Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy

Department of Internal Medicine, Chonnam National University Hospital, Gwangju, Korea.
Journal of Korean medical science (Impact Factor: 1.27). 09/2009; 24(4):760-2. DOI: 10.3346/jkms.2009.24.4.760
Source: PubMed


Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph node sinuses and lymphatics in extranodal sites. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. A 26-yr-old man presented with painless palpable lymph node in cervical area. Radiographic studies revealed pleural effusion with lymphadenopathy and calcification in mediastinum. The cervical lymph node biopsy showed dilated sinuses filled with histiocytes with clear cytoplasm. The cells stained positive with CD68 and S-100. These cytologic and immunohistochemical findings were considered consistent with the diagnosis of SHML.

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Available from: In-Jae Oh, Jan 07, 2014
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    • "Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease [1], is a rare pathological condition characterized by fever and lymph node enlargement. Sometimes the disease involves extra-nodal sites such as the skin, upper respiratory tract, bone, and retro-orbital tissue [2-6]. Although most patients have prominent bilateral cervical lymph node involvement, some cases do not have such findings and present as fever of unknown origin (FUO). "
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    ABSTRACT: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites. However, clinical suspicion is difficult and there is also a lack of useful diagnostic markers for this disorder prior to histological confirmation. High elevation of serum ferritin is known to be a useful diagnostic marker for various hematologic diseases, including hemophagocytic lymphohistiocytosis and lymphoma. Here, we report a case of fever of unknown origin that presented along with highly elevated serum ferritin (5,780 ng/mL), and was finally diagnosed as Rosai-Dorfman disease by lymph node biopsy.
    Annals of Laboratory Medicine 03/2012; 32(2):158-61. DOI:10.3343/alm.2012.32.2.158 · 1.48 Impact Factor
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    ABSTRACT: We report a patient with cutaneous Rosai-Dorfman disease that presented on the right buttock, a red brown plaque with yellowish areas and red-brown satellite papulas. The histopathologic exam revealed inflammatory infiltrate predominantly of lymphocytes and histiocytes with wide and clear cytoplasm containing intact inflammatory cells (emperipolesis). Any other alteration in the physical exam and laboratorial was not found. The Rosai-Dorfman disease is a rare form of histiocytic proliferation and the strictly cutaneous form is still rarer. The aetiology is unknown and the clinical and histologic correlation enables the correct diagnosis.
    Jornal Brasileiro de Patologia e Medicina Laboratorial 02/2011; 47(1):65-69. DOI:10.1590/S1676-24442011000100009
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    ABSTRACT: Rosai-Dorfman disease (RDD) is also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). It is a rare, benign, self-limiting disease of phagocytic histiocytes affecting a young age group presenting with massive painless cervical lymphadenopathy. RDD has nodal and also extra-nodal involvement with episodes of exacerbation and remissions, whose aetiology remains poorly elucidated. It is highly variable in its clinical presentation and response to treatment. Its treatment is poorly defined but the prognosis is usually favourable. Here we are reporting a rare, unusual clinical presentation of cervical lymphadenopathy and nasal mass diagnosed as RDD with cyto-histopathological correlation. Only a few such cases have been reported in the literature. We also emphasise that clinicians and pathologists should always be aware of RDD in making a differential diagnosis of cervical lymphadenopathy.
    Australasian Medical Journal 03/2014; 7(2):68-72. DOI:10.4066/AMJ.2014.1931
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