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Pediatr Int 2019 Feb 15. Epub 2019 Feb 15.

Department of Pediatrics, Asahikawa Medical University, Hokkaido, Japan.

Front Immunol 2018 29;9:3148. Epub 2019 Jan 29.

Research Institute Hospital 12 Octubre (I+12), Madrid, Spain.

NF-κB1 is a master regulator of both acquired and innate responses. loss-of-function mutations elicit a wide clinical phenotype with asymptomatic individuals at one end of the spectrum and patients with common variable immunodeficiency, combined immunodeficiency or autoinflammation at the other. Impairment of acquired and innate immunity and disseminated infection expands the clinical and immunological phenotype of NF-κB1 deficiency. Read More

Mult Scler Relat Disord 2019 Feb 6;30:94-97. Epub 2019 Feb 6.

Department of Neurology, Santa Lucía University Hospital, Cartagena, Spain. Electronic address:

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis that presents potential impairment of the central nervous system (CNS). Frequent CNS impairment makes ECD a disease worth considering in the differential diagnosis of multiple sclerosis (MS). We report the case of a patient initially diagnosed with relapsing-remitting MS with an atypical course who developed ECD during the disease progression. Read More

J Pediatr Hematol Oncol 2019 Feb 1. Epub 2019 Feb 1.

Neonatal Unit, Doce de Octubre University Hospital, Madrid, Spain.

Introduction: Enterocolitis is a relatively common disease in neonatal period that can be a result of many underlying pathologies. One of them, which is an unusual disorder especially in neonatal age and with gastrointestinal involvement, is Langerhans cell histiocytosis (LCH). This case shows a severe neonatal LCH with digestive involvement which required intensive care and had an abnormal presentation, being hard to diagnose attributable to the diversity of symptoms. Read More

J Eur Acad Dermatol Venereol 2019 Feb 1. Epub 2019 Feb 1.

Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.

Histiocytoses are rare proliferative disorders of the mononuclear-phagocyte system. Within non-Langerhans-cell histiocytoses (NLCH), multisystem (MS) cases potentially constitute a subset at high risk of complications and progression. The current 2016 revised classification subdivides histiocytoses in 5 groups, based on highly heterogeneous criteria. Read More

Pediatr Hematol Oncol 2019 Jan 29:1-8. Epub 2019 Jan 29.

a Key Laboratory of Pediatric Hematology & Oncology, Department of Hematology and Oncology, Shanghai Children's Medical Center , Shanghai Jiao Tong University School of Medicine , Shanghai , China.

Background: This is a descriptive review of the clinical patterns and outcomes of children with Langerhans cell histiocytosis and single-system involvement (SS-LCH) treated at Shanghai Children's Medical Center.

Procedure: 60 evaluable newly diagnosed patients (37 boys, 23 girls) with a median age of 3.9 years (range: 0. Read More

ANZ J Surg 2019 Jan 28. Epub 2019 Jan 28.

Department of Otolaryngology, The Royal Victorian Eye and Ear Hospital, Melbourne, Victoria, Australia.

Radiologia 2019 Jan 24. Epub 2019 Jan 24.

Department of Radiology and Medical Imaging, University of Virginia Health System, Charlottesville, Virginia, Estados Unidos; Department of Radiology, One Hospital Dr, University of Missouri Health System, Columbia, Missouri, Estados Unidos. Electronic address:

Objective: To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans cell histiocytosis in Rosai-Dorfman disease and Erdheim-Chester disease. We also review the etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. Read More

Sci Rep 2019 Jan 22;9(1):317. Epub 2019 Jan 22.

Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.

Accurate risk stratification according to the extent of Langerhans cell histiocytosis (LCH) determined on whole-body evaluation is important for determining the treatment plans and prognosis in patients with LCH. This study aimed to compare the lesion detectability and the accuracy of risk stratification of skeletal survey, bone scan, and whole-body magnetic resonance imaging (WB-MRI) in patients with LCH. Patients with newly-diagnosed LCH who underwent all three imaging modalities were retrospectively included (n = 46). Read More

J Emerg Med 2019 Jan 17. Epub 2019 Jan 17.

Department of Emergency Medicine, Staten Island University Hospital, Northwell Health, Staten Island, New York.

Hum Pathol 2019 Jan 15. Epub 2019 Jan 15.

Department of Pathology, 75 Francis Street, Brigham and Women's Hospital, Boston, MA, 02115. Electronic address:

The prevalence of incidental non-neoplastic lung disease in patients undergoing resection for mass lesions is unknown. We determined the prevalence and characteristics of parenchymal findings in patients with lung nodules, aiming to increase awareness of findings that could potentially impact patient management. 397 patients with benign or malignant mass lesions with available pre-surgical chest computed tomography scans resected between January 2001 and July 2015 were included. Read More

Pediatr Ann 2019 Jan;48(1):e30-e35

Certain rashes and cutaneous lesions in a newborn can be clues to more concerning diseases and conditions if recognized and evaluated promptly. Langerhans cell histiocytosis, cutaneous forms of cancer (such as leukemia cutis, neuroblastoma, and rhabdomyosarcoma), developmental abnormalities such as neural tube or spinal dysraphism, and aplasia cutis congenita, nutritional deficiency, and immunodeficiency all have a range of cutaneous findings that will be reviewed herein to guide diagnosis and management. [Pediatr Ann. Read More

J Pathol Transl Med 2019 Jan 16. Epub 2019 Jan 16.

Department of Otorhinolaryngology-Head and Neck Surgery, Gachon University Gil Medical Center, Gachon University College of Medicine, Incheon, Korea.

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. Read More

Zhonghua Bing Li Xue Za Zhi 2019 Jan;48(1):17-21

Department of Pathology, Beijing Children Hospital, Beijing 100042, China.

To investigate the clinicopathological and ultrastructural characteristics of Langerhans cell histiocytosis (LCH) in children. A total of 345 cases of LCH from the Department of Pathology, Beijing Children Hospital from January 2012 to March 2016 were investigated by hematoxylin-eosin stain, EnVision immunohistochemistry and transmission electron microscopy. The rate of primary clinical diagnosis of LCH in children was 46. Read More

Kathmandu Univ Med J (KUMJ) 2018 Apr-Jun;16(62):201-203

Department of Oral Surgery, Peoples Dental College, Shorakhutte, Kathmandu, Nepal.

Eosinophilic Granuloma is the mildest and localized form of Langerhans Cell Histiocytosis and is characterized by clonal proliferation of Langerhans cells. It is a rare disease, accounting for less than 1% of all the osseous neoplasms. It has predilection for the axial skeleton and incidence in jaws is just 7. Read More

Dev Period Med 2018 ;22(4):376-378

Zakład Patologii i Diagnostyki Laboratoryjnej oraz Zakład Onkologii Molekularnej i Translacyjnej,Centrum Onkologii - Instytut im. Marii Skłodowskiej-Curie, Warszawa, Polska.

Langerhans Cell Histiocytosis (LCH) is a rare disease involving the occurrence of disturbances in the mitogene-activated kinases pathway (MAPK). At present, it is known that the appearance of such disorders is usually connected with a more aggressive form of the disease, more frequently resistant to conventional chemotherapy, as well as characterised by a higher probability of relapse and progression. Since recently, it has been possible to apply BRAF inhibitors in the treatment of LCH patients, however, there are no clear guidelines regarding the criteria for implementing this type of therapy. Read More

J Obstet Gynaecol 2019 Jan 11:1-3. Epub 2019 Jan 11.

a Department of Obstetrics and Gynecology , West China Second University Hospital, Sichuan University , Chengdu , China.

Medicine (Baltimore) 2019 Jan;98(2):e13853

Department of Endocrinology and Metabolism.

Rationale: Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation of immature dendritic cells, mainly affects children. LCH in adult sellar region is extremely rare. In literature, optimal treatments remain unclear and only a few cases of LCH were treated using surgery. Read More

Neurol Sci 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

Langerhans cell histiocytosis (LCH) is a rare disorder in adults which usually manifests with involvement of multiple organ systems, including the central nervous system. We describe an unusual case of biopsy-proven LCH presenting with frontotemporal-dominant cognitive impairment with hypothalamic involvement, along with multisystem disease. We propose that the dementia was probably an immune-mediated process triggered by LCH which responded dramatically to high-dose steroids. Read More

Acta Derm Venereol 2019 01 9. Epub 2019 Jan 9.

Unit of Anatomic Pathology, Department of Molecular Medicine, IRCCS San Matteo Foundation, University of Pavia, IT-27100 Pavia, Italy.

Mod Pathol 2019 Jan 9. Epub 2019 Jan 9.

Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA.

Recent studies have demonstrated recurrent activating mutations involving the classical MAPK and PI3K signaling pathways in a large proportion of histiocytic neoplasms, such as Langerhans cell histiocytosis. However, very little is known about the molecular genetics of histiocytic sarcoma, a rare aggressive malignant neoplasm that shows pathologic characteristics of mature macrophages. Here we report the genomic characteristics of a large cohort of histiocytic sarcomas (n = 28) using a targeted next-generation sequencing approach to identify driver alterations. Read More

BMC Pulm Med 2019 Jan 8;19(1). Epub 2019 Jan 8.

Department of Thoracic Surgery, West China Hospital, Sichuan University, Chengdu, 610041, People's Republic of China.

Background: Cystic lung lesions involving both lungs include a variety of diseases, such as pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis and pulmonary metastasis. Primary pulmonary meningioma accompanied with intrapulmonary metastasis was extremely rare and we were not aware of previous studies reporting with cystic radiological manifestation.

Case Presentation: A 64-year-old female patient was admitted to our department for a mass located in right posterior mediastinum with multiple cystic pulmonary lesions. Read More

Clin Exp Dermatol 2019 Jan 4. Epub 2019 Jan 4.

Department of Pediatrics, The Seventh Affiliated Hospital, Sun Yat-Sen University, Shenzhen, China.

Congenital self-healing reticulohistiocytosis (CSHR) is a rare disorder characterized by benign skin lesions with a tendency to self-heal. Multiple skin lesions are usually present in CSHR. It is very difficult to distinguish between CSHR and an invasive Langerhans cell histiocytosis. Read More

Cir Cir 2019 ;87(1):96-100

Delegación Baja California. Instituto Mexicano del Seguro Social, Baja California, México.

Background: Langerhans cell histiocytosis is a rare disease in the adult, predominates in the pediatric age and is more common in men than in women. The sites of greater affection are the long bones, followed by the bones of the skull. Patients usually have symptoms related to the site of affection, usually polyostotic lesions, monostotic lesions occur less frequently. Read More

Cancer Med 2019 Jan 30;8(1):58-66. Epub 2018 Dec 30.

Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York.

Background: Langerhans cell histiocytosis (LCH) is a rare disorder of histiocyte proliferation. Previous case studies suggest a higher prevalence of hematologic and solid malignancies among LCH patients, possibly due to treatment with tumorigenic agents such as etoposide. We report the first large, single-institution experience of adult LCH patients with additional malignancies to study the characteristics of these patients. Read More

Pediatr Hematol Oncol 2018 Dec 31:1-7. Epub 2018 Dec 31.

a Texas Children's Cancer Center , Houston , Texas , USA.

Objective: Since patients with langerhans cell histiocytosis and neurologic dysfunction (LCH-ND) often have incomplete treatment responses we sought a new treatment regimen. Because of clinical benefit from rituximab in multiple sclerosis patients with neurodegeneration, we evaluated its use in patients with LCH-ND.

Participants: Eight LCH-ND patients who had failed prior therapies. Read More

Neth J Med 2018 Dec;76(10):445-449

Department of Internal Medicine, Maastricht University Medical Centre, the Netherlands.

Langerhans cell histiocytosis (LCH) is a rare disorder, characterised by a monoclonal proliferation of aberrant histiocytes that accumulate in and infiltrate into different organs. When the hypothalamic-pituitary axis is involved, central diabetes insipidus (CDI) can be its first manifestation. Three cases of LCH with central diabetes insipidus were retrospectively analyzed: Case 1 is a 41-year old female presenting with polyuria and polydipsia. Read More

BMC Med Imaging 2018 Dec 19;18(1):54. Epub 2018 Dec 19.

Institute of Pathology, School of Medicine, University of Belgrade, Dr Subotica 1, 11000, Belgrade, Republic of Serbia.

Background: This is the first reported case of a primary intraosseous angioleiomyoma and the second case of a primary leiomyoma of the rib, irrespective of age. Angioleiomyomas mostly occur in patients of advanced age, in any part of the body, particularly the lower extremities and present as painful, slow-growing nodules in the dermis, subcutaneous fat or deep fascia. Other localizations, especially bone, are considered extremely rare, as well as their occurrence in paediatric patients. Read More

Pediatr Blood Cancer 2018 Dec 17:e27573. Epub 2018 Dec 17.

Infectious Disease Research Program, Center for Bone Marrow Transplantation, Department of Pediatric Hematology and Oncology, Children's University Hospital Münster, Münster, Germany.

Bartonella henselae, the causative agent of cat-scratch disease, has been recognized to be responsible for a broad range of clinical syndromes. We report the case of a patient with disseminated B. henselae infection mimicking Langerhans cell histiocytosis at presentation and its successful management with neurosurgery, prolonged antibacterial therapy, and observation. Read More

Clin Lab 2018 Oct;64(11)

Background: Castleman disease (CD) is a lymphoproliferative disorder and Langerhans cell histiocytosis (LCH) is a clonal disease of the monocyte-macrophage system. The authors describe a rare case of CD coexistent with LCH at diagnosis in one lymph node. Methods: Hematologic investigation and intrapulmonary mass biopsy were performed. Read More

J Dermatol 2019 Feb 7;46(2):161-165. Epub 2018 Dec 7.

Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Japan.

Histiocytoses, including Langerhans cell histiocytosis (LCH), juvenile or adult xanthogranuloma (AXG) and Rosai-Dorfman disease (RDD), are rare disorders characterized by the proliferation of cells derived from monocyte/macrophage lineages. A few cases of LCH coexisting with xanthogranuloma or RDD have been reported. The etiology of these diseases remains unclear. Read More

Rev Bras Ginecol Obstet 2018 Dec 7;40(12):803-807. Epub 2018 Dec 7.

Department of Anaesthesiology, Centro Hospitalar Lisboa Norte, Lisboa, Portugal.

Thrombocytopenia is the most common hemostatic change in pregnancy, but severe thrombocytopenia is rare. One of the causes, immune thrombocytopenic purpura (ITP), is characterized by increased platelet destruction by immunoglobulin G (IgG) antibodies, presenting a high risk of hemorrhage for the patient, but also for the fetus, since antibodies may cross the placenta. We present the case of a 23-year-old pregnant woman with a history of Langerhans cell histiocytosis of the mandible submitted to surgery and chemotherapy when she was 10 years old, with diagnosis of ITP since then. Read More

Cancer 2018 Dec 6. Epub 2018 Dec 6.

Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Background: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the presence of abnormal CD1a-positive (CD1a )/CD207 histiocytes. Hemophagocytic lymphohistiocytosis (HLH) represents a spectrum of hyperinflammatory syndromes typified by the dysregulated activation of the innate and adaptive immune systems. Patients with LCH, particularly those with multisystem (MS) involvement, can develop severe hyperinflammation mimicking that observed in HLH. Read More

Oman J Ophthalmol 2018 Sep-Dec;11(3):284-287

Department of Ophthalmology, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. Read More

World Neurosurg 2019 Feb 29;122:632-637. Epub 2018 Nov 29.

Department of Neurosurgery, Ebina General Hospital, Kanagawa, Japan.

Background: Langerhans cell histiocytosis (LCH) is a rare dendritic histiocytic disorder that affects the bones, especially the skull. Langerhans cell histiocytosis (LCH) developing in a burr hole site for chronic subdural hematoma is extremely rare.

Case Description: A 53-year-old man underwent a burr hole irrigation for chronic subdural hematoma, and the burr hole was covered with a burr hole button made of hydroxyapatite. Read More

Medicine (Baltimore) 2018 Nov;97(48):e13366

Department of Radiology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon, Republic of Korea.

Rationale: Langerhans' cell histiocytosis (LCH), also called histiocytosis X, is an uncommon disorder manifesting in a variety of ways. Although LCH can involve various organs including bone, skin, and lymph nodes, multisystem involvement of LCH is rare in adults.

Patient Concerns: A 31-year-old woman first presented to our hospital with left leg pain. Read More

Hematol Oncol Clin North Am 2019 Feb;33(1):163-172

Lymphoma Clinic, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA. Electronic address:

In reviewing cutaneous manifestations of various hematologic malignancies, the authors focus on secondary cutaneous lymphomas and cutaneous manifestations of histiocyte disorders. Secondary cutaneous lymphomas are defined as skin lesions that develop secondary to infiltration by systemic lymphomas with predominantly extracutaneous involvement. In their review of histiocytic disorders with skin involvement, the authors focus on Langerhans cell histiocytosis and Rosai-Dorfman disease. Read More

Zhonghua Gan Zang Bing Za Zhi 2018 Sep;26(9):702-704

Department of Infectious Diseases, Lishui People's Hospital, Lishui 323000, China.

Tuberk Toraks 2018 Sep;66(3):205-211

Clinic of Chest Diseases, Istanbul Yedikule Chest Diseases and Chest Surgery Training and Research Hospital, Istanbul, Turkey.

Introduction: Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease affecting young smokers. It is more common between the ages of 20-40 and equals the male/female ratio. Lung biopsy is the most useful methods for diagnosis. Read More

Pediatr Res 2018 Nov 24. Epub 2018 Nov 24.

Children's Cancer Research Institute, St. Anna Kinderkrebsforschung, Vienna, Austria.

Background: Langerhans cell histiocytosis (LCH) is a histiocytic disorder driven by a constitutive activation of the MAPK signaling pathway in myeloid cells. In 50-60% of cases, it is caused by the BRAFV600E mutation. There is evidence that levels of BRAFV600E in the peripheral blood of patients with LCH correlate with disease burden and could be used as marker for disease extent and response to therapy. Read More

Diagn Pathol 2018 Nov 24;13(1):94. Epub 2018 Nov 24.

Department of Pathology, The Ohio State University Wexner Medical Center, 410 W. 10th Ave, N#308, Columbus, OH, 43210, USA.

Background: Erdheim Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by widespread tissue infiltration by CD68-positive, CD1a-negative foamy histiocytes. ECD can be difficult to identify, and diagnosis relies on the presence of histiocytes with certain histologic and immunophenotypic features in an appropriate clinical and radiologic setting. Clinical signs and symptoms are variable depending on which organ systems are involved. Read More

Endoscopy 2019 Feb 23;51(2):E26-E27. Epub 2018 Nov 23.

Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Pediatr Hematol Oncol 2018 Nov 23:1-7. Epub 2018 Nov 23.

a Texas Children's Cancer and Hematology Centers, Baylor College of Medicine , Houston , TX , USA.

Tumor necrosis factor alpha (TNF-α) is produced in Langerhans cell histiocytosis (LCH) lesions and is elevated in plasma of patients with active LCH. It has been postulated that TNF-α may play a role in the pathophysiology of LCH. Etanercept, an anti-TNF-α antibody, has been used in TNF-modulated diseases such as rheumatoid arthritis (RA). Read More

Surg Neurol Int 2018 3;9:197. Epub 2018 Oct 3.

Department of Neurosurgery, Ishikawa Prefectural Central Hospital, Kanazawa, Japan.

Background: Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system; it is caused by dendritic cell proliferation, and typically occurs in children. LCH frequently appears in the pituitary stalk and rarely results in multiple enhanced lesions in the brain parenchyma.

Case Description: We present a case of a 40-year-old woman who deveolped panhypopituitarism and central diabetes insipidus in the postpartum period requiring hormone replacement therapy. Read More

Spec Care Dentist 2019 Jan 15;39(1):45-50. Epub 2018 Nov 15.

Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Gazi University, Ankara, Turkey.

Eosinophilic granuloma is the most common form of Langerhans cell histiocytosis and corresponds with bone lesions characterized by pain, rapid growth, and high tendency of recurrence after inadequate curettage. It is a rare disease that is difficult to diagnose clinically and radiographically because it mimics other odontogenic cysts and tumors. In this report, the reconstruction of an osseous defect with an iliac graft and a modified implant-supported hybrid prosthesis after surgical excision of an eosinophilic granuloma in the mandible of a 27-year-old male patient was described. Read More

Br J Dermatol 2018 Nov 15. Epub 2018 Nov 15.

Section of Dermatology and Venereology, Department of Medicine, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.

J Ultrason 2018 ;18(74):265-270

Department of Neurosurgery, Antwerp University Hospital and Antwerp University, Edegem, Belgium.

Langerhans cell histiocytosis is a tumor-like condition characterized by idiopathic proliferation of Langerhans cells. The disease may involve the skeleton as well as other organs systems. Bone involvement may be solitary or multifocal. Read More

Indian J Med Res 2018 Sep;148(3):351-352

Department of Endocrinology, Command Hospital, Lucknow 226 002, India.

Br J Haematol 2018 Nov 12;183(4):608-617. Epub 2018 Nov 12.

French Reference Centre for Langerhans Cell Histiocytosis, Trousseau Hospital, Paris, France.

Neurodegenerative (ND) complications in Langerhans cell histiocytosis (LCH) are a late-onset but dramatic sequelae for which incidence and risk factors are not well defined. Based on a national prospective registry of paediatric LCH patients, we determined the incidence rate of clinical ND LCH (cND-LCH) and analysed risk factors, taking into account disease extent and molecular characteristics. Among 1897 LCH patients, 36 (1·9%) were diagnosed with a cND-LCH. Read More