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Haematologica 2019 Apr 19. Epub 2019 Apr 19.

Division of Hematology, Mayo Clinic, Jacksonville, FL, USA;

Rosai-Dorfman disease is a rare subtype of non-Langerhans cell histiocytosis. With the last major report published in 1990, there is a paucity of contemporary data on this disease. Our objective was to report the clinicopathological features, treatments and outcomes of patients seen at a tertiary referral center. Read More

J Eur Acad Dermatol Venereol 2019 Apr 19. Epub 2019 Apr 19.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus Systémique, Syndrome des Anticorps Antiphospholipides et Histiocytoses, Paris, 75013, France.

Almost 70% of patients with Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, harbor the somatic V600E mutation of the BRAF gene. BRAF and MEK inhibitors are dramatically efficacious for treating ECD, particularly in cases of life-threatening manifestations [1]. Sarcoidosis-like disorders have been described during treatments with targeted therapies for cancers [2]. Read More

Pediatr Int 2019 Apr 17. Epub 2019 Apr 17.

Department of Pediatrics, Jichi Medical University School of Medicine, Shimotsuke, Tochigi, Japan.

J Cytol 2019 Apr-Jun;36(2):131-132

Department of Pathology, Dong-A University College of Medicine, Busan, Republic of Korea.

Am J Clin Pathol 2019 Apr 16. Epub 2019 Apr 16.

Division of Hematology and HSCT, Department of Oncology, King Abdul Aziz Medical City, Riyadh, KSA.

Objectives: The prevalence of concomitant myeloid neoplasms was recently reported to be unexpectedly high among adults without non-Langerhans cell histiocytosis (non-LCH); however, the coexistence of non-LCH with RUNX1 genetic aberration has not been reported previously.

Methods: Herein, we report a 23-year-old woman with severe pancytopenia diagnosed with non-LCH following presentation with pancytopenia and marrow examination showing histiocytosis positive for CD45, CD68, CD136, and lysozyme but negative for CD1a, langerin, and S100.

Results: Whole-exome sequencing showed RUNX1 mutation and NF1 mutation. Read More

Medicine (Baltimore) 2019 Apr;98(15):e15240

Department of Critical Care Medicine, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Rationale: Isolated pulmonary Langerhans cell histiocytosis (PLCH) is rare in adults. The gold standard diagnosis requires surgical lung biopsy. However, few cases have been diagnosed with transbronchial cryobiopsy (TBCB) sampling in the early stages of the disease, particularly in China. Read More

ANZ J Surg 2019 Apr 15. Epub 2019 Apr 15.

Department of Ear, Nose and Throat, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.

J Feline Med Surg 2019 Apr 12:1098612X19842384. Epub 2019 Apr 12.

1 Department of Veterinary Pathology, College of Veterinary Medicine, Federal University of Rio Grande do Sul, Porto Alegre, Brazil.

Objectives: The aim of this study was to report the clinical, radiographic and pathological features of pulmonary Langerhans cell histiocytosis in four cats, and carry out a literature review of feline histiocytic diseases.

Methods: Necropsy reports archived at the Department of Veterinary Pathology were reviewed. The clinical information was then obtained from the clinical records at the Veterinary Hospital. Read More

Open Access Maced J Med Sci 2019 Mar 28;7(6):1025-1028. Epub 2019 Mar 28.

Department of Anesthesiology and Intensive Care, Faculty of Medicine, Udayana University, Bali, Indonesia.

Background: Cataract is an opacification of the lens. Pediatric cataracts can be congenital or acquired. Acquired cataract including the one caused by corticosteroid used. Read More

Indian J Pathol Microbiol 2019 Apr-Jun;62(2):199-205

Department of Pathology, Regional Cancer Centre, Thiruvananthpuram, Kerala, India.

Small round cell lesions of the bone encompass a heterogeneous group of tumors and tumor-like lesions, including Ewing sarcoma, small cell osteosarcoma, mesenchymal chondrosarcoma, neuroblastoma, non-Hodgkin's lymphoma (NHL), "Ewing-like" undifferentiated round cell sarcomas, metastasizing small cell carcinoma, along with plasma cell dyscrasia and Langerhan's cell histiocytosis. At the same time, there are tumor mimics, for example, chronic osteomyelitis, which has overlapping radiologic features with Ewing sarcoma and a primary intraosseous NHL. An exact diagnosis necessitates integration of clinical, radiologic, pathologic, and ancillary test results, including immunohistochemical and molecular results. Read More

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Division of Hematology/Oncology, University of Alabama at Birmingham.

Langerhans cell histiocytosis (LCH) often has a recurrent and refractory course despite multiagent treatment modalities. Common relapse treatments include intense or prolonged cytotoxic chemotherapy regimens. There are a few prior reports that the nonsteroidal anti-inflammatory drug indomethacin demonstrated activity against bone LCH. Read More

Mol Imaging Radionucl Ther 2019 Mar;28(1):30-33

Shaukat Khanum Memorial Cancer Hospital and Research Centre, Clinic of Medical Oncology, Lahore, Pakistan

Erdheim-Chester disease (ECD) is a rare, multisystemic, idiopathic disease often associated with BRAF V600E mutation. Its diagnosis is typically delayed and challenging due to its variable manifestations. Although it has an indolent course, advanced stages can manifest fulminant behavior with multiple vital organ involvement. Read More

Contact Dermatitis 2019 Apr 2. Epub 2019 Apr 2.

Department of Dermatology, University Hospital Complex, Santiago de Compostela, Spain.

Cureus 2019 Jan 24;11(1):e3951. Epub 2019 Jan 24.

Neurosurgery, Penn State Hershey Medical Center, Hershey, USA.

Here, the authors present the first documented case of a patient developing central nervous system (CNS) vasculitis secondary to Langerhans cell histiocytosis (LCH) ultimately leading to stroke. LCH is a rare histiocytic disorder affecting males and females equally and typically presents in pediatric patients with a median age of 30 months. Presentation of the disease can be single-site or multisystem; and, classification of treatment is further demarcated by high risk and low risk depending on the organ systems involved. Read More

J Clin Anesth 2019 Mar 28;57:87-88. Epub 2019 Mar 28.

Department of Anesthesiology, Baylor College of Medicine/Texas Children's Hospital, 6701 Fannin St. A3300, Houston, TX 77030, USA. Electronic address:

Ann Hematol 2019 Mar 28. Epub 2019 Mar 28.

Institute of Pathology and Neuropathology and Comprehensive Cancer Center, University Hospital Tübingen, Liebermeisterstraße 8, 72076, Tübingen, Germany.

Langerhans cell histiocytosis (LCH) is a clonal histiocytic disorder with recurrent mutations of BRAF and MAP2K1, but data on the impact of genetic features on progression and long-term sequelae are sparse. Cases of pediatric LCH with long-term follow-up from our institution were analyzed for mutations in BRAF and MAP2K1 exons 2 and 3 by immunostaining with mutation-specific VE1 antibody, as well as allele-specific PCR and sequencing, respectively. Clinical and follow-up data were obtained from our files and a questionnaire sent to all former patients. Read More

Rev Esp Patol 2019 Apr - Jun;52(2):130-135. Epub 2018 Jul 4.

Servicio de Anatomía Patológica, Hospital Universitario Joan XXIII, Tarragona, España; Universidad Rovira i Virgili, Tarragona, España; Institut d'Investigació Sanitària Pere Virgili, Tarragona, España.

Langerhans cell histiocytosis (LCH) is a disease characterized by proliferation of CD1a+dendritic cells with local or diffuse organ compromise. The identification of recurrent gene mutations has confirmed the hypothesis of LCH as a true neoplasm. Lymphomatoid papulosis (LyP) belongs to the spectrum of CD30+primary cutaneous lymphomas. Read More

Indian J Dent Res 2019 Jan-Feb;30(1):157

Department of Anatomy and Surgical Anatomy, School of Medicine, National and Kapodistrian University of Athens, Athens,, Greece.

J Curr Ophthalmol 2019 Mar 18;31(1):109-112. Epub 2018 Apr 18.

Centers for Disease Control and Prevention, Atlanta, GA, USA.

Purpose: To describe a case of bilateral choroidal osteoma (CO) in a patient with a history of langerhans cell histiocytosis (LCH).

Methods: A 24-year-old man complaining of gradually decreasing visual acuity in both eyes is presented. He had a history of lymphadenopathy, respiratory symptoms, and pathology-proven diagnosis of LCH. Read More

J Dent Sci 2018 Jun 1;13(2):175-176. Epub 2018 May 1.

Division of Oral and Maxillofacial Surgery, Tri-Service General Hospital, Taiwan, ROC.

J Med Case Rep 2019 Mar 19;13(1):67. Epub 2019 Mar 19.

Department of Paediatric Surgery, University Hospital of Hassan II, Fez, Morocco.

Background: Langerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2-0. Read More

Vet Comp Oncol 2019 Mar 18. Epub 2019 Mar 18.

Laboratory of Veterinary Pathology, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Japan.

A cell line named FB-LCH01, derived from a dog diagnosed with Langerhans cell histiocytosis, was established and characterized. FB-LCH01 had C-shaped nucleoli, characterized by modal chromosome aberrations. The original tumor cells as well as established FB-LCH01 cells were immunopositive for HLA-DR, Iba-1 and E-cadherin, and immunonegative for CD163 and CD204, suggesting Langerhans cell origin. Read More

Curr Pulmonol Rep 2018 Jun 3;7(2):56-62. Epub 2018 Apr 3.

Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, 231 Albert Sabin Way, MSB Room 6053, ML 0564, Cincinnati OH 45267.

Purpose Of Review: Spontaneous pneumothorax (SP) is a common manifestation of patients with diffuse cystic lung diseases (DCLDs) such as lymphangieoleiomyomatosis (LAM), pulmonary Langerhans cell histiocytosis (PLCH) and Birt-Hogg-Dubé syndrome (BHD). Air travel may pose an additional risk for the development of SP. Here, we summarize the literature pertaining to air travel related SP in DCLDs in order to assist patients and clinicians in appropriate decision-making with regards to air travel. Read More

Medicine (Baltimore) 2019 Mar;98(10):e14531

Department of Nephrology, Zhejiang Provincial People's Hospital, People's Hospital Of Hangzhou Medical College, Hangzhou, China.

Rationale: Langerhans cell sarcoma (LCS) is a rare, high-grade neoplasm characterized by overtly malignant cytologic features and a poor prognosis. Herein, we present a rare case of langerhans cell histiocytosis (LCH) that later transformed into langerhans cell sarcoma 11 months after the benign mass was excised from soft tissue in the right groin.

Patient Concerns: A 41-year-old patient who presented with a mass in the right groin for 3 years earlier after being bitten by ants. Read More

J Pediatr Hematol Oncol 2019 Mar 6. Epub 2019 Mar 6.

Hematology-Oncology Division.

We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurological symptoms. Read More

Pediatr Blood Cancer 2019 Jun 8;66(6):e27680. Epub 2019 Mar 8.

Morgan Adams Foundation Pediatric Brain Tumor Research Foundation, Aurora, Colorado.

Preoperative diagnosis for tumors arising in the optic chiasm/sellar/suprasellar region in children is helpful to determine surgical necessity and approach, given the high operative risk in this area. We evaluated the ability to differentiate tumor type by preoperative neuroimaging. Thirty-eight of 53 tumors were correctly diagnosed by neuroimaging based on final pathologic diagnosis (prediction accuracy 72%). Read More

Clin Adv Hematol Oncol 2019 Feb;17(2):122-131

University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada.

Langerhans cell histiocytosis (LCH) is an inflammatory neoplasm of myeloid origin characterized by the presence of classic CD1a+/CD207+ cells. An ongoing debate over the grouping of LCH was finally settled in favor of neoplasm after the discovery of the BRAF V600E mutation in 2010. The pathologic cells were found to involve an almost universal activation of the MAPK/ERK pathway, with mutations identified in most kinases upstream of ERK (RAS/RAF/MEK). Read More

J Pediatr Orthop 2019 Apr;39(4):e308-e311

Department of Orthopedic Surgery, Yokohama City University.

Background: Pediatric Langerhans cell histiocytosis (LCH) often results in vertebral compression fracture. However, few reports have reported vertebral remodeling during the course of LCH. We aimed to investigate the longitudinal reconstitution and transformation of the affected vertebrae and the adjacent structures in young children with spinal LCH. Read More

J Med Case Rep 2019 Mar 5;13(1):51. Epub 2019 Mar 5.

Department of Orthopaedics Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.

Introduction: Xanthoma disseminatum is a very rare disease classified as a benign non-Langerhans cell histiocytosis, which is rarely associated with osteoarticular lesions. There is only a report of tumor abrasion during treatment of osteoarticular lesions of this disease, artificial joint replacement has not been reported. We describe a patient in whom bilateral total joint replacement was performed for disseminated xanthoma lesions of the hip joints. Read More

Zhonghua Bing Li Xue Za Zhi 2019 Mar;48(3):220-224

Department of Pathology, Peking Union Medical, College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing 100730, China (Huang Haijian is working on the Department of Pathology, Fujian Provincal Hospital, Provincial Clinical Medical College of Fujian Medical University, Fuzhou 350001, China).

To investigate the clinicpathological and molecular features of Erdheim-Chester disease (ECD) as well langerhans cell histiocytosis (LCH). The clinical, histopathological, molecular findings, immunophenotype, treatment and prognosis in 4 cases of ECD combined LCH were evaluated from February 2015 to September 2018 with review of the relevant literature. 2 cases were male, and 2 were female, aged from 7-55 years. Read More

Clin Nucl Med 2019 Mar 1. Epub 2019 Mar 1.

From the Department of PET Center, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.

Langerhans cell histiocytosis (LCH) is a rare cancer that represents a set of pathological outcomes due to the abnormal proliferation of Langerhans cells, which is more commonly seen in pediatric patients. FDG PET/CT findings of an adult LCH patient with extensive infiltration in the subcutaneous tissue and muscles was described in this case. Read More

Neurosurgery 2019 Mar 4. Epub 2019 Mar 4.

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota.

Background: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses.

Objective: To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation. Read More

Magn Reson Med Sci 2019 Mar 4. Epub 2019 Mar 4.

Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine.

J Clin Neurosci 2019 May 28;63:22-26. Epub 2019 Feb 28.

Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Aichi 466-8550, Japan. Electronic address:

Primary tumors of the spine are uncommon compared to metastases. The objective of the study was to examine the utility of O-arm navigation-assisted surgery for a primary spine tumor. A prospective study was performed in 18 consecutive patients who underwent O-arm navigation-assisted surgery for a primary spine tumor at our hospital between 2014 and 2017. Read More

Eur J Pediatr 2019 May 2;178(5):771-776. Epub 2019 Mar 2.

Dermatology Division, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, 40002, Thailand.

Cutaneous manifestation is a common presentation of LCH and is usually a leading clue for the disease diagnosis. Having cutaneous lesions did not show a significantly early age onset at diagnosis compared to those without skin lesions, P value = 0.71. Read More

Acta Med Okayama 2019 Feb;73(1):61-65

Department of Pediatrics, Okayama University Hospital, Okayama 700-8558, Japan.

Patients with multi-system (MS)-type langerhans cell histiocytosis (LCH) show poor outcomes, especially congenital MS LCH cases were shown in high mortality rate. We experienced a congenital case of MS LCH with high risk organs, who needed intensive respiratory support after birth. Even though intensive chemotherapy was discontinued, this patient's lung LCH lesions gradually became reduced and his respiratory condition recovered; therefore, we restarted and completed maintenance chemotherapy. Read More

Radiol Case Rep 2019 Apr 13;14(4):505-509. Epub 2019 Feb 13.

Hospital for Lung Diseases, Brezovik, Njegoseva bb, Niksic 81400, Montenegro.

Respiratory bronchiolitis-associated interstitial lung disease (ILD), desquamative interstitial pneumonia (DIP), and pulmonary Langerhans cell histiocytosis are entities of smoking-related ILD. While clinically regarded as 2 separate forms of idiopathic interstitial pneumonia, DIP, and respiratory bronchiolitis-associated ILD are thought to be representing ends of a continuous spectrum of disease that primarily affects tobacco smokers. This case report presents a 53-year-old female patient who has 58 pack-year smoking history who has been experiencing a dry cough and epigastric pains for 2 years. Read More

J Gastroenterol Hepatol 2019 Feb 27. Epub 2019 Feb 27.

Department of Pathology, University of Pamukkale, Denizli, Turkey.

Acta Derm Venereol 2019 02 27. Epub 2019 Feb 27.

Dermatology Unit, Bambino Gesù Children's Hospital, IRCCS, IT-00165 Rome, Italy.

Eur J Radiol Open 2019 14;6:97-100. Epub 2019 Feb 14.

Department of Diagnostic and Interventional Radiology and Neuroradiology of IRCCS San Matteo University Hospital Foundation, Pavia, Italy.

Langerhans cell histiocytosis (LCH) is a rare disease of the myeloid precursor cells, it predominantly occurs in the skull and long bones as unifocal bone lesions. Aneurysmal bone cysts (ABC) are benign, expansive and lytic bone. Reports of secondary ABC occurring in LCH are rare, having only been reported twice in the skull. Read More

Case Rep Pulmonol 2019 27;2019:6583842. Epub 2019 Jan 27.

Department of Internal Medicine, The Queen's Medical Center, Honolulu, HI 96813, USA.

Background: Simultaneous bilateral primary spontaneous pneumothorax (SBPSP) is an extremely rare and potentially fatal condition. Patients usually have no relevant medical conditions. Some cases, however, may have certain risk factors such as smoking, being young, and male gender. Read More

J Pediatr Hematol Oncol 2019 Feb 21. Epub 2019 Feb 21.

Department of Pediatric Hematology Oncology & BMT, Medanta The Medicity Hospital, Gurgaon, India.

J Indian Soc Pedod Prev Dent 2019 Jan-Mar;37(1):107-109

Department of Pedodontics and Preventive Dentistry, UCMS and GTB Hospital, New Delhi, India.

Teeth which erupt in the 1 month of postnatal life are known as "neonatal tooth." The incidence of these teeth ranges from 1:2000 to 1:3500 live births. Natal teeth are more common in mandibular central incisor region, followed by maxillary incisor region and mandibular canine region. Read More

BMC Cancer 2019 Feb 22;19(1):170. Epub 2019 Feb 22.

Department of Pathology, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.

Background: Langerhans cell histiocytosis (LCH) is a rare clonal disease, characterized by hyperproliferation of Langerhans cells. It may rarely involve the thyroid gland. Its association with papillary thyroid carcinoma (PTC) is extremely rare; with only few case reports available in the English literature. Read More

Pediatr Int 2019 Mar 21;61(3):315-317. Epub 2019 Feb 21.

Department of Pediatrics, Hirosaki University School of Medicine, Hirosaki, Japan.

J Pediatr Hematol Oncol 2019 Mar;41(2):e122-e124

Department of Hematology, All India Institute of, Medical Sciences (A.I.I.M.S), New Delhi, India.

Langerhans cell histiocytosis is a multisystem disease affecting young children. Lung involvement has a myriad of manifestations and the outcomes for these patients have been poorly defined. We present a 2-year-old child who presented with multisystem disease with multiple lung cysts. Read More

Saudi J Med Med Sci 2019 Jan-Apr;7(1):47-50. Epub 2018 Dec 14.

Department of Surgery, Dental and Maxillofacial Surgery Unit, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

Langerhans cell histiocytosis is a rare condition ranging in manifestation from a focal boney lesion to multisystem involvement. Several treatment modalities have been proposed including curettage, low-dose radiotherapy, chemotherapy and intralesional injection of corticosteroids. These treatment options can be used as a single or combined approach. Read More

World Neurosurg 2019 Feb 13. Epub 2019 Feb 13.

Department of Orthodontics, Manipal College of Dental Sciences, Manipal Academy of Higher education, Manipal, India.

Purpose: Spinal eosinophilic granulomas(EGs) are uncommon tumors, constituting <1% of all bone tumors. They are mostly seen in the pediatric age group while adult onset is rare. Cervical spine is an infrequent location for involvement by EG. Read More

Eur Spine J 2019 Feb 15. Epub 2019 Feb 15.

Department of Histopathology, PGIMER, Chandigarh, India.

Purpose: Juvenile xanthogranuloma (JXG) presenting as solitary vertebral body lesion is infrequently seen and usually limited to one or two levels. We report a case of an isolated JXG with extensive cervical spinal (bony and extradural) involvement in a 6-year-old child. There was a diagnostic dilemma as the radiologic and intraoperative picture resembled tuberculosis. Read More