Cases reported "Histiocytosis, Sinus"

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1/26. Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder.

    AIMS: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Immunophenotypically the histiocytes of SHML express intensively the S100 protein and in addition a panel of macrophage-associated antigens. Their exact relationship to either monocytes/macrophages or immune accessory dendritic cells is, however, still controversial. methods AND RESULTS: In this report recurrent nodal and extranodal manifestations of SHML of a 70-year-old patient were analysed by differential phenotyping using a panel of monoclonal and polyclonal antibodies to macrophage and immune accessory dendritic cell related antigens and by applying nonradioactive in-situ hybridization. CONCLUSIONS: We conclude that stimulation of monocytes/macrophages via macrophage colony stimulating factor (M-CSF) leading to immune suppressive macrophages represents a main mechanism for the pathogenesis of SHML. The study further provides evidence for the monocyte/macrophage but not dendritic cell differentiation of SHML histiocytes.
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2/26. Cutaneous Destombes-Rosai-Dorfman disease: absence of detection of HHV-6 and HHV-8 in skin.

    BACKGROUND: We report three new cases of cutaneous Destombes-Rosai-Dorfman disease (DRDD). Two were skin-limited, and one was associated with systemic involvement. In all cases typical large S100 positive macrophages with emperipolesis were present, but different patterns were seen. A viral etiology has long been suspected in DRDD. methods: Thus, all cases were investigated for presence of HHV-6 major capsid protein gene by polymerase chain reaction (PCR), and for presence of HHV-8 latency-associated nuclear antigen 1 by immunohistochemistry. RESULTS: All results were negative. HHV-6 antibody tests were positive for IgG but not for IgM in one case. With regard to HHV-6, previous studies supplied inconclusive results while, to our knowledge, HHV-8 has never been investigated in DRDD. CONCLUSION: This study suggests that HHV-8 is not involved in the pathogenesis of cutaneous DRDD. However, this should be confirmed by further studies. We can postulate three hypotheses in regard to HHV-6 role in DRDD pathogenesis: 1) its presence in lesionnal tissues is fortuitous, 2) HHV-6 plays a role in promoting more aggressive diseases and 3) various causes, including HHV-6, are implicated in the pathogenesis of DRDD.
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3/26. Sinus histiocytosis with massive lymphoadenopathy (Rosai-Dorfman disease). Clinico-pathological analysis of a paediatric case.

    Histochemical and immunohistochemical studies performed in only a few cases of sinus histiocytosis with massive lymphoadenopathy (SHML) indicated that SHML cells belong to the macrophage--histiocyte system, though their exact origin is still uncertain. We analyzed the morphological, antigenic and enzymatic characteristics of the histiocyte-like cells in one paediatric case of SHML (also named Rosai-Dorfman disease). The SHML cells expressed the S-100 protein, lectins concanavalin a, peanut agglutinin and monocyte-macrophage related antigens CD 11c, CD 14, CD 33, CD 68 and LN 5. Reactivity with other anti-macrophage antibodies (MAC387, lysozyme, alpha-1 anti-chymotrypsin) was variable. The CD1a antigen was present only in scattered cells, whereas HLA-DR and the HLA-DR associated invariant chain were absent. Cytochemistry demonstrated an intense activity of acid phosphatase and non specific esterase of SHML cells. A large amount of medium sized mononuclear cells were located in the sinuses and intersinusoidal tissue. Our findings suggest that SHML cells have intermediate features between phagocytes and langerhans cells/interdigitating reticulum cells. The heterogeneity of marker expression on SHML cells might be related to the local content of factors (e.g., cytokines), capable of modulating the phenotype of monocyted and derived cells.
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keywords = macrophage
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4/26. Primary Rosai-Dorfman disease of bone without lymphadenopathy diagnosed by fine needle aspiration cytology. A case report.

    BACKGROUND: Solitary bone involvement without lymphadenopathy is a rare manifestation of Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML). Only 11 cases have been reported in the literature to date, all diagnosed on histology. CASE: A 7-year-old girl had a radiolucent, lytic lesion in the shaft of the tibia clinically simulating Ewing's sarcoma. Fine needle aspiration cytology (FNAC) showed a microscopic picture typical--of SHML. There was no lymphadenopathy. CONCLUSION: Rosai-Dorfman disease sometimes involves bone without lymphadenopathy and can be diagnosed confidently on FNAC. To the best of our knowledge, this is the 12th case report of solitary bone involvement.
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keywords = bone
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5/26. Rosai-Dorfman disease manifesting as multiple subcutaneous nodules. Report of a case with diagnosis on a fine needle aspirate.

    BACKGROUND: Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a benign, self-limiting histiocytic proliferative disorder commonly involving the lymph nodes. Extranodal disease occurs in about 40% of cases, and the sites involved are skin, nasal cavity, paranasal sinuses, eyelids, orbit, bone and central nervous system. CASE: A case of RDD presented as subcutaneous nodules and was diagnosed on a fine needle aspirate. The aspirate revealed numerous histiocytes with phagocytosis of lymphocytes, plasma cells and neutrophils. Surgical biopsy and immunocytochemical stain for S-100 protein confirmed the diagnosis. Later the patient developed lymphadenopathy and involvement of the nasal septum. CONCLUSION: Extranodal RDD can be diagnosed by FNAC in conjunction with immunocytochemistry.
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ranking = 0.00034829383173868
keywords = bone
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6/26. Extranodal Rosai-Dorfman disease. Clinical and pathological characteristics in a patient with a pseudotumor of bone.

    We report the clinical and pathological findings of a 19-year-old Venezuelan patient with a proximal tibial lesion showing the histological and immunohistochemical characteristics of Rosai-Dorfman disease. The radiological studies showed a lithic bone lesion that was interpreted as a giant cell tumor.
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ranking = 0.0017414691586934
keywords = bone
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7/26. Sinus histiocytosis with massive lymphadenopathy associated with malignant lymphoma.

    Sinus histiocytosis with massive lymphadenopathy (SHML) is a distinct benign clinicopathological entity, characterized by painless enlargement of lymph nodes due to sinus histiocytosis. Here, we report a case of SHML with diffuse large B-cell lymphoma. A 64-year-old man was admitted to our hospital because of fever. He presented with enlargement of a small cervical lymph node and huge abdominal paraaortic lymphadenopathy. Cervical lymph node biopsy revealed SHML and bone marrow biopsy showed infiltration of large B-cell lymphoma. Several cases of SHML associated with lymphoma have been documented to date, but this type of simultaneous occurrence has not yet been reported.
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ranking = 0.00034829383173868
keywords = bone
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8/26. Extranodal Rosai-Dorfman disease of bone, subcutaneous tissue and paranasal sinus mucosa with a review of its pathogenesis.

    We report an unusual case of extranodal Rosai-Dorfman disease presenting in a 36-year-old man with lesions of bone, subcutaneous tissue of the arm and maxillary sinus mucosa unassociated with lymphadenopathy or systemic symptoms. These lesions appeared metachronously within a 6-month period. The diagnostic light microscopic and immunohistochemical findings and pathogenesis of this interesting disease are discussed.
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ranking = 0.0017414691586934
keywords = bone
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9/26. Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression.

    Sinus histocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting, cervical lymphadenopathy. We present a case, a diagnostic dilemma for multiple consultation services, of an otherwise well 17-year-old boy without lymphadenopathy who, 8 months after excision of a T9 lytic vertebral lesion and epidural mass that caused cord compression, again presented with cord compression from progressive vertebral disease, recurrent epidural mass, and development of a paraspinal mass and tibial lesion. The excised vertebral and epidural lesions, 2 paraspinal biopsies, and tibial biopsy were interpreted as chronic inflammation until large histiocytes were noted, which were positive for CD68, S100 protein, fascin, and MAC387, and demonstrated characteristic emperipolesis (lymphophagocytosis) that was diagnostic of Rosai-Dorfman disease. This atypical clinical behavior and sites of involvement of multiple bones but not of lymph nodes is unusual and constitutes the aggressive end of the clinical spectrum and a rare cause for cord compression.
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ranking = 0.0017414691586934
keywords = bone
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10/26. Isolated Rosai-Dorfman disease of intracranial meninges.

    Rosai-Dorfman disease (RDD) is a non-neoplastic proliferative histiocytic disorder that primarily affects lymph nodes (sinus histiocytosis with massive lymphadenopathy). Primary RDD of the central nervous system is most uncommon. We report on a 35-year-old man with isolated RDD of the meninges overlying the left cerebral hemisphere. Presenting signs and symptoms included severe progressive ipsilateral headaches of 4 months duration, as well as laboratory evidence of mild non-specific systemic inflammatory reaction. On magnetic resonance imaging, the lesion was seen as a contrast-enhancing, plaque-like thickening of the dura mater over the left convexity,without impinging on adjacent bone or cerebral parenchyma. Meningeal biopsy revealed a mixed mononuclear infiltrate dominated by CD68( ), S100( ), CD1a(-) non-Langerhans type histiocytes on a background of fibrosis. bacteria, in particular mycobacteria, and fungi were excluded with special stains. Extensive clinical workup, encompassing computed tomography of thoracal and abdominal organs, bone marrow biopsy, and bronchoalveolar lavage failed to reveal any extracranial involvement. Laboratory tests for autoimmunity, including C- and P-antineutrophil cytoplasmic antibodies, antinuclear antibody, and serum rheumatoid factor, were negative. methylprednisolone therapy induced complete remission of symptoms, with the neuroradiologic status remaining unchanged on follow-up after 2 months. We discuss the complex clinicopathologic differential diagnosis and therapeutic issues of this rare condition. While the correct diagnosis of central nervous system RDD is unlikely to be established without invasive procedures (biopsy), a conservative therapeutic approach may be considered a legitimate option.
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ranking = 0.00069658766347736
keywords = bone
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