Hepatobiliary and Gastrointestinal Involvement in Langerhans Cell Histiocytosis—Spectrum of Three Cases

AbstractLangerhans cell histiocytosis (LCH) is a rare group of disorder, due to clonal neoplastic proliferation of dendritic cells in the bone marrow expressing a Langerhans cell phenotype.1 This disease particularly affects the pediatric age group and young adults and often presents with skin rashes, lung infiltrates, and bone lesions. It can also manifest with endocrine dysfunction and hematopoietic disorders. Involvement of gastrointestinal tract is very rare. Herein, we present a spectrum of three cases with gastrointestinal involvement.

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Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Pediatric and Developmental Pathology ◽

10.1177/1093526617750947 ◽

2018 ◽

Vol 21 (2) ◽

pp. 208-251 ◽

Cited By ~ 2

Author(s):

Alejandro A Gru ◽

Louis P Dehner

Keyword(s):

Langerhans Cell Histiocytosis ◽

Molecular Characteristics ◽

Juvenile Xanthogranuloma ◽

Age Group ◽

Hematologic Disorders ◽

Pediatric Age Group ◽

Frequent Form ◽

Epidemiologic Profile ◽

Hematopoietic Disorders ◽

Leukemias And Lymphomas

This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.

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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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CD79a+ T-Cell Lymphoblastic Lymphoma With Coexisting Langerhans Cell Histiocytosis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2001-125-0958-ctcllw ◽

2001 ◽

Vol 125 (7) ◽

pp. 958-960

Author(s):

Shiyong Li ◽

Michael J. Borowitz

Keyword(s):

T Cell ◽

Langerhans Cell Histiocytosis ◽

Close Association ◽

Cervical Lymphadenopathy ◽

Lymphoblastic Lymphoma ◽

Langerhans Cell ◽

Terminal Deoxynucleotidyl Transferase ◽

Simultaneous Occurrence ◽

Cell Phenotype ◽

Malignant Neoplasms

Abstract Although there is a close association between Langerhans cell histiocytosis and malignant neoplasms, simultaneous occurrence of lymphoblastic lymphoma and Langerhans cell histiocytosis in the same lymph node is an extremely rare finding. Herein, we describe such a case in a 26-year-old woman who presented with progressive cervical lymphadenopathy. The lymphoma cells have an immature T-cell phenotype (terminal deoxynucleotidyl transferase+, HLA-DR+, CD34+, CD38+, and CD7+) with expression of both CD3 and CD79a on immunohistochemical stain. The Langerhans cells are present focally with the characteristic morphologic features and immunophenotype (CD1a+ and S100+). The significance of CD79a coexpression in T-cell lymphoblastic lymphoma and the association between lymphoblastic lymphoma and Langerhans cell histiocytosis are discussed.

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Cutaneous Langerhans cell histiocytosis with gastrointestinal involvement treated with dabrafenib

JAAD Case Reports ◽

10.1016/j.jdcr.2017.07.028 ◽

2018 ◽

Vol 4 (1) ◽

pp. 95-97 ◽

Cited By ~ 1

Author(s):

Jacob R. Stewart ◽

Era C. Murzaku ◽

Titilola T. Sode ◽

Katherine A. Gordon

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Gastrointestinal Involvement

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Langerhans Cell Histiocytosis with Isolated Central Diabetes Insipidus, Low Grade Fever and Sellar Erosion

Trends in Pediatrics ◽

10.5222/tp.2021.86580 ◽

2021 ◽

Author(s):

İclal Okur ◽

Hasan Ari ◽

Semra Çetinkaya ◽

Betül Emine Derinkuyu ◽

Gizem Çağlar ◽

...

Keyword(s):

Diabetes Insipidus ◽

Langerhans Cell Histiocytosis ◽

Bone Structure ◽

Sella Turcica ◽

Langerhans Cell ◽

Central Diabetes Insipidus ◽

Bone Lesions ◽

Warning Sign ◽

Low Grade ◽

First Case

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. Although it is known that bone involvement is seen very frequently in cases with LCH, our case is the first case with a lytic-destructive lesion in the bone structure forming sella turcica. A 4-year-old, 5-month-old male patient who applied to our outpatient clinic was diagnosed with Langerhans cell histiocytosis in further examination after the diagnosis of central diabetes insipidus (CDI) was made. On cranial magnetic resonance imaging (MRI), widespread lytic-destructive bone lesions were observed in the bone structure forming the sella (sphenoid bone), sellar destruction not previously described in the literature. Sellar erosion has not been reported before in cases diagnosed with LCH in the literature. The presence of low-grade fever in a patient presenting with isolated CDI is a warning sign for the diagnosis of LCH.

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Gastrointestinal involvement in Langerhans' cell histiocytosis (Histiocytosis X): A clinical report of three cases

European Journal of Pediatrics ◽

10.1007/bf02171558 ◽

1990 ◽

Vol 149 (5) ◽

pp. 325-329 ◽

Cited By ~ 28

Author(s):

R. M. Egeler ◽

M. E. I. Schipper ◽

H. S. A. Heymans

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Histiocytosis X ◽

Clinical Report ◽

Gastrointestinal Involvement

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Calvarial Langerhans cell histiocytosis in an Adult: Typical imaging findings in an atypical age group

Radiology Case Reports ◽

10.1016/j.radcr.2019.09.009 ◽

2019 ◽

Vol 14 (12) ◽

pp. 1478-1482 ◽

Cited By ~ 1

Author(s):

Amjad Samara ◽

Jordan Nepute ◽

Hsiang-Chih Lu ◽

Richard J. Perrin ◽

Rami W. Eldaya

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Age Group ◽

Imaging Findings

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Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

Journal of Biomedical and Clinical Research ◽

10.1515/jbcr-2016-0001 ◽

2016 ◽

Vol 9 (1) ◽

pp. 3-16 ◽

Cited By ~ 1

Author(s):

Vera E. Papochieva ◽

Dimitrinka S. Miteva ◽

Penka I. Perenovska ◽

Guergana Petrova

Keyword(s):

Langerhans Cell Histiocytosis ◽

Eosinophilic Granuloma ◽

Langerhans Cell ◽

Unknown Etiology ◽

Bone Lesions ◽

Multisystem Disease ◽

The Past ◽

System Disease ◽

Young Smokers ◽

Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

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A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system

Neurology neuropsychiatry Psychosomatics ◽

10.14412/2074-2711-2020-6-117-123 ◽

2020 ◽

Vol 12 (6) ◽

pp. 117-123

Author(s):

L. V. Lukina ◽

V. A. Mikhailov ◽

N. I. Ananyeva ◽

G. E. Mazo ◽

L. I. Sitnik ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Langerhans Cell Histiocytosis ◽

Correct Diagnosis ◽

Brain Lesion ◽

Clinical Findings ◽

Langerhans Cell ◽

Unknown Etiology ◽

Bone Lesions ◽

The Central Nervous System

Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material.

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