Langerhan's Cell Histiocytosis
Langerhan's Cell Histiocytosis (also known as Histiocytosis X) is a rare disease. It is not really a cancer, though it may behave like one in some respects. Histiocytes are normal cells found throughout the body, in this disease abnormally large numbers are found. LCH is more common in children but it is often seen in adults too. The disease will be classed as either single-system or multi-system depending on how many of the body's systems are effected.
In a study of 314 people treated for LCH at the Mayo Clinic (Howarth et al, Cancer 1999;85:2278-90) 69% had single-system disease and 31% had multi-system LCH. Approximately half of those treated at Mayo were under 25 years old at diagnosis. Of people with single-system disease the system involved was bone (52%), pulmonary (lung) (40%), skin/mucous membrane (7%) and other sites (1%). Bone involvement was more common in younger patients while pulmonary involvement was mostly seen in those over 15 years old at diagnosis.
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MeSH term: Histiocytosis, Langerhans-Cell
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Langerhans cell histiocytosis of the jaw, a mimicker of osteomyelitis on CT and MR images: A retrospective analysis.
Medicine (Baltimore). 2019; 98(27):e16331 [PubMed] Free Access to Full Article Related Publications
Langerhans Cell Histiocytosis in the Pediatric Population: Treatment of Isolated Craniofacial Lesions.
J Craniofac Surg. 2019; 30(4):1191-1193 [PubMed] Related Publications
Cervical langerhans cell histiocytosis mimicking cervical tuberculosis: A Case report.
Medicine (Baltimore). 2019; 98(20):e15690 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS: An 18-year-old male patient exhibited a 1-month history of neck pain, restricted neck mobility, and numbness and weakness of both upper limbs. The patient reported no pain at other sites, exhibited no fever or night sweats, and was unable to recall any recent injury.
DIAGNOSES: On the basis of the radiological features of the lesion and laboratory tests, there was a high possibility that the patient had a tuberculosis lesion. Postoperative GeneXpert and Mycobacterium tuberculosis (MTB) culture results showed MTB negative. Postoperative pathological results showed: (Cervical 4 vertebrae) LCH.
INTERVENTIONS: Our department did an anterior approach operation. The patient was treated with prednisone combined with vincristine after operation.
OUTCOMES: The patient was discharged from the hospital with complete remission of cervical pain and rapid relief of neurological symptoms.
LESSONS: Computed tomography-guided biopsy of lesion tissue must be performed when a suspected infection occurs in young patients. If possible, the lesion tissue obtained during the operation should be cultured and pathologically examined for early diagnosis.
Successful management of isolated pulmonary Langerhans cell histiocytosis in a 50-year-old man with early diagnosis using transbronchial cryobiopsy: A case report.
Medicine (Baltimore). 2019; 98(15):e15240 [PubMed] Free Access to Full Article Related Publications
PRESENTING CONCERNS: A 50-year-old man was referred for dry cough and exertional dyspnea of more than 1 week. High-resolution computed tomography (HRCT) of the chest revealed symmetric nodules and cyst lesions with upper lobe infiltrate. Further history taking indicated that he had smoked 20 cigarettes per day for more than 30 years. Therefore, PLCH was highly suspected. However, he refused surgical lung biopsy, and TBCB was attempted to complete diagnosis.
DIAGNOSIS: Emission computed tomography excluded the possibility of extrapulmonary involvements, and pathological findings supported the diagnosis of isolated PLCH.
INTERVENTIONS: Smoking cessation and prednisone treatment were used for patient management.
OUTCOMES: The symptoms receded with significant improvement of chest HRCT during 2-months of follow-up.
LESSONS: Early diagnosis contributes to the prognosis of isolated PLCH in adults, and TBCB may be an alternative to conventional surgical lung biopsy for pathological diagnosis of PLCH.
Pediatric Langerhans cell histiocytosis: the impact of mutational profile on clinical progression and late sequelae.
Ann Hematol. 2019; 98(7):1617-1626 [PubMed] Related Publications
Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report.
J Med Case Rep. 2019; 13(1):67 [PubMed] Free Access to Full Article Related Publications
CASE REPRESENTATION: An 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months.
CONCLUSION: The particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing's sarcoma.
Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report.
Medicine (Baltimore). 2019; 98(10):e14531 [PubMed] Free Access to Full Article Related Publications
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.
DIAGNOSES: The patient was diagnosed with langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis.
INTERVENTIONS: The patient underwent with 6 cycles of a modified etoposide, cyclophosphamide, vindesine, dexamethasone (E-CHOP) regimen.
OUTCOMES: The patient is currently receiving follow-up care.
LESSONS: LCH transformed into LCS is a rare case. E-CHOP as an effective first-line therapy to treat LCS cases, but, the mechanism is unclear. Due to their rarity, further data on clinical outcomes are necessary to establish the optimal treatment strategy for LCS.
Pediatric Langerhans cell histiocytosis: state of the science and future directions.
Clin Adv Hematol Oncol. 2019; 17(2):122-131 [PubMed] Related Publications
Characteristic Reconstitution of the Spinal Langerhans Cell Histiocytosis in Young Children.
J Pediatr Orthop. 2019; 39(4):e308-e311 [PubMed] Free Access to Full Article Related Publications
METHODS: We recruited 13 patients, including 16 affected vertebrae, diagnosed with LCH via biopsy. The average age at first visit was 3.6 years. The average follow-up period was 10.2 years. Vertebral lesions involved L2 in 3 cases; T12, L1, or L5 in 2 cases; and C4, C5, C7, T5, T8, T9, or L3 in 1 case. We measured the ratios of the height of the affected vertebra and 1 vertebra above the affected one to that of the second vertebra above the affected one, local kyphotic angles, and the ratio of the height of the center of the adjacent disk to that of one disk above it.
RESULTS: The collapse of the affected vertebra was most severe after 1 year of disease onset. The rate of reconstitution accelerated at 2 years or later of disease onset. The recovery speed of the anterior wall was faster than that of the center height. While the height of the affected vertebrae was restored, the thickness of the adjacent disk also increased. Further, the height of the adjacent vertebrae increased in a similar manner. The average local kyphosis angle shifted to lordosis within the first 3 years.
CONCLUSIONS: The heights of not only the disk but also the adjacent vertebra increased during the vertebral collapse phase in pediatric spinal LCH patients. These transformations may affect the realignment of the sagittal spinal balance at the earlier stage of the disease. During the collapse phase, the heights of the adjacent vertebrae and disks increase but after the affected vertebrae reconstituted, the augmentation of adjacent vertebrae and disks diminished.
LEVEL OF EVIDENCE: Level IV.
Diffuse Subcutaneous and Muscular Langerhans Cell Histiocytosis on FDG PET/CT.
Clin Nucl Med. 2019; 44(7):589-590 [PubMed] Related Publications
Diverse cutaneous manifestation of Langerhans cell histiocytosis: a 10-year retrospective cohort study.
Eur J Pediatr. 2019; 178(5):771-776 [PubMed] Related Publications
Remission of Congenital Multi-system Type Langerhans Cell Histiocytosis with Chemotherapy.
Acta Med Okayama. 2019; 73(1):61-65 [PubMed] Related Publications
Neonatal molar in a child with Langerhan cell histiocytosis.
J Indian Soc Pedod Prev Dent. 2019 Jan-Mar; 37(1):107-109 [PubMed] Related Publications
BRAF gene mutations in synchronous papillary thyroid carcinoma and Langerhans cell histiocytosis co-existing in the thyroid gland: a case report and literature review.
BMC Cancer. 2019; 19(1):170 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: Here we present a rare case of a 36-year-old Indonesian female patient with dysphagia associated with neck mass which was complicated by skin sinus formation. The diagnosis of PTC was rendered on fine needle aspiration (FNA). Debulking thyroidectomy revealed co-existeence of PTC and LCH. On subsequent molecular testing, BRAF V600E and V600K mutations were detected in tissues macrodissected from both lesions, respectively. To the best of our knowledge, this case is the first case to report two different BRAF mutations in tissues of a Langerhans cell histiocytosis and a papillary thyroid carcinoma co-existing in the thyroid gland. The patient received chemotherapy of etoposide combined with prednisone. At the most recent follow-up, the patient is in a stable clinical condition.
CONCLUSIONS: The coexistence of a PTC with LCH harboring BRAF mutation may suggest etiologic relation between the two conditions that involves the BRAF gene. Clinically, it may suggest an aggressive, locally advanced thyroid cancer, an impression that may reflect on the selected surgical management, chemotherapy and BRAF mutation-targeting therapy to these patients.
Pulmonary Langerhans Cell Histiocytosis: A not so Benign Lesion.
J Pediatr Hematol Oncol. 2019; 41(2):e122-e124 [PubMed] Related Publications
Outcome of children with Langerhans cell histiocytosis and single-system involvement: A retrospective study at a single center in Shanghai, China.
Pediatr Hematol Oncol. 2018 Oct - Nov; 35(7-8):385-392 [PubMed] Related Publications
PROCEDURE: 60 evaluable newly diagnosed patients (37 boys, 23 girls) with a median age of 3.9 years (range: 0.3-15.3 years) and histiopathology-confirmed SS-LCH were enrolled from 2010 to 2014. All patients received systemic chemotherapy using either the DAL HX-83 or LCH-II protocol as determined by the physician.
RESULTS: Bone was the most frequently affected organ (56/60, 93.3%). Of the 56 patients suffering from SS-bone disease, 35 (62.5%) had unifocal disease and 21 (37.5%) had multifocal disease. CNS-risk lesions were seen in nine patients (16.1%, 9/56) at diagnosis. Thirty-two patients were treated with the LCH-II protocol and 28 received the DAL HX-83 protocol. No patient received intralesional steroid injection at the time of surgery. CNS-risk lesion correlated with an inferior event-free survival (EFS) for patients with bone disease (62.5 ± 17.1% vs. 90.7 ± 4.5%; p = 0.039). The difference in the 5-year EFS between patients with unifocal and multifocal SS-bone LCH did not reach the statistical significance (93.8 ± 4.3% vs. 75.0 ± 9.7%; p = 0.074). No deaths were observed, leading to a 5-year OS of 100% in the present cohort of patients. Permanent consequences and secondary malignancies were not observed but were also limited by short follow-up.
CONCLUSIONS: Optimal therapy for patients with SS-bone LCH has not been established. Less toxic therapeutic approaches should be considered for these patients and tested in prospective trials.
Isolated hypothalamic-pituitary langerhans' cell histiocytosis in female adult: A case report.
Medicine (Baltimore). 2019; 98(2):e13853 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS: A 50-year-old woman was admitted to our hospital, presenting with polydipsia and polyuria for over 3 months.
DIAGNOSES: Radiological studies revealed lesions (0.5 × 0.9 × 0.4 cm) on posterior pituitary and enlarged pituitary stalk, which was moderately enhanced on contrast magnetic resonance imaging (MRI) of sellar region. In biopsy, pathological examination of Langerhans cells were observed with positive S-100 protein and Ki-67 antigen markers, findings were sufficient to establish a diagnosis of central nervous system (CNS) LCH.
INTERVENTIONS: The patient with LCH restricted in the sellar region received both surgery and chemotherapy. Gamma knife radiosurgery was performed after diagnosed as central diabetes insipidus (CDI) induced by pituitary lesion. And tumorectomy was performed 5 years later. However, in the latest MRI in 2017, the nodular shadow became larger (about 1.4 cm), chemotherapy and further systemic therapy were given.
OUTCOMES: At 12-month follow-up, no local reoccurrence was noticed.
LESSONS: For LCH, though difficult to be diagnosed and none defined standard therapeutic approach to adults, surgery should be considered if there are neurological symptoms or histological diagnosis. The present study showed that some manifestations can be meaningful when central nervous system (CNS) is involved. For complex diseases in the sellar region, multi-disciplinary team (MDT) model of diagnosis and treatment should be helpful for better clinical efficacy.
Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome.
Neurol Sci. 2019; 40(5):1055-1058 [PubMed] Related Publications
Rituximab therapy for patients with Langerhans cell histiocytosis-associated neurologic dysfunction.
Pediatr Hematol Oncol. 2018 Oct - Nov; 35(7-8):427-433 [PubMed] Related Publications
PARTICIPANTS: Eight LCH-ND patients who had failed prior therapies.
METHODS: Charts of the 8 patients treated with rituximab were reviewed. Signs/symptoms and MRI responses were assessed.
RESULTS: Seven of eight patients experienced some clinical improvement: gait abnormalities and tremors in four children, proprioceptive deficits in 2, and dysarthria/dysphagia in 2. Five of eight patients demonstrated improvement in intellectual/behavioral/psychological symptoms.
CONCLUSION: These findings suggest that prospective studies are warranted to define safety and efficacy of rituximab for patients with LCH-ND.
Cutaneous adult xanthogranuloma with a small portion of BRAF
J Dermatol. 2019; 46(2):161-165 [PubMed] Related Publications
Langerhans Cell Histiocytosis of the Skull in a Burr Hole Site Covered with Hydroxyapatite Material.
World Neurosurg. 2019; 122:632-637 [PubMed] Related Publications
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. Seven months after the first surgery, the connective tissue rapidly proliferated around the burr hole button, and the pathologic diagnosis was LCH. LCH recurred at 13 and 19 months after the first operation, with curettage performed each time. At 3 months after the final operation, no recurrence was identified on magnetic resonance imaging.
CONCLUSIONS: If there is rapid proliferation of connective tissue at an operative site where artificial material has been used, LCH should be considered.
Adult Langerhans' cell histiocytosis with multisystem involvement: A case report.
Medicine (Baltimore). 2018; 97(48):e13366 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS: A 31-year-old woman first presented to our hospital with left leg pain. She had a history of a 20-kg weight gain over three months.
DIAGNOSES: X-ray, magnetic resonance imaging (MRI), computed tomography (CT), and bone scan images revealed enhancing lesions in the left femur and right temporal bone, multiple cystic lesions in the lung, enhancing mass in the pituitary stalk, and fat density lesions in the liver. The patient underwent excisional biopsy for the femoral lesion and histologic examination confirmed the diagnosis of LCH.
INTERVENTIONS: Excisional biopsy was performed for the bony lesion in the left femur. She received chemotherapy with vinblastine and prednisolone.
OUTCOMES: The patient expired after 21 months from initial admission following recurrent episodes of pneumothorax, pneumonia, and sepsis.
LESSONS: Our case showed LCH involvement in bone, lung, central nervous system (CNS), and liver. Although it is occasionally difficult to discriminate LCH from other disorders, systemic evaluation might be helpful for differential diagnosis. Familiarity with the various multisystemic involvements of LCH on imaging is vital for diagnosing and managing patients in daily practice.
General features of patients with Pulmonary Langerhans Cell Histiocytosis followed in our instution.
Tuberk Toraks. 2018; 66(3):205-211 [PubMed] Related Publications
Materials and Methods: We retrospectively evaluated patients with PLCH in our clinic between January 1999 and June 2017.
Result: The female and male distribution of the 21 patients was 11/10. The average age was 35.04 ± 11.78 years. All patients were active smokers at the time of admission. The most common symptom was dyspnea. The most common finding in the pulmonary function tests was obstructive ventilatory defect. The DLCO value of the 70% patient in the carbonmonooxid diffusion test was below 80%. The most common pathologic findings detected in high-resolution chest tomography (HRCT) were cystic lesions involving bilateral upper and middle areas. There were 3 (14%) patients with pneumothorax at the time of admission and 6 (28.5%) patients with pneumothorax history before. The most common diagnostic method was open lung biopsy. All the patients quit cigarette after the diagnosis. There were 6 patients using steroid therapy, 1 patient receiving steroid and bosentan therapy, and 1 patient made pleurectomy due to recurrent pneumothorax. Lung transplantation was done to patient who received combined bosentan treatment with steroids.
Conclusions: PLCH is a rare disease and should be considered in young, smokers with spontaneous pneumothorax and cystic lung disease in the differential diagnosis. As more diffusions are affected in patients, respiratory functions for follow-up should be evaluated with diffusion tests. It is essential to quit smoking in therapy.
Anti TNF-α therapy in patients with relapsed and refractory Langerhans cell histiocytosis: a phase II study.
Pediatr Hematol Oncol. 2018 Aug - Sep; 35(5-6):362-368 [PubMed] Related Publications
Incidence and risk factors for clinical neurodegenerative Langerhans cell histiocytosis: a longitudinal cohort study.
Br J Haematol. 2018; 183(4):608-617 [PubMed] Related Publications
Langerhans cell histiocytosis complicated with hemophagocytic lymphohistiocytosis in a boy with a novel XIAP mutation: A case report.
Medicine (Baltimore). 2018; 97(44):e13019 [PubMed] Free Access to Full Article Related Publications
PATIENT CONCERNS AND DIAGNOSIS: The diagnosis of multisystem LCH was confirmed by skin biopsy and other examinations in a 13-month boy with recurrent ear discharge, fever and hemorrhagic papules for 3 months. A good therapeutic response to LCH-III protocol-directed induction chemotherapy was achieved but unremitting HLH developed 6 weeks after the initiation of induction chemotherapy. To identify possible underlying genetic causes, gene mutation analysis was undertaken. A novel XIAP gene mutation (c.99delT, p.F33fsX37) was documented.
INTERVENTIONS: After the diagnosis of HLH had been confirmed, HLH-2004-directed chemotherapy was instituted.
OUTCOMES: The clinical condition of the patient had become progressively deteriorating after 8-week chemotherapy of HLH-2004 protocol, requiring frequent infusions of RBC suspensions and apheresis platelets. His parents decided to receive no further therapy, and the patient died soon after discharge.
LESSONS: Meticulous laboratory investigations including genetic studies should be undertaken in young children with LCH complicated with HLH and poor therapeutic response.
Multisystem Involvement of Langerhans Cell Histiocytosis.
J Assoc Physicians India. 2018; 66(4):75-6 [PubMed] Related Publications
Oral Langerhans Cell Histiocytosis in an Infant.
J Dent Child (Chic). 2018; 85(2):75-78 [PubMed] Related Publications
Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment.
Cancer Sci. 2018; 109(12):3707-3713 [PubMed] Free Access to Full Article Related Publications
A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a
World Neurosurg. 2019; 121:4-11 [PubMed] Related Publications
CASE DESCRIPTION: We report the case of a 42-year-old man who had complained of right-side hemicranial pain and left arm minor paresis. The symptoms were due to a right insular lobe heterogeneous-enhancing lesion associated with extensive vasogenic edema. The first diagnostic impression suggested glioblastoma multiforme or localized metastasis. The thoracic, abdominal, pelvic computed tomography scan only detected small upper lung inactive nodules suggesting silent focal LCH. A very hard lesion was almost completely removed through a pterional craniotomy approach, with no fluorescence after aminolevulinic acid infusion. The intraoperative biopsy findings ruled out glioma but could not confirm lymphoma. The definitive cerebral biopsy findings showed lymphocytes and histiocytes (CD1a
CONCLUSIONS: The present case is so rare it should not be used as a guide. We probably will never see a single intraparenchymal supratentorial central nervous system LCH lesion. However, we hope our report will help colleagues in the future with the thought process.