Two Cases of C1q Nephropathy in Siblings

C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.

Histopathological Studies of 300 Cases of Non-Hodgkin's Lymphoma in Korean Patients

Non-Hodgkin's malignant lymphoma is a relatively frequent lymphoreticular malignancy, and has been reported to constitute up to 5.2% of all malignant tumors in Korean patients. Various morphologic classifications of non-Hodgkin's lymphoma have been proposed, and among them, the Rappaport's classification has been most widely accepted. In 1982, a National Cancer Institute sponsored study on classification led to the creation of the Working Formulation in an attempt to resolve the controversy anddebate regarding the various classifications of non-Hodgkin's lymphoma. Angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis are lymphoreticular proliferative disorders which have reported to transform to malignant lymphoma. The purpose of the present study is to reclassify non-Hodgkin's lymphomas according to the Working Formulation and to investigate the histopathological and immunocytochemical characteristics of angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis. This study reviewed 300 cases of nodal and extranodal non-Hodgkin's lymphoma, 26 cases of polymorphic reticulosis, and 7 cases of angioimmunoblastic lymphadenopathy with dysproteinemia examined in the Departments of Pathology, Yonsei University College of Medicine, Youngdong Severance Hospital and Yonsei University Wonju College of Medicine from January 1977 to December 1986. In non-Hodgkin's lymphoma, each case was classified according to the Working Formulation and the Rappaport classification. All angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis cases were subjected to histopathological analysis and a review of the clinical records. Immunocytochemical studies were done using kappa and lambda chains for B-cell markers and alpha-1-antichymotrypsin for histiocytic marker. The results obtained were as follows; 1) Among 300 cases of non-Hodgkin's lymphoma, the primarily involved tumor sites were the lymph nodes (141 cases), the gastrointestinal tract (67 cases), and the tonsils (32 cases) in descending order of frequency. 2) Using the Working Formulation, intermediategrade lymphomas occurred in 66.4% of the patients, and the most common subtype was "diffuse, large cell" (32.7%). By the Rappaport classification, 3 patients had nodular lymphomas, and "diffuse, histiocytic" was the most common subtype. 3) Infarction was present in 32 cases in which the "diffuse, lagre cell" type was most frequently associated. 4) In immunoperoxidase stains of 7 cases of angioimmunoblastic lymphadenopathy with dysproteinemia, proliferating immunoblasts revealed a polyclonal positivity for kappa and lambda chains. Atypical reticulocytes present in 26 cases of polymorphic reticulosis revealed a negativity for kappa, lambda and alpha-1-antichymotrypsin.

Histopathological Studies of 300 Cases of Non-Hodgkin's Lymphoma in Korean Patients

Non-Hodgkin's malignant lymphoma is a relatively frequent lymphoreticular malignancy, and has been reported to constitute up to 5.2% of all malignant tumors in Korean patients. Various morphologic classifications of non-Hodgkin's lymphoma have been proposed, and among them, the Rappaport's classification has been most widely accepted. In 1982, a National Cancer Institute sponsored study on classification led to the creation of the Working Formulation in an attempt to resolve the controversy anddebate regarding the various classifications of non-Hodgkin's lymphoma. Angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis are lymphoreticular proliferative disorders which have reported to transform to malignant lymphoma. The purpose of the present study is to reclassify non-Hodgkin's lymphomas according to the Working Formulation and to investigate the histopathological and immunocytochemical characteristics of angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis. This study reviewed 300 cases of nodal and extranodal non-Hodgkin's lymphoma, 26 cases of polymorphic reticulosis, and 7 cases of angioimmunoblastic lymphadenopathy with dysproteinemia examined in the Departments of Pathology, Yonsei University College of Medicine, Youngdong Severance Hospital and Yonsei University Wonju College of Medicine from January 1977 to December 1986. In non-Hodgkin's lymphoma, each case was classified according to the Working Formulation and the Rappaport classification. All angioimmunoblastic lymphadenopathy with dysproteinemia and polymorphic reticulosis cases were subjected to histopathological analysis and a review of the clinical records. Immunocytochemical studies were done using kappa and lambda chains for B-cell markers and alpha-1-antichymotrypsin for histiocytic marker. The results obtained were as follows; 1) Among 300 cases of non-Hodgkin's lymphoma, the primarily involved tumor sites were the lymph nodes (141 cases), the gastrointestinal tract (67 cases), and the tonsils (32 cases) in descending order of frequency. 2) Using the Working Formulation, intermediategrade lymphomas occurred in 66.4% of the patients, and the most common subtype was "diffuse, large cell" (32.7%). By the Rappaport classification, 3 patients had nodular lymphomas, and "diffuse, histiocytic" was the most common subtype. 3) Infarction was present in 32 cases in which the "diffuse, lagre cell" type was most frequently associated. 4) In immunoperoxidase stains of 7 cases of angioimmunoblastic lymphadenopathy with dysproteinemia, proliferating immunoblasts revealed a polyclonal positivity for kappa and lambda chains. Atypical reticulocytes present in 26 cases of polymorphic reticulosis revealed a negativity for kappa, lambda and alpha-1-antichymotrypsin.

Histopathological and Immunohistochemical Studies of Primary Gastrointestinal Lymphomas in Korean Patients

The present study is aimed to investigate the clinical and pathologic features of GI lymphomas and the immunocytochemical characteristics, using 71 cases of primary GI lymphomas examined at the Department of Pathology, Yonsei University College of Medicine, Youngdong Serverance Hospital and Yonsei University Wonju College of Medicine from January, 1976 to December, 1985. Results obtained were as follows: 1) Sixty eight cases were Non-Hodgkin's lymphoma and 3 Hodgkin's lymphoma. Surgical resection was done in 58 patients and surgical biopsy in 13. 2) The primary sites of the tumors were stomach, small intestine, ileocecal area and large intestine in a descending order of frequency. 3) The mean age at the time of diagnosis was 45 years. The tumor of the small intestine was generally detected in the younger age and that of the large intestine in the older age. The overall male to female ratio was 1.9 : 1. 4) Abdominal pain was the most common presenting symptom irrespective of the sites of the tumor. Duration of the symptoms were evenly distributed from hours to 12 monthes. 5) The size of the tumors was less than 10 cm in the largest dimension in most of the cases (79.3%). The gross types of the GI lymphomas were ulcerative (27.6%), polypoid (20.7%), multinodulated (17.2%) and diffuse thickening. The rate of lymph node involvement was 44.4% in tumors confined to the serosa, while 82.6% in tumors with serosal penetrations. 6) Classification of 68 cases of Non-Hodgkin's lymphoma according to Working Formulation revealed 50 cases of intermediate grade, 13 cases of high grade and 5 cases of low grade. According to Rappaport classification, all were diffuse types, among which diffuse histiocytic type was the most common. According to Lukes-Collins classification, 60 cases were B cell types, 4 cases genuine histiocytes types and 3 cases T cell types. Most of the gastric and intestinal lymphomas belonged to the follicular center cell (small and large cleaved, large cleaved) types. 7) Immunoperoxidase stains were done in 55 cases of malignant lymphomas. Thirty (65.9%) of 47 B cell lymphomas revealed a positivity for B cell markers. Twenty three (48.9%) of them showed a monoclonality, in which kappa/IgG pattern was most frequently demonstrated. Four cases of true histiocytic lymphomas were positive for alpha-1-antichymotrypsin and lysozyme, the former of which was seemed to be a more sensitive marker for histiocytic differentiation. An attempt was made to evaluate the significance of the degree of reactive histiocytic infiltrates within the lymphoma in relation to the progosis, but correlation could not be made because of the limited cases.