course may be relatively benign and self-limiting in some cases, whereas in others it may be much more aggressive and life-threatening. [5][6][7] Most adult patients with histiocytic neoplasms receive empiric systemic immunosuppressive or cytotoxic therapies because of the lack of approved treatments. Recently, vemurafenib was approved by the US Food and Drug Administration for the treatment of BRAF-V600-mutant ECD.…”

mentioning

confidence: 99%

Tumor mutational burden and other predictive immunotherapy markers in histiocytic neoplasms

Goyal

Lau

Nagle

et al. 2019

Blood

Self Cite

View full text Add to dashboard Cite

mentioning

confidence: 99%

Tumor mutational burden and other predictive immunotherapy markers in histiocytic neoplasms

Goyal

Lau

Nagle

et al. 2019

Blood

Self Cite

View full text Add to dashboard Cite

“…Erdheim‐Chester disease (ECD) is a rare non‐Langerhans cell histiocytosis (non‐LCH) that is included under histiocytic neoplasms due to the discovery of recurrent clonal MAP‐kinase pathway ( RAS‐RAF‐MEK‐ERK ) mutations (Swerdlow et al , ). A population‐based study found LCH to be associated with increased risk of myeloid neoplasms (Goyal et al , ). A recent study also reported a high frequency of myeloid neoplasms in adults with ECD (10%), with the most common neoplasm being chronic myelomonocytic leukaemia (CMML) (Papo et al , ).…”

mentioning

confidence: 99%

Concomitant Erdheim‐Chester disease and chronic myelomonocytic leukaemia: genomic insights into a common clonal origin

et al. 2019

Self Cite

View full text Add to dashboard Cite

“…Taken together with the higher rates of smoking and higher median pack‐years of the additional malignancy cohort compared to the LCH‐only cohort, our findings suggest environmental exposures such as cigarette smoke may cause a localized reactive process which contributes to additional tumorigenesis similar to the association of lung cancer with smoking. Nonetheless, the increased incidence of secondary malignancies has been previously documented in the literature, including a recent report noting the increased incidence of AML as a second primary malignancy in adult LCH by Goyal et al and a review of 102 patients with pulmonary LCH which noted a possible association with increased susceptibility to cancer development …”

Section: Discussionmentioning

confidence: 96%

Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies

Laird

Chau

et al. 2018

Cancer Medicine

View full text Add to dashboard Cite

BackgroundLangerhans cell histiocytosis (LCH) is a rare disorder of histiocyte proliferation. Previous case studies suggest a higher prevalence of hematologic and solid malignancies among LCH patients, possibly due to treatment with tumorigenic agents such as etoposide. We report the first large, single‐institution experience of adult LCH patients with additional malignancies to study the characteristics of these patients.MethodsWe identified 132 consecutive patients >18 years of age with histologically confirmed LCH at our center between 1990 and 2015. Demographics and detailed oncologic history were recorded to identify patients with additional malignancies.ResultsOf 132 adult LCH patients, 42 (32%) patients had an additional malignancy. There were 53 malignancies among the 42 patients, with 31 (58%) preceding LCH diagnosis, 11 concurrent (≤3 months; 21%) with LCH diagnosis, and 11 (21%) after. Median age was 54 years (range 28‐89) with a median follow‐up of 3.7 years (0.1‐22.2) for this cohort. OS at 3 years was 98% in patients with LCH alone and 82% among patients with additional malignancies, with 30 (71%) alive at last follow‐up. Solid tumors, lymphomas, and other hematologic malignancies were observed as follows: 39 (74%), 9 (17%), and 5 (9%).ConclusionOur cohort of adult LCH patients demonstrates an unusually high number of additional malignancies. Our study includes predominantly malignancies diagnosed preceding or concurrent with LCH, suggesting a cause of malignancy independent of LCH treatment. Further exploration of the biology of this rare disease may elucidate the mechanism of frequent additional malignancies.

show abstract

Adult disseminated Langerhans cell histiocytosis: incidence, racial disparities and long‐term outcomes

Cited by 52 publications

References 9 publications

Tumor mutational burden and other predictive immunotherapy markers in histiocytic neoplasms

Tumor mutational burden and other predictive immunotherapy markers in histiocytic neoplasms

Concomitant Erdheim‐Chester disease and chronic myelomonocytic leukaemia: genomic insights into a common clonal origin

Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies

Contact Info

Product

Resources

About