Diagnosis Of Sinus Histiocytosis With Massive Lymphadenopathy Research Articles

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) and Anaplastic Large Cell Lymphoma.

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy mainly at cervical nodal sites, composed of a population of histiocytes. Since its description, over 400 cases have been described, but there are few reports in the literature on its association with lymphomas. This case report describes a case with co-existing SHML and anaplastic large cell lymphoma (ALCL). The diagnosis of SHML in our patient did not alter the clinical outcome and the patient responded well to treatment for ALCL. Clinicians should maintain a high index of suspicion in cases of infra-diaphragmatic SHML for the presence of occult lymphoma.LEARNING POINTSA rare presentation of sinus histiocytosis with massive lymphadenopathy (SHML) with anaplastic large cell lymphoma in the same lymph node at the same time is described.SHML has no proven clinical significance.A diagnosis of SHML in the presence of infra-diaphragmatic lymphadenopathy should raise a high index of suspicion for lymphoma.

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

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.

Rosai-Dorfman Disease

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and self-limiting benign disorder that most commonly involves the cervical lymph nodes. The authors describe two cases of SHML. Fine-needle aspiration of the lymphadenopathy was performed in both patients. Immunocytochemical and histologic features, as the evidence of emperipolesis and S100 protein positivity on immunostaining, were typical of SHML. Fine-needle aspiration cytology plays an important diagnostic role in SHML and may be conclusive in a typical clinical setting. The diagnosis of SHML should be considered in the differential diagnosis of massive, painless cervical lymphadenopathy. Long-term follow-up is necessary to observe the complete regression of the massive lymphadenopathy. However, specific therapy is available and should be limited to patients with compressive symptoms or extranodal disease.

Rosai-Dorfman Disease Involving the Premaxilla

Sinus histiocytosis with massive lymphadenopathy (SHML), introduced in 1969 by Rosai and Dorfman, is a benign and self-limited (albeit often relapsing) pseudolymphomatous entity with characteristic histopathologic features. Although the presence of massive cervical lymphadenopathy often aids in the diagnosis of SHML, the disease may present in the absence of detectable nodal involvement (Rosai-Dorfman disease). Therefore, the physician must be aware of such extranodal presentations. A recent review of the 365 cases in the SHML registry has revealed a preponderance of eyelid and/or orbital involvement; only 26 cases were found with subcutaneous or soft tissue involvement in areas other than the eyelid and/or orbit. This is a case report of one such atypical presentation of SHML without clinically apparent lymphadenopathy or eyelid or orbital involvement.