Reactivation and risk of sequelae in Langerhans cell histiocytosis

Pollono, Daniel; Rey, Guadalupe; Latella, Antonio; Rosso, Diego; Chantada, Guillermo; Braier, Jorge

doi:10.1002/pbc.21145

Cited by 51 publications

(32 citation statements)

References 16 publications

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“…Nearly half of RO+ group reactivated vs. one third of patients in other groups (p 0.096). This is concordant with others showing that up to 63 % of patients reactivated within 2 y from diagnosis [3,24,25]. Interestingly, LCH II, although with heavier treatment but of similar duration, did not show reduced number of reactivation compared to LCH I [20].…”

Section: Discussionsupporting

confidence: 93%

Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?

et al. 2015

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Section: Discussionsupporting

confidence: 93%

Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?

et al. 2015

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“…6 The criteria of hepatic involvement 4 were hepatic enlargement >3 cm below costal margin in the midclavicular line, and/or hypoproteinemia, hypoalbuminemia < 25 g/L not due to other causes, and/or histopatological diagnosis.…”

Section: Methodsmentioning

confidence: 99%

Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey.

Tokgöz¹,

Çalışkan²

2016

UHOD

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The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. Medical records of children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding was bone lesions (%88,8), followed by hepatosplenomegaly (%44.4), lymphadenopathy (%44.4), seborrheic dermatitis (33,3%) and jaundice (33.3%). Of the 9 patients, 3 (33.3%) had single system disease [1 (11.1%) had unifocal while 2 (22.2%) had multifocal disease], 6 (66.6%) had multisystem disease [2 (22.2%) had risk organ negative and 4 (44.4%) had risk organ positive]. At the end of the 6th week of therapy, 5 patients (55.5%) had all the dead children had better response, 1 (11.1%) had an intermediate response while 3 (33.3%) had worse response. In terms of outcome, 5 (55%) patients were in complete remission, 3 (33%) patients were dead, and 1 patient was lost to follow up. All dead children had multisystem disease with risk organ involvement and were worse responders at the 6th week of therapy. In children with LCH, the most important factors determining prognosis were risk organ involvement, multisystem disease and response at 6th week of treatment. New treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor. Keywords: Langerhans cell histiocytosis, Child ÖZET Çocuklarda Langerhans Hücreli Histiyositoz: Türkiye'den Tek Merkez DeneyimiBu çalışmanın amacı, merkezimizde Langerhans hücreli histiyositoz (LCH) tanısı alan çocuklar ile ilgili deneyimlerimizi sunmaktadır.LCH tanılı çocukların tıbbi kayıtları, hastaların cinsiyeti, tanı yaşı, klinik bulgular, risk grupları, tedavi rejimleri ve gidişat açısından değerlendirilmek üzere incelendi. Medyan yaşı 22 ay olan 9 hasta çalışmaya dâhil edildi. En sık görülen klinik bulgu kemik lezyonlarıydı

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“…Disease reactivations have been related to an increased risk of pituitary involvement [11,13,31,32] . Nevertheless, reactivations were significantly less in the APD group.…”

Section: Discussionmentioning

confidence: 99%

“…These lesions were either isolated or in the context of MS. Radiotherapy applied to the hypothalamic-pituitary region was especially recorded to evaluate the possible effect of radiotherapy on hypothalamic-pituitary function. Disease reactivation was defined according to a previous report [11] , and for the purpose of this study, it was considered when the patient had 1 or more new episodes during follow-up. Endocrine Assessment Anterior pituitary involvement was considered when at least 1 pituitary hormone axis was affected and there were clinical signs and symptoms of hormone deficiency (polyuria/polydipsia, growth retardation, pubertal delay).…”

Section: Methodsmentioning

confidence: 99%

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Predictor Variables of Developing Anterior Pituitary Deficiencies in a Group of Paediatric Patients with Central Diabetes Insipidus and Langerhans Cell Histiocytosis

Vaiani

Malossetti²,

Vega³

et al. 2016

Horm Res Paediatr

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Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Central diabetes insipidus (CDI) is the most frequent endocrine manifestation and is a known risk factor for the development of further anterior pituitary hormone deficiencies (APD). However, not all CDI patients develop APD, as observed during prolonged periods of follow-up. Aim: To find predictors of developing APD in LCH children with CDI followed in our institution. Methods: We retrospectively analysed 44 patients over a median period (quartiles) of 12.3 years (8.79-14.24). Patients were subdivided into group 1 and group 2, according to absence or presence of APD, respectively. The main variables studied were: (1) chronological age (CA) at LCH diagnosis, (2) the primary site of LCH at diagnosis: low risk (LR) and multisystemic risk organs, and (3) the presence of reactivation. Results: Multivariate Cox regression analysis showed that APD was positively associated with CA at LCH diagnosis [relative risk (RR) 1.14, p < 0.01], the LR clinical form (RR 8.6, p < 0.03), and negatively associated with the presence of reactivations (RR 0.3, p < 0.01). Conclusions: Patients with older CA at LCH diagnosis, LR clinical forms, and fewer reactivation episodes might represent a subgroup of paediatric LCH CDI patients with a higher risk of developing APD.

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Reactivation and risk of sequelae in Langerhans cell histiocytosis

Abstract: Incidence of reactivation correlates with the stage of the disease at diagnosis. Incidence of sequelae correlates with the occurrence of reactivations.

Cited by 51 publications

References 16 publications

Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?

Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?

Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey.

Predictor Variables of Developing Anterior Pituitary Deficiencies in a Group of Paediatric Patients with Central Diabetes Insipidus and Langerhans Cell Histiocytosis

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