Cutaneous Lymphomas, especially cutaneous T cell lymphoma (CTCL), which is a new disease entity of mycosis fungoides, Sézary syndrome and related disorders, were reviewed on the data of cutaneous lymphoma study group in Japan and our department.
CTCL is one of the helper/inducer T cell malignancies. It is generally confined to the skin for a long period of time, distinct from other malignant lymphomas, and may then progress to involve the lymph node and internal organs with a fetal outcome. Thus, diagnosis and therapy of CTCL may be different from those of other malignant lymphomas.
Diagnostic characteristics of clinical, histopathological, haematological, immunological findings were discribed. Differentiation between CTCL, adult T cell leukemia/lymphoma (ATL) and benign reaction disorders were discussed. The therapy and prognosis of CTCL were also discussed on the base of the new clinical staging classification.

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Here we report two cases of Epstein-Barr virus related T-cell lymphomas manifested primarily as hemophagocytic syndrome. In case 1, although acute non-A non-B hepatitis with DIC was suspected, bone marrow aspiration was interpreted to be compatible with T cell malignancy. Autopsy revealed a widespread T cell lymphoma with prominent hemophagocytosis by macrophages. The second case was diagnosed as malignant histiocytosis on clinical symptoms and bone marrow findings. Lymph node biopsy was reported as diffuse lage cell lymphoma of T cell origin with reactive macrophages, which was confirmed at autopsy.
In both cases, approximately one in each twenty neoplastic cells contained EB virus genomes as revealed by in situ hybridization. HPS in these cases might be explained by reactivation of lymphoma related EB virus. Macrophages should be activated by both viruses and lymphokines which were produced by tumor cells.

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We report here 2 cases of T-cell rich B-cell lymphoma (TCRBCL) subsequent to autoimmune disease. Case 1 is a 45 years old woman with rheumatoid arthritis and case 2 is a 58 years old woman with Sjögren's syndrome. Both of them developed non-Hodgkin's lymphoma. Histological examination revealed that many small T-lymphocytes and scattered large B-lymphocytoid cells, immunoblast like cells were proliferated interfollicularly with small follicules in case 1 and definitive diagnosis was not accordant with among several pathologists. Large and medium sized lymphoid cells were proliferated intermingled with small lymphocytes, macrophages and myoepithelial island in parotid in case 2 and malignant lymphoma was questioned in clinically.
As for case 1, rearrangement of T-cell receptor β gene was not detected but that of immunoglobulin JH gene using Southern blotting. DNA of case 2 were extracted from paraffin embedded material and amplified by PCR. Monoclonality of B-cell lineage was demonstrated. So we came to conclude these cases were non-Hodgkin's lymphoma of B-cell phenotype and classified TCRBCL. There is a hypothesis of “biological interaction” about mechanism of onset of TCRBCL and in these 2 cases, TCRBCL was subsequent to autoimmune disease, in which immunological inbalance may present. Immunological abnormality may be involved closely in onset of certain malignant lymphoma.

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We report herein a rare autopsy case of malignant lymphoma arising in the stomach, which recurred in the superficial lymph nodes and the skin 21 years after gastrectomy.
The patient, a 46-year-old man, had a history of gastrectomy under the clinical diagnosis of gastric cancer 23 years ago. At that time, on histological examination, the resected stomach was diagnosed as peptic ulcer. About 10 years after gastrectomy, superficial lymph node swellings and therapy-resistant eruptions in the skin appeared. Twenty-one years after gastrectomy, malignant lymphoma of the submandibular lymph node was diagnosed. Reevaluation of the resected stomach and regional lymph nodes revealed the presence of atypical small lymphocytes with irregular nuclei throughout the gastric wall and in the lymph nodes, indicating neoplastic proliferation rather than inflammatory infiltrates. Seventeen months after the diagnosis of malignant lymphoma, the patient died due to disseminated intravascular coagulation and multiple organ failure.
An autopsy disclosed hepatomegaly, splenomegaly and enlargement of the systemic lymph nodes. Histologically small to medium-sized atypical lymphoid cells infiltrated in the liver, spleen, systemic lymph nodes, kidneys, lungs, pericardium, pancreas, gallbladder and adrenal glands. The most interesting finding was the presence of Hodgkin-like cells admixed with small to medium-sized atypical lymphoid cells, especially in the liver, spleen, and lymph nodes.
An immunohistochemical examination on formalin-fixed and paraffin-embedded sections revealed that atypical lymphoid cells were reacted for T-cell markers (MT-1, UCHL-1, OPD4) but Hodgkin-like cells were not reacted for those antibodies.
In conclusion, the present case is interpreted that low-grade malignant lymphoma arising in the stomach transformed into a more aggressive type after 23 years.

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