Blastoid mantle cell lymphoma involving skin and orbit with hypercalcemia: A case report and literature review

Mantel cell lymphoma is one of the small B-cell non-Hodgkin's lymphomas and usually involves lymph nodes, bone marrow, spleen, liver, gastrointestinal tract, and Waldeyer's ring, but rarely skin and orbit. A 53-year-old man presented skin nodules and plaques on the head, trunk, and lower e...

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Main Authors: Juan Li (Author), Han Ma (Author), Xiuzhen Tong (Author), Chang Su (Author), Dong Zheng (Author), Mei Chen (Author), Chun Lu (Author)
Format: Book
Published: Wolters Kluwer Medknow Publications, 2013-06-01T00:00:00Z.
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Summary:Mantel cell lymphoma is one of the small B-cell non-Hodgkin's lymphomas and usually involves lymph nodes, bone marrow, spleen, liver, gastrointestinal tract, and Waldeyer's ring, but rarely skin and orbit. A 53-year-old man presented skin nodules and plaques on the head, trunk, and lower extremities for half a month, and the left periorbital region swelled 4 days ago. Serum calcium and lactate dehydrogenase were increased to 3.12 mmol/L (normal 2.03-2.65 mmol/L) and 853 U/L (normal 71-231 U/L), respectively. Histopathologic examination of the skin nodule revealed tumor cells infiltrated nodular distribution in the dermis and subcutaneous tissue. Immunophenotyping of the abnormal lymphocytes indicated positive reactions for L26, CD79a, Bcl-2, Cyclin D1, and Ki-67 (>80%), but negative for CD5, CD21, CD23, CD38, CD3, CD10, UCHL-1, TdT, MPO, CD30, ALK, CD117, and CD34. Fluorescence in situ hybridization analysis with the CCND1/IGH probe revealed a fusion signal on the abnormal lymphocytes. The final diagnosis was a rare case of blastoid mantle cell lymphoma involving skin and orbit with hypercalcemia. Following the R-Hyper-CVAD treatment plan, the patient achieved a quick and excellent recovery on the 6th day. Unfortunately, the patient eventually died of pneumonia one month later.
Item Description:1027-8117
10.1016/j.dsi.2012.08.006