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    • br Introduction Mantel cell lymphoma

    2018-11-12


    Introduction Mantel cell lymphoma (MCL) is a rare B-cell non-Hodgkin\'s lymphoma with a poor long-term prognosis. Skin involvement occurs in only 2–6% of all cases of MCL, but is seen in 17% of stage IV patients. It is important for the dermatologist to realize and master the primary clinic presentation and pathologic features of the skin, as these will help in early and correct diagnosis of the disease. We report a case of blastoid mantle cell lymphoma involving skin and orbit with hypercalcemia.
    Case report A 53-year-old man presented with dark purple nodules and plaques on the left temporal region, right palpebra frontalis (Figure 1A), abdomen (Figure 1B), and bilateral lower extremities for ½ month, and the left periorbital region swelled 4 days prior to his initial visit to our department of dermatology. Physical examination revealed significant superficial lymph node enlargement and splenomegaly. Serum calcium and lactate dehydrogenase (LDH) were increased to 3.12mmol/L (normal 2.03–2.65mmol/L) and 853U/L (normal 71–231U/L), respectively. The agarose gel electrophoresis showed a monoclonal immunoglobulin IgM (κ chain). Positron emission tomography computed tomography scan showed multiple areas of abnormal uptake in lymph nodes, back wall of pharynx, and spleen. Bone marrow aspirate revealed active myeloid proliferation and 42% of lymphoblast was observed. Flow cytometry displayed CD20 (93.7%), CD22 (91.0%), human leukocyte antigen (HLA)-DR (99.3%), CD19 (82.9%), CD79a (95.2%), cytoplasma K (98.0%), CD2 (51.5%), and CD15 (32.7%). CD13, CD56, CD41, CD61, and CD42a were all negative. Histopathologic examination of the skin nodule in the neck revealed atypical lymphoid WP1130 (Figure 2B) infiltrated nodular distribution in the dermis and subcutaneous tissue (Figure 2A). Immunophenotype of the abnormal lymphocytes indicated positive reactions for L26 (Figure 2C), CD79a, Bcl-2, Cyclin D1 (Figure 2D), Mum-1 (partly), and Ki-67 (>80%), but negative for CD5 (Figure 2D), CD21, CD23, CD38, CD3, CD10, Bcl-6, UCHL-1, TdT, MPO, CD30, ALK, CD117, and CD34. The translocation t(11:14) juxtaposes the cyclin D1 gene (CCND1) at 11q13 to the immunoglobulin heavy chain (IgH) promoter locus at chromosome 14q32, which may be implicated in the pathogenesis and development of lymphomas, and can be found in virtually all cases of MCL by using fluorescence in situ hybridization (FISH) techniques. The patient\'s result of FISH analysis with the CCND1/IGH probe revealed a yellow fusion signal on the abnormal lymphocytes (Figure 3). Based on the cytological characterization of the infiltrated tumor cells (monomorphic sheets of small- to medium-sized blasts with finely dispersed chromatin mimicking lymphoblastic lymphoma) and clinical data, the diagnosis was blastoid MCL (stage IVB) with hypercalcemia. R-Hyper-CVAD chemotherapy was delivered: rituximab 700mg d0 (only 50mg was used as the patient did not tolerate it well because of chilling and fever), cyclophosphamide 1.5g q12h d1–3, vincristine 2mg d4 and d11, theprubicin (THP) 90mg d4, and dexamethasone 40mg d1–4 and d11–14. Three days later, serum calcium became normal. On the 6th day, the lesions on the temporal site of the head, abdomen, and bilateral lower extremities had significantly diminished. Edema around the orbit ameliorated, and the patient could open his eyes (Figure 1). Unfortunately, the patient eventually died of pneumonia 1 month later.
    Discussion MCL is mostly found among adults with a median age ranging from 60 to 65 years and a high male-to-female ratio. At presentation, there is involvement of lymph nodes (75%), spleen (massive splenomegaly in 45–60% of cases), liver (hepatomegaly in 35%), Waldeyer\'s ring, bone marrow (>60%), blood (13–77%), and extranodal sites, especially the gastrointestinal tract. Less commonly, skin, lung, breast, soft tissue, salivary glands, and orbit are involved. According to cytological characterization, MCL could be divided into four types: classical, blastoid, small cell, and pleomorphic. The cells of MCL are usually positive for CD5, CD43, CD19, CD20, CD22, CD79a, and CD79b, and negative for CD3, CD23, CD11c, CD10, and Bcl-6. Especially, MCL characteristically display nuclear positivity for cyclin D1 (bcl-1), which is strongly associated with all variants of this lymphoma, including the small group of CD5-negative cases.