Adult Langerhans Cell Histiocytosis with Independently Relapsing Lung and Liver Lesions That was Successfully Treated with Etoposide

Konno, Satoshi; Hizawa, Nubuyuki; Betsuyaku, Tomoko; Matsumura, Yasuo; Yamamoto, Hiroshi; Koizumi, Tomonobu; Nishimura, Masaharu

doi:10.2169/internalmedicine.46.6433

Cited by 15 publications

(10 citation statements)

References 14 publications

(14 reference statements)

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“…Case reports (74,75) have suggested that this intervention alone may lead to marked improvement or resolution of the disease. However, relapse or worsening despite smoking cessation have also been described (76,77). Nevertheless, because of the high rate of PLCH-associated pulmonary malignancy, the established relationship between smoking and airflow obstruction and many other health benefits, smoking cessation is the most important step in the management of PLCH.…”

Section: Smoking Cessationmentioning

confidence: 99%

“…Most of the data on these agents come from studies in children showing improvement or remission of multisystem LCH (14,(79)(80)(81). Nevertheless, small case reports and case series (77,80,(82)(83)(84)(85)(86) suggest that these agents may have a role in the treatment of LCH with multisystem involvement in adults, including pulmonary disease, although lung-specific indexes of improvement vary.…”

Section: Cytotoxic Drugsmentioning

confidence: 99%

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Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

Juvet

Hwang²,

Downey³

2010

Canadian Respiratory Journal

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Pulmonary Langerhans' cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.

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Section: Smoking Cessationmentioning

confidence: 99%

Section: Cytotoxic Drugsmentioning

confidence: 99%

Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

Juvet

Hwang²,

Downey³

2010

Canadian Respiratory Journal

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“…First, haematological disorders (leukaemia or lymphoma) may be associated with LCH, including adult PLCH [ 11 , 22 , 23 ], and they may thus be screened using these blood tests. Second, although it was not the case in our cohort, liver LCH involvement in adult PLCH patients has been reported [ 24 – 26 ]. Finally, the presence of lymphopaenia and biological liver alterations is important to consider in patients who will eventually need treatment for their disease.…”

Section: Discussionmentioning

confidence: 66%

Extrathoracic investigation in adult patients with isolated pulmonary langerhans cell histiocytosis

Tazi

Margerie-Mellon

Vercellino

et al. 2016

Orphanet J Rare Dis

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BackgroundAn important objective on diagnosis of patients with Langerhans cell histiocytosis (LCH) is to determine the extent of disease. However, whether systematic extrathoracic investigation is needed in adult patients with clinically isolated pulmonary LCH (PLCH) has not been evaluated.MethodsIn this prospective, multicentre study, 54 consecutive patients with newly diagnosed clinically isolated PLCH were systematically evaluated at inclusion by bone imaging and blood laboratory testing to search for subclinical extrapulmonary LCH involvement. The patients were followed over a 2-year period. At each visit, they were asked about the presence of extrapulmonary manifestations of LCH.ResultsIn the absence of bone symptoms, the skeletal X-ray survey results were normal for all but two patients who had a localised bone lesion consistent with possible LCH involvement, that remained unchanged over 2 years of follow-up. Whole-body bone scintigraphy did not add information to the plain radiography findings for the detection of asymptomatic bone involvement in isolated PLCH. Conversely, it showed nonspecific focal bone uptake in 18 % of the patients, mainly corresponding to post-traumatic or degenerative abnormalities unrelated to LCH. Mild leucocytosis due to neutrophilia was observed in 22 % of the patients and was not related to their smoking habits. Three patients had mild isolated lymphocytosis without haematological disease, whereas two patients had mild lymphopaenia. A mild inflammatory biological syndrome was observed in a minority of patients without infection or constitutional symptoms and was not associated with progressive disease. A substantial proportion (24.5 %) of the patients had abnormal biological liver test results, including elevated liver enzymes and/or cholestasis, which were not linked to LCH involvement in this cohort.ConclusionsObtaining a thorough history and performing comprehensive physical examination are essential for staging patients diagnosed with PLCH. In the absence of symptoms or signs suggestive of extrapulmonary LCH involvement, the systematic performing of recommended bone imaging does not appear informative. Although the observed blood laboratory abnormalities were not specifically related to LCH, performing these tests in the diagnostic workup for PLCH is useful because some of these alterations may impact patient management.Trial registrationClinicalTrials.gov: No. NCT01225601; URL: www.clinicaltrials.gov

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“…Literature addressing the management of refractory/relapsed MS-LCH in adults is much less robust, most consisting of case reports (Table 2) [12,[23][24][25][26][27]. More recently, B-RAF mutations have been observed in 38% to 69% of cases of LCH [27], making salvage with vemurafenib an appropriate choice in patients that harbor the mutation.…”

Section: Discussionmentioning

confidence: 99%

A Case of Langerhans Cell Histiocytosis With Risk Organ Involvement in a Young Adult

Kam¹,

Mahon²,

Karmali³

2014

J Hematol

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Multisystem Langerhans cell histiocytosis (MS-LCH) is a rare disease for which the standard of care has not been clearly established. We are the first to address frontline and salvage options for the management of an adolescent young adult (AYA) male with MS-LCH with "risk organ" involvement, a population for which there are no therapeutic guidelines. Our 33-year-old male patient presented with MS-LCH with generalized lymphadenopathy, hepatic and splenic involvement. He was initially treated with vinblastine and prednisolone with progression in disease. Our exploration of the literature revealed that salvage options in adults include single-agent chemotherapy, hematopoietic stem cell transplant, imatinib, and vemurafenib and are limited to small case series with questionable efficacy. We opted to use a pediatric regimen with the combination of cladribine and cytarabine and demonstrate that this approach can in fact be effective in an AYA patient.

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Adult Langerhans Cell Histiocytosis with Independently Relapsing Lung and Liver Lesions That was Successfully Treated with Etoposide

Abstract: A 35-year-old

Cited by 15 publications

References 14 publications

Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

Extrathoracic investigation in adult patients with isolated pulmonary langerhans cell histiocytosis

A Case of Langerhans Cell Histiocytosis With Risk Organ Involvement in a Young Adult

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