Abstract
ALK NEGATIVE ANAPLASTIC LARGE CELL LYMPHOMA WITH CUTANEOUS AND BONY PRESENTATION

*Dr. Seena K. Sivan and Dr. Seena A. R.

ABSTRACT

Anaplastic large cell lymphoma (ALCL) consist of a proliferation of predominantly large lymphoid cells with strong expression of cytokine receptor CD30. Two major groups of ALCL have been described. First is a spectrum of CD30+ T cell lymphoproliferative disorder. Second is systemic nodal ALCL which is again subdivided into anaplastic lymphoma kinase (ALK) positive and ALK negative systemic ALCL. ALK expression is caused by chromosomal translocation most commonly t(2;5). We report the case of a 55 year old male presented with pain in right elbow. He had an enlarged left supraclavicular node. Xray showed a lytic lesion in supracondylar region of Right Humerus. Scanning revealed multiple enlarged nodes in mediastinum. Fine needle aspiration cytology from both the sites were showing scattered cells with marked nuclear atypia, multinucleation, prominent nucleoli and varying amounts of cytoplasm. Morphologically the cells, especially because they showed clustering in areas, were suggestive of Carcinoma cells, but multiple nodes being involved, suggested a Lymphoma. Biopsy was advised for a definite opinion. Biopsy from both the sites have showed diffuse sheets of pleomorphic cells, binucleate and multinucleate cells with moderate to abundant cytoplasm. Also noted cells with prominent eosinophilic nucleoli (RS like cells) and cells with reniform nucleus. Marker studies revealed that these cells are negative for Cytokeratin and LCA positive. Next line of markers included CD 30, CD 15, PAX-5 and EMA, of which PAX-5 was negative, thus ruling out Hodgkins Lymphoma. EMA was faint and focal positive. Epithelial like cells and RS like cells along with reinform nucleus were suggestive of Anaplastic large cells lymphoma(ALCL). EMA positivity was also supported this. ALK staining was done and found to be negative. Here the case was reported as ALK negative Anaplastic Large Cell Lymphoma. Unlike ALK positive Anaplastic Large Cell Lymphoma, this type is seen in fourth and sixth decades. Both nodal and extranodal sites like bone, soft tissue and skin are affected. Cells may show cohesive pattern mimicking carcinoma. Prognosis is poorer than ALCL-ALK positive cases.

Keywords: ALK negative, anaplastic large cell lymphoma, immunohistochemistry, PAX 5 negativity, bony and cutaneous lesions.[3]


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