A case of adult Langerhans cell histiocytosis showing successfully regenerated osseous tissue of the skull after chemotherapy

Suzuki, Takahiro; Izutsu, Koji; Kako, Shinichi; Ohta, Satoshi; Hangaishi, Akira; Kanda, Yoshinobu; Motokura, Toru; Kurokawa, Mineo

doi:10.1007/s12185-008-0033-3

Cited by 7 publications

(7 citation statements)

References 21 publications

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“…The few data available concerning vinblastine treatment in adult LCH patients mainly concern patients with bone disease [11, 21–23]. Individual case reports showed a rather good response to vinblastine [21–23].…”

Section: Discussionmentioning

confidence: 99%

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Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study

Tazi

Lorillon

Haroche

et al. 2017

Orphanet J Rare Dis

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BackgroundVinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a median time of 83 months. The objectives were to determine the overall response rate (based on the Histiocyte Society criteria), disease reactivation rate, toxicity, permanent consequences, and survival rate corresponding to this treatment. The lung involvement outcome was based on serial lung function tests. The distribution of right-censored end points was estimated by the Kaplan-Meier method. Univariate Cox model with time-fixed and time-varying covariates was used for the predictive analysis of reactivation in the responders. Univariate analyses of risk factors for neurotoxicity were based on nonparametric Wilcoxon rank sum tests and exact Fisher tests.ResultsThe median duration of the first course of vinblastine was 7.6 months, with a median cumulative dose of 160 mg [IQR 120–212]. Seventy percent of the patients were responders at the end of this treatment. Subsequently, LCH reactivation occurred with a 5-year cumulative incidence of 40%. During the study, 27 reactivations were observed in 17 patients, and half of these episodes were retreated with vinblastine. At the end of the last vinblastine treatment, 70% of the patients were responders. None of the patients with impaired lung function improved. No grade 3–4 peripheral neuropathy was observed. At the final vinblastine treatment, permanent LCH consequences, primarily pituitary stalk involvement, were present in 15 (43%) patients, and all were present at the time of vinblastine initiation. The 10-year survival rate was 86.2% (95CI, 71.8–100%), and the 2 patients who died from LCH had risk organ localizations.ConclusionsVinblastine is an effective and well-tolerated first-line treatment for adult LCH except in patients with lung involvement and impaired lung function. However, a significant portion of patients experienced LCH reactivation during long-term follow up. As in childhood LCH, the presence of risk organ involvement has a negative impact on patient prognosis.

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Section: Discussionmentioning

confidence: 99%

“…Individual case reports showed a rather good response to vinblastine [21–23]. In contrast, in a series of adult patients with bone disease, [11] found that 84% patients treated with vinblastine had a poor response, defined by either the absence of response or relapse of LCH within a year.…”

Section: Discussionmentioning

confidence: 99%

Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study

Tazi

Lorillon

Haroche

et al. 2017

Orphanet J Rare Dis

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“…The whole liver of this patient was hypodense on CT images, as previously reported. 13 The patient had never drunk alcohol, and laboratory tests ruled out viral hepatitis (hepatitis A, B, and C, Epstein-Barr virus, and cytomegalovirus) and autoimmunerelated diseases. Thus, nonalcoholic fatty liver disease (NAFLD) was considered, which caused the hepatic failure, in addition to LCH infiltration.…”

Section: Discussionmentioning

confidence: 99%

“…[9][10][11] On the other hand, in adult cases, optimal treatment has not been established, and the treatment strategy is employed referring to child cases. [4][5]8,12,13 We herein present a case of adult-onset MS-LCH, which rapidly progressed to multi-organ failure after steroid reduction and showed no improvement despite combination chemotherapy.…”

Section: Introductionmentioning

confidence: 95%

Rapidly Progressing Fatal Adult Multi-Organ Langerhans Cell Histiocytosis Complicated with Fatty Liver Disease

Yuasa¹,

Fujiwara²,

Oh³

et al. 2012

J Clin Exp Hematopathol

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Langerhans cell histiocytosis (LCH) is a clonal neoplasm that shows diverse clinical manifestations and courses of disease progression. The etiology and pathophysiology of LCH remain uncertain. We describe the clinical course of a 23-year-old Japanese woman with multi-system LCH, who showed rapid progression after steroid reduction and developed multi-organ failure. Liver biopsy showed LCH infiltration with fatty degeneration. She was treated with cytarabine, vincristine, and prednisolone according to the Japan LCH study group 02 protocol, without any clinical improvement. Low expression of Ki67 and bcl-2 failed to explain the rapid clinical course. Panhypopituitarism and hypothalamic dysfunction may have caused nonalcoholic fatty liver disease and liver failure. This case indicates that some multi-system LCH patients with hypopituitarism and hypothalamic dysfunction show very rapid progression and are difficult to treat.

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“…Hasta steroid tedavisine yanıtsızsa, progresyon gösteriyorsa ve çoklu organ tutulumu varsa diğer tedavi seçe-neklerinin (etoposid, vinblastin, metotreksat, siklofosfamid, siklosporin, talidomid gibi) denenebileceğine dair yayınlar vardır. 11 Kadın hastamız aksiller lenf bezi tutulumu ve solunum fonksiyon testlerinde bozulma nedeniyle evre II kabul edilmiş ve 0,5 mg/kg/gün dozda sistemik steroid tedavisi başlatılmıştır. Erkek hastamız ise izole PLHH'dir ve sadece solunum fonksiyon testlerinde bozulma vardır.…”

Section: Resim 3 1x100 H-e Boyamaunclassified

Two Cases of Langerhans Cell Histiocytosis with Initial Diagnosis of Tuberculosis

Yıldırım¹,

Karalezli²,

Şentürk³

et al. 2012

Solunum

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ÖZETLangerhans hücreli histiyositozis (LHH) etiyolojisi bilinmeyen bir hastalık grubudur. Bu yazıda uzun süre tanı konulamayıp tüber-küloz düşünülen LHH tanılı iki olgu sunulmaktadır.Olgu 1: Tüberküloz lenfadenit ön tanısıyla kliniğimize sevk edilen 21 yaşındaki kadın hastanın efor dispnesi vardı. Sigara kullanımı yoktu. İki kez sağ aksiller lenfadenit nedeniyle lenf nodu (LN) diseksiyonu geçirmişti ancak lezyon nüks etmekteydi. Toraks BT'sinde yaygın kistik lezyonları, aksiller LN biyopsisinde CD1a ve S100 ile boyanan Langerhans hücreleri görüldü.Olgu 2: Efor dispnesi ve polidipsi şikâyeti olan 27 yaşında erkek hasta polikliniğimize başvurdu. On paket/yıl sigara kullanım öyküsü vardı ama 2 yıl önce sigarayı bırakmıştı. On üç yaşında 3 kez spontan pnömotoraks geçirmiş ve sonuncusunda dekortikasyon yapılmıştı. Sekiz yıl önce şüpheli bir akciğer tüberkü-lozu nedeniyle antitüberküloz tedavi görmüştü. Toraks BT'sinde yaygın kistik lezyonlar mevcuttu. Hastaya, sigara içen genç erkek olması, rekürren spontan pnömotoraks öyküsü, toraks BT'deki görünümü nedeniyle LHH tanısı konuldu.LHH, nadir bir hastalık olduğu için, tıpkı iki olgumuzda görüldüğü gibi ayırıcı tanıda akla gelmeyebilmekte ve ülkemizde yaygın bir hastalık olan tüberküloz yanlış tanısıyla hastalara gereksiz tedaviler uygulanabilmektedir. Özellikle tipik radyolojik görünüm bu hastalığı düşündürmelidir.Anahtar Kelimeler: Langerhans hücreli histiositozis, pulmoner LHH, tüberküloz ABSTRACTLangerhans cell histiocytosis (LCH) is a group of diseases with unknown etiology. In this article, two cases of LCH with initial diagnosis of tuberculosis were reported.Case 1: A 21-year-old woman who was admitted with dyspnea with the prediagnosis of tuberculosis lenfadenitis. She was not a smoker. Lymph node (LN) dissection was done twice because of right axillary lymphadenitis. Computed tomography (CT) revealed common cystic lesions. Axillary LN biopsy showed CD1 and S100 positive Langerhans cells.Case 2: A 27-year-old male admitted with and polydipsia. He is an ex-smoker with a history of 10 packs/year cigarette smoking. He had had spontaneous pneumothorax three times. He had spontaneous pneumothorax three times at 13 year-old and underwent decortication at the last time. He had received antituberculosis treatment eight years ago. Common cystic lesions were observed in his thorax CT. He was diagnosed as LCH because he was a smoking young man with a recurrent spontaneous pneumothorax history and a typical view in his thorax CT.If the possibility of LCH is not considered as a distinctive diagnosis, in patients like these two cases, patients could be treated incorrectly for diagnosis of tuberculosis, which is a common disease in our country. Especially typical radiological appearance should lead to consideration of this disease.Keywords: Langerhans cell histiocytosis, pulmonary LCH, tuberculosis

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A case of adult Langerhans cell histiocytosis showing successfully regenerated osseous tissue of the skull after chemotherapy

Cited by 7 publications

References 21 publications

Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study

Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study

Rapidly Progressing Fatal Adult Multi-Organ Langerhans Cell Histiocytosis Complicated with Fatty Liver Disease

Two Cases of Langerhans Cell Histiocytosis with Initial Diagnosis of Tuberculosis

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