Primary cutaneous CD30 positive lymphoproliferative disorders: a clinicopathologic analysis / 中华皮肤科杂志

Objective To analyze the clinicopathologic features of primary cutaneous CD30 positive lymphoproliferative disorders. Methods A clinical, pathological and immunohistochemical analysis was carried out in 4 cases of lymphomatoid papulosis and 5 cases of primary cutaneous anaplastic large cell lymphoma. Results Lymphomatoid papulosis was divided into 3 subtypes, A, B and C. The lymphomatoid papulosis of subtype A was pathologically characterized by pleomorphic anaplastic large cells or Steinberg-reed cells scattered or patchly distributed among many inflammatory cells; subtype B showed pathological changes characteristic of granuloma fungoides, and manifested as a broad infiltration zone of lymphocytes in dermis with scattered small- to middle-sized atypical gyrus-like lymphocytes; subtype C was characterized by a diffuse distribution of anaplastic large cells and could clinically subside spontaneously. Primary cutaneous anaplastic large cell lymphoma clinically manifested as subcutaneous nodules or papules, and was pathologically characterized by large, pleomorphic, round or ellipse cells with plentiful, eosinophilic or bicolor cytoplasm, large nuclei and obvious nucleoli. The neoplastic cells characteristically expressed CD30 antigen in both lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, and all the cases showed a favorable prognosis.Conclusions Primary cutaneous CD30 positive lymphoproliferative disorders are a spectrum of cutaneous T cell lymphoma with favorable prognosis, and a synthetic analysis of clinical, histopathological and immunohistochemical findings is beneficial to the diagnosis of these entities.

Primary Systemic Anaplastic Large Cell Lymphoma in a Single Korean Institution: Clinical Characteristics and Treatment Outcome

Despite advances in the characterization of anaplastic large cell lymphoma (ALCL), little data is available on Asian patients. We report here upon single Korean institution's experience regarding the clinical characteristics and outcomes of ALCL. We performed a retrospective study of 32 adults with ALCL. Most of the patients received anthracycline-based chemotherapy. Ann Arbor stage III-IV, B symptoms, high-intermediate/ high International Prognostic Index (IPI), and extranodal disease at diagnosis were present in 56%, 44%, 41%, and 63%, respectively. Compared with Western studies, the male/female ratio (4.3) was markedly higher and skin (9%) and bone involvement (9%) were less frequent. The staining results for anaplastic lymphoma kinase were positive in 6 (33%) of 18 cases available. The complete response (CR) rate was 62% (95% CI, 44-80%). With a median follow-up of 51.0 months, 5 yr overall survival was 40+/-11%. The 3 yr relapse-free survival for the 18 patients who achieved CR was 74+/-12%. Age, performance status, lactate dehydrogenase, extranodal disease sites number, and IPI were correlated with treatment response and survival. Our data suggest that Korean ALCL patients appear to have a higher male/female ratio, less frequent skin/bone involvement, and lower CR rate compared with those of Western studies.

A Case of Primary Gastric CD30-Positive Anaplastic Large-Cell Lymphoma

Gastric CD30-positive anaplastic large-cell lymphoma is a very rare disease. It is sometimes difficult to distinguish it from undifferentiated carcinoma, sarcoma and so on. We report here on a case of primary gastric anaplastic large-cell lymphoma. A 50-yr-old woman complained of epigastric pain and severe chest pain for 1 week. The gastroendoscopic examination revealed geographic mucosal irregularities with shallow ulceration at the antrum. She underwent a total gastrectomy. The gross finding of the resected stomach was an 8 x 4.5 cm sized ulceroinfiltrative lesion at the pyloric antrum along the lesser curvature. The microscopic examination revealed diffuse and solid proliferations of large atypical cells with pleomorphic nuclei. Immunohistochemically, the tumor cells were positive for CD30, vimentin and CD3, and this was a finding compatible with anaplastic large-cell lymphoma. To the best of our knowledge, this is the first such reported case in Korea.

Immunoreactivity of CD99 in Non-Hodgkin's Lymphoma: Unexpected Frequent Expression in ALK-positive Anaplastic Large Cell Lymphoma

To verify the spectrum of CD99-expressing lymphoid malignancy, an immunohistochemical study for CD99 was carried out in 182 cases of non-Hodgkin's lymphoma, including 21 lymphoblastic lymphomas, 11 small lymphocytic lymphomas, 9 mantle cell lymphomas, 12 follicular lymphomas, 37 diffuse large B cell lymphomas, 18 Burkitt's lymphomas, 28 NK/T-cell lymphomas, 8 angioimmunoblastic T-cell lymphomas, 23 peripheral T-cell lymphomas, unspecified, and 15 systemic anaplastic large cell lymphomas. CD99 was positive in all T-lymphoblastic lymphomas and in 60% of B-lymphoblastic lymphomas. Majority of T and NK cell lymphomas were negative for CD99, except anaplastic large cell lymphomas (ALCLs). Eight of 15 cases (54%) of ALCLs reacted with anti CD99 antibody. Seven of 10 (70%) ALK positive ALCLs expressed CD99, whereas only 1 of 5 (20%) ALK negative ALCLs were positive. Of the mature B-cell lymphomas, 5.4% (2/37) of diffuse large B cell lymphomas and 11.1% (2/18) of Burkitt's lymphomas expressed CD99. In conclusion, CD99 is infrequently expressed in mature B and T cell lymphomas, except ALK-positive ALCL. High expression of CD99 in ALK-positive ALCL is unexpected finding and its biologic and clinical significances have yet to be clarified.