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1/206. Simultaneous occurrence of Hodgkin's disease, nodal Langerhans' cell histiocytosis and multiple myeloma IgA(kappa).

    A 35-year-old man suffered simultaneously from nodular sclerosis Hodgkin's disease (HD), Langerhans' cell histiocytosis and multiple myeloma (MM). There was no prior history of irradiation or chemotherapy, and clinically the lymphoma was confined to cervical lymph nodes. Immunohistochemically, neoplastic lymphoma cells reacted with CD15 and CD30 markers. The patient's bone marrow exhibited a diffuse infiltration by rather atypical plasma cells showing kappa immunoglobulin light-chain restriction. At 14 months after the diagnosis, after autologous bone marrow transplantation, the clinical evolution is favourable with complete remission of the diseases. This is the first time that the coexistence of these three haematological disorders has been discussed, and only the fourth documented case of simultaneous HD and MM. Speculations about the significance of this finding are discussed.
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keywords = bone
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2/206. Labyrinthine involvement in Langerhans' cell histiocytosis.

    BACKGROUND: Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature. patients: We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy.
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3/206. Occurrence of acute nonlymphoblastic leukemia in two girls after treatment of recurrent, disseminated Langerhans cell histiocytosis.

    The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.
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4/206. Reconstruction of the mandibular condyle using transport distraction osteogenesis.

    Transport distraction osteogenesis was used to reconstruct the articulation of the temporomandibular joint in two patients. Patient 1 underwent a gap arthroplasty for release of a bony ankylosis. Patient 2 had degenerative joint disease after tumor resection. Both patients had painful, limited mouth opening and facial asymmetry as a result of condylar shortening. A reverse-L osteotomy was performed from the sigmoid notch to the posterior border of the mandible. An external transport distraction device was applied, and the segment was advanced superiorly, 1.0 mm per day, until contact was made with the glenoid fossa. After distraction, the bone was found to have remodeled, inducing a new cortical layer on the articular surface. Additionally, a pseudodisk was generated as a result of the transport distraction. The two patients tolerated the procedure well, returning to pain-free normal mouth opening and being able to masticate regular diets.
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5/206. A case of bilateral eosinophilic granuloma in the temporal bone.

    We present a case of bilateral eosinophilic granuloma in the temporal bone in a 47-year-old woman, who visited our hospital with a headache and a feeling of occlusion in her left ear. Her left tympanic membrane was slightly turbid and pure tone audiometry revealed mild left sensorineural deafness. CT disclosed a shadow of soft tissue in the left mastoid antrum and mastoid cells, which was indicative of marked destruction of the bone. Because MRI findings led us to suspect otitis media cholesteatoma, a mastoidectomy was performed. The mastoid antrum and mastoid cells were filled with easily bleeding granulation, and there was a wide range of bone deficit in the posterior cranial fossa. Histopathologically, the granulation tissue was an eosinophilic granuloma. Her postoperative clinical progress was good and she was discharged. However 2 months after discharge, she had a feeling of occlusion in the right ear and CT revealed a shadow in the right mastoid antrum and cells. Therefore, right tympanoplasty was performed and the same findings as in the left ear were obtained. A histopathological diagnosis of eosinophilic granuloma was made again. To date, there has been no recurrence.
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6/206. Expression of Fas ligand in Langerhans' cell histiocytosis: A case report of a boy with multisystem involvement.

    Previous reports of patients with Langerhans' cell histiocytosis have shown characteristics of osteolytic lesion, visceral involvement and organ dysfunction. We report a 2-year-old boy who was diagnosed as Langerhans' cell histiocytosis with a prominent hepatomegaly. X-Radiogram, computed tomography and magnetic resonance imaging revealed the osteolysis of the right iliac bone, the absence of the left eighth rib as well as the right mandible, and an enhancing mass in the left cerebellum. The data of radiography were highly suggestive of Langerhans' cell lineage. The presence of large CD1a-positive mononuclear cells associated with inflammatory cells in peripheral blood smear and bone marrow aspirate further confirmed the diagnosis. In addition, expressions of S100, CD25, CD68, CD80, CD86, and Fas ligand were identified on these cells by immunocytochemical staining. The results indicate that although these cells are activated Langerhans' cells, progression of the disease and the bone destruction could be mediated by the overt FasL expression of the cells.
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7/206. Spontaneous regression of orbital Langerhans cell granulomatosis in a three-year-old girl.

    PURPOSE: To report a case of spontaneous regression of orbital Langerhans cell granulomatosis. METHOD: Case report. A 3-year-old girl was initially examined with a 5-week history of slowly progressive blepharoptosis and periorbital swelling of the left eye. RESULTS: Computed tomographic scan showed a mass in the left orbit eroding into the left frontal bone; fine-needle aspiration confirmed diagnosis of Langerhans cell granulomatosis. After initial biopsy, the patient was treated by close observation alone. Six months after initial examination, the monostotic lesion had completely resolved. CONCLUSIONS: In some cases of monostotic Langerhans cell granulomatosis, initial biopsy followed by observation alone may allow for the spontaneous regression of the lesion. This conservative approach to treatment is an important therapeutic option that may spare the patient the adverse effects of surgical resection, radiation, or chemotherapy.
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8/206. Unifocal Langerhans' cell histiocytosis in the clivus of a child with abducens palsy and diplopia.

    histiocytosis X, described by Lichtenstein in 1953, is an uncommon disorder that is characterized by an abnormal proliferation of Langerhans' cells. The Langerhans' cell normally occurs in the epidermis and T-cell-dependent areas of lymph nodes and functions as a macrophage. histiocytosis X is predominantly a disease of childhood but can occasionally be seen in adults. eosinophilic granuloma of the skull is the most common presentation of the disease, and the associated symptoms depend on the location of the lesion. It has been reported to occur in the temporal bone, including the petrous apex. We present the first reported case, to our knowledge, of eosinophilic granuloma, or unifocal Langerhans' cell histiocytosis, in the clivus of a child.
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ranking = 21.496087680615
keywords = macrophage, bone
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9/206. Punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis: report of four new cases.

    Four new cases of punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis (LCH) are added to the four previously reported cases. All cases were shown on CT scans; plain films were rarely diagnostic. Pathologic correlation remains elusive since the usual biopsies of the LCH have been on skin or bone biopsies. A single prior pathologic study of the thymus in untreated LCH showed microscopic calcospherites. The thymic punctate calcific densities in patients with LCH may represent further accretion so that the calcospherites become macroscopic. The finding of such punctate calcific densities in an enlarged thymus of an infant with skin or bone or lung disease is strongly suggestive of LCH.
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10/206. Isolated Langerhans cell histiocytosis of the thyroid: a report of two cases with nuclear imaging-pathologic correlation.

    We describe two cases of isolated langerhans cell histiocytosis (LCH) of the thyroid gland, one of which was found in conjunction with an incidental papillary carcinoma. The first case was that of a 43-year-old man who presented with a 1- to 2-cm nodule within the left lobe of the thyroid. Fine-needle aspiration cytology revealed atypical cells with convoluted nuclei in a background of eosinophils and lymphocytes. The findings prompted a recommendation for excision secondary to the high suspicion of a hematologic malignancy. Histologic sections demonstrated LCH in association with a small focus of papillary carcinoma. The second case involved a 43-year-old woman who presented with a 1.8-cm nodule within the right lobe of the thyroid. Fine-needle aspiration in this case demonstrated abundant hemosiderin-laden macrophages, occasional lymphocytes, and a single benign sheet of follicular cells. No eosinophils were seen; however, a single group of atypical histiocytic cells with cleaved nuclei was noted. The nodule was subsequently resected. Histologic examination demonstrated LCH in association with follicular nodular hyperplasia with cystic degeneration. Immunohistochemical studies were performed in both cases, revealing CD1a and S100 immunoreactivity in the Langerhans' cells. Although LCH may occur as a manifestation of systemic disease, its occurrence as an isolated finding in the thyroid is rare. Its occurrence in association with papillary carcinoma of the thyroid is even more uncommon. We present two cases of isolated LCH of the thyroid, one of which was found in association with papillary carcinoma of the thyroid. The cytologic, histologic, immunohistochemical, and radiologic features are described in each case. The ultrastructural findings from the first case are also presented.
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ranking = 20.996087680615
keywords = macrophage
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