Adult-onset Neurological Langerhans Cell histiocytosis and Response to Treatment

Introduction: Langerhans Cell Histiocytosis (LCH) is a rare form of cancer that mostly affects children and rarely adults. LCH involves an abnormal clonal proliferation of Langerhans cells in the bone marrow. These cells are capable of migrating from the skin to lymph nodes. Therefore, it is characterized as a multisystem disease. Neurological manifestations are not common, and often patients’ present with endocrine dysfunction with neuroimaging findings of hypothalamic and pituitary masses can mimic pituitary adenoma. Here, we discuss two instances of unusual adult-onset, primary neurological LCH in patients with a positive response to therapy-these two patients presented with mass lesion and neurodegenerative form of LCH, respectively. LCH can manifest features of mass lesions or neurodegeneration on brain Magnetic Resonance Imaging (MRI). Since it is rare in adults, it is crucial to identify this condition as timely treatment can have a better prognosis.

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Pediatric Langerhans Cell Histiocytosis (LCH) Clinical Outcome in Hospital Civil De Guadalajara, México

Blood ◽

10.1182/blood-2018-99-115794 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 4951-4951 ◽

Cited By ~ 1

Author(s):

Katterine Rojas Rodríguez ◽

Veronica Soto ◽

Carlos Rodriguez-Galindo ◽

Paola M. Friedrich ◽

Edwin Guzmán ◽

...

Keyword(s):

Clinical Outcome ◽

Langerhans Cell Histiocytosis ◽

Disease Risk ◽

Conflicts Of Interest ◽

Systemic Disease ◽

Giant Cells ◽

Langerhans Cell ◽

Induction Treatment ◽

Multisystem Disease ◽

Clonal Proliferation

Abstract Pediatric Langerhans cell histiocytosis (LCH) clinical outcome in Hospital Civil de Guadalajara, México. Introduction : LCH results from clonal proliferation of functionally and immunophenotyped inmature round Langerhans cells along with eosinophiles, machrophages, lymphocytes and ocasionally multinucleated giant cells (1). Its incidence is 2-10 cases by million of children below 15 yr in US (2). Our objective was to describe the clinical characteristics and treatment outcome of patients with LCH at Departement of Hematology-Oncology of Hospital Civil de Guadalajara México. Methods: It was a retrospective design and 41 pediatric patients below 18 yr were included. The diagnosis was corroborated by pathology and immunohistochemistry. Variables as age, gender, localised vs systemic disease, risk organ commitment, global survival (GS) and event free survival (EFS) were analysed. We used descriptive and inferencial statistics with SPSS program. Results: There were included 41 patients from January 1st 2012 to December 31st 2017. Relation male:female was 1.1:1. Mean presentation was localised disease (58%). Bone was the principal affected structure (34%) and it was 71% to be combined with lung, lymph node and CNS compromise. Risk organ commitment was presented in 32%, being more frecquent bone marrow and liver in 22% each one. Time induction treatment was equal or below 12 weeks in 66% of patients. The 25% of patients had reactivation of LCH, with similar lesions to the beggining in 19.5%. We found statistically significant differences between dead patients (DP) (14.6%) and not dead patients (NDP) (85.4%) in clinical presentation: localised (0% in DP vs 69% in NDP) and systemic disease (100% vs 31%) (p=0,003) and risk organ commitment (100% in DP vs 20% in NDP) (p=0,000). Median age of 13 vs 24 months was for DP and NDP respectively. Conclusion: Dead patients were younger than 13 months old, with systemic disease, and risk organ commitment. We found a later asking of medical advice in DP (6 months) vs NDP (2 months). Keys words: langerhans cell histiocytosis, multisystem disease, risk organ Figure. Figure. Disclosures No relevant conflicts of interest to declare.

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Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

Case Reports in Dermatology ◽

10.1159/000440643 ◽

2015 ◽

Vol 7 (3) ◽

pp. 237-240 ◽

Cited By ~ 1

Author(s):

Hisayuki Tono ◽

Taku Fujimura ◽

Aya Kakizaki ◽

Sadanori Furudate ◽

Masaya Ishibashi ◽

...

Keyword(s):

Immunohistochemical Staining ◽

Langerhans Cell Histiocytosis ◽

Skin Lesions ◽

Langerhans Cell ◽

Adult Onset ◽

Cutaneous Involvement ◽

System Disease ◽

Clonal Proliferation ◽

Old Japanese ◽

Precise Diagnosis

Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH.

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Case series of cutaneous Langerhans cell histiocytosis in Indonesian children; The clinicopathological spectrum

Dermatology Reports ◽

10.4081/dr.2020.8777 ◽

2020 ◽

Vol 12 (2) ◽

Author(s):

Irianiwati Widodo ◽

Nita Sahara ◽

Ery Kus Dwianingsih ◽

Paranita Ferronika

Keyword(s):

Langerhans Cell Histiocytosis ◽

Case Series ◽

Langerhans Cell ◽

Multisystem Disease ◽

Systemic Involvement ◽

Clonal Proliferation ◽

S 100 ◽

Microscopic Features ◽

Skin Biopsies ◽

Histological Reactions

Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of Langerhans cells, which are immunoreactive to S-100 and CD-1a/ CD207 (Langerin). Cutaneous involvement is the most common presentation of LCH in children. It is suggested that the patients with single-system LCH limited to the skin have a better prognosis than those with systemic involvement. Three histologic reactions of cutaneous LCH have been reported and are associated with the clinical types of LCH. These histological reactions include: proliferative, granulomatous, and xanthomatous. This study presents the clinicopathological features of ten cutaneous LCH cases collected from Dr. Sardjito General Hospital Yogyakarta Indonesia between 2014-2018. The ten cases showed various clinical features, in which some features mimic other diseases. The microscopic features of skin biopsies showed granulomatous reaction in 80% of cases and proliferative reaction in the other 20%. Five patients (50% of cases) who died had systemic manifestation of thrombocytopenia, anemia, icterus, hepatosplenomegaly, and revealed the granulomatous type from their skin biopsy specimens. The clinical recognition of LCH and subsequent histological reaction determination are important since some cases may develop multisystem disease and have a poor prognosis.

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Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System

Hormone Research in Paediatrics ◽

10.1159/000517040 ◽

2021 ◽

pp. 1-9

Author(s):

Elisa Vaiani ◽

Guido Felizzia ◽

Fabiana Lubieniecki ◽

Jorge Braier ◽

Alicia Belgorosky

Keyword(s):

Anterior Pituitary ◽

Langerhans Cell Histiocytosis ◽

Endocrine System ◽

Langerhans Cell ◽

Hormone Deficiency ◽

Bone Lesions ◽

Pituitary Hormone ◽

Multisystem Disease ◽

Anterior Pituitary Hormone

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.

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Adult-Onset Langerhans Cell Histiocytosis And The Role of Vinblastine In The Treatment -Ankara Oncology Hospital Experience

Acta Oncologica Turcica ◽

10.5505/aot.2020.37097 ◽

2020 ◽

Vol 53 (3) ◽

pp. 492-499

Author(s):

Ayşegül Tetik ◽

Bahar Uncu Ulu ◽

Mehmet Bakırtaş ◽

Tuğçe Nur Yiğenoğlu ◽

Jale Yıldız ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Adult Onset ◽

Hospital Experience

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Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-020-01495-5 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Solenne Le Louet ◽

Mohamed-Aziz Barkaoui ◽

Jean Miron ◽

Claire Galambrun ◽

Nathalie Aladjidi ◽

...

Keyword(s):

Targeted Therapy ◽

Langerhans Cell Histiocytosis ◽

Treatment Guidelines ◽

Langerhans Cell ◽

Infection Prophylaxis ◽

Lung Involvement ◽

Multisystem Disease ◽

Abnormal Chest ◽

Life Threatening ◽

Severe Lung

Abstract Background Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed. Methods Among 1482 children (< 15 years) registered in the French LCH registry (1994–2018), 111 (7.4%) had lung involvement. This retrospective study included data for 17 (1.1%) patients that required one or more intensive care unit (ICU) admissions for respiratory failure. Results The median age was 1.3 years at the first ICU hospitalization. Of the 17 patients, 14 presented with lung involvement at the LCH diagnosis, and 7 patients (41%) had concomitant involvement of risk-organ (hematologic, spleen, or liver). Thirty-five ICU hospitalizations were analysed. Among these, 22 (63%) were secondary to a pneumothorax, 5 (14%) were associated with important cystic lesions without pneumothorax, and 8 (23%) included a diffuse micronodular lung infiltration in the context of multisystem disease. First-line vinblastine–corticosteroid combination therapy was administered to 16 patients; 12 patients required a second-line therapy (cladribine: n = 7; etoposide-aracytine: n = 3; targeted therapy n = 2). A total of 6 children (35%) died (repeated pneumothorax: n = 3; diffuse micronodular lung infiltration in the context of multisystem disease: n = 2; following lung transplantation: n = 1). For survivors, the median follow-up after ICU was 11.2 years. Among these, 9 patients remain asymptomatic despite abnormal chest imaging. Conclusions Severe lung involvement is unusual in childhood LCH, but it is associated with high mortality. Treatment guidelines should be improved for this group of patients: viral infection prophylaxis and early administration of a new LCH therapy, such as targeted therapy.

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