• The Korean Journal of Cytopathology
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• v.2 no.2
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• pp.153-159
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• 1991

Langerhans' cell histiocytosis (LCH), known for histiocytosis X, is a clinicopathologic entity characterized by proliferation of Langerhans' cells (LCs) throughout the body including the reticuloendothelial system, bone, and skin. LCs is currently considered as a distinct type of histlocytic cells, not primarily phagocytic in nature. Recently, we could make the diagnosis on cytologic specimen in a 3 month-old-boy and a 3 year-old-boy. The cases were diagnosed on scraping smear from the skin and fine needle aspiration cytology from the lymph node, respectively. The characteristic cytologic features of Langerhans' cells were noted in the nuclei, namely eccentric, indented, elongated, and grooved nuclei. The cells also had abundant and acidophilic cytoplasm. The cytologic diagnoses were confirmed on the biopsies from the skin and lymph node, respectively.

• The Korean Journal of Cytopathology
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• v.18 no.1
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• pp.87-91
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• 2007

Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.

• Journal of Chest Surgery
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• v.34 no.6
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• pp.506-510
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• 2001

Langerhans\` Cell Histiocytosis(LCH) is a disease of unknown origin, characterized by abnormal proliferation of Langerhans\` cells. Previously, it has been called histiocytosis X, which included eosinophilic granuloma, Hand-Sch ller-Christian\`s disease, and Letterer-Siwe disease. Any organs or tissues such as skin, lymph nodes, bone and bone marrow can be involved. However, LCH of chest wall is rarely reported in our country. We experienced a 18 month old male child, who had osteolytic lesion involving the rib with axillary lymph node metastasis. The tumor was confirmed as LCH after surgery.

• Journal of Yeungnam Medical Science
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• v.22 no.2
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• pp.259-265
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• 2005

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

• Clinical and Experimental Pediatrics
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• v.50 no.6
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• pp.524-530
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• 2007

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.

• Investigative Magnetic Resonance Imaging
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• v.24 no.1
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• pp.61-65
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• 2020

Langerhans cell histiocytosis (LCH) is generally considered a childhood disease that exhibits various nonspecific clinical and radiological manifestations that mimic infection or malignancy. Here, we present a case of LCH involving the rib in an adult patient. CT and MRI revealed an expansile lytic lesion with periosteal reaction on the left 8th rib, suggesting a malignant bone tumor. Surgical resection was performed and histopathological examination was consistent with LCH. Owing to its rare occurrence in adults and nonspecific aggressive features, LCH should be included in the differential diagnosis of an aggressive-appearing rib lesion in both adults and children.

• Journal of Korean Neurosurgical Society
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• v.47 no.6
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• pp.458-460
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• 2010

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.

• Korean Journal of Head & Neck Oncology
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• v.37 no.2
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• pp.67-69
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• 2021

Langerhans cell histiocytosis (LCH) is commonly characterized by abnormal function and differentiation or proliferation of monocytes. In LCH, granulomatous lesions, including langerine-positive histocytes and inflammatory infiltrates, can occur to all tissues, particularly well in the bones, skin, lungs, and pituitary gland. In case of external auditory canal LCH, conductive hearing loss may occur, and the most common symptom is otorrhea. Here we present a case that 49-year-old male with external auditory canal mass. Since no invasive findings were seen in radiologic study, endoscopic transcanal excision was performed and LCH was proven by pathologic report. We present this case of external auditory canal LCH with the review of literature.

• Korean Journal of Veterinary Research
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• v.62 no.4
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• pp.34.1-34.4
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• 2022

We describe four cases of feline progressive histiocytosis (FPH) including three females (one intact, two spayed) and one castrated male cat, with a mean age of 5.95 years at diagnosis. Masses were found under the skin of head, lip, neck, and vulva. Histologically, proliferative round cells had ovoid nuclei, foamy eosinophilic cytoplasm, distinct cytoplasmic processes, and numerous mitotic figures. Immunohistochemically, all cases were positive for Iba1 and MHC II (Dako). One case showed cytoplasmic positive staining for E-cadherin. To the best of our knowledge, this is the first documented report of FPH in Korea.

• Applied Microscopy
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• v.8 no.1
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• pp.17-22
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• 1978

Two cases of Histiocytosis X, a Hand-Schuller-Christian disease and a Letterer-Siwe disease, are,examined light and electron microscopically. Many of the histiocytes without appreciable lipid contained numerous rod-shaped structure identical to Langerhan's granules in the cytoplasm. The significance of this structure was discussed together with review of literature.