Evolution of angio-immunoblastic lymphadenopathy.
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Biomedical subjects
Publications and source records attributed to G Frizzera.
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Blue naevi are reported for what is believed to be the first time in the capsule and fibrous trabeculae of lymph nodes. In one of the two cases that nodal blue naevus was accompanied by an ordinary blue naevus in the regional skin. These blue naevi are compared with the 'naevus-cell aggregates' in nodal capsule, the histogenesis of which is currently disputed. A developmental migratory arrest of melanocytes is thought to be the histogenetic mechanism involved in the production of these blue naevi in lymph nodes.
The pathologic and clinical features of 31 cases of childhood non-Hodgkin's lymphoma (NHL) were reviewed retrospectively using Rappaport's classification and a modification of the Ann Arbor staging system. Twenty-nine (93.5%) of the patients had diffuse and 2 (6.5%) had nodular lymphoma. Diffuse histiocytic lymphoma accounted for 10 cases (32.3%), diffuse undifferentiated for 9 (29%), and diffuse lymphocytic, poorly differentiated for 5 (16.1%). Five cases (16.1%) were unclassifiable. No cases of well-differenitated lymphocytic or mixed cell lymphoma were found. A modified classification was attempted, which included also large basophilic cell (LBC), convoluted T-lymphocytic (CTL), and Burkitt's lymphomas. These pathologic subgroups accounted for 35.4%, 16.1%, and 6.5% of the cases, respectively. The patients were almost equally divided between clinically localized and generalized stages, and their survival was stage-dependent. The overall survival was 32.3%; the 3-year survival was 50% for Stages I and II, compared to 7.7% for Stages III and IV. The gastrointestinal tract was the most common site of origin. In 22% of the cases, the disease originated in extra-lymphatic tissues. Central nervous syste, involvement occurred in 10 of 31 children (32%), and a leukemic picture developed in 6 of 31 (19%). The Ctl lymphomas were confined to the mediastinum, whereas the LBC lymphomas arose mostly in Waldeyer's ring and Peyer's patches. We conclude that the extent of the disease as determined by clinical staging had prognostic significance in childhood NHL. The prognostic value of the histological classification could not be clearly established from our data.
The clinical and pathologic findings in 24 patients with "angio-immunoblastic lymphadenopathy with dysproteinemia" (AILD) are presented. The patients' ages ranged from 44 to 80 years, with a median age of 68 years. The disease has an acute onset. In many respects, the clinical presentation is suggestive of malignant lymphoma. Generalized lymphadenopathy was always present. Hepatomegaly was found in 20 patients, splenomegaly in 17, constitutional symptoms in 20 and skin rashes in nine. Twenty patients had anemia, with positive Coombs' test in eight of 14 tested. Polyclonal hypergammaglobulinemia was found in 17 of 22 patients. Two patterns of evolution were recognizable: (1) long survival (24 to 67 months) without treatment or after the administration of intensive combination chemotherapy; and (2) rapid progression (one to 19 months) regardless of the treatment given. Sixteen patients died; postmortem examination in 10 cases showed the cause of death to be attributable to severe infection in eight patients, to renal disease in one and to cardiovascular disease in one. No evidence of malignant lymphoma was seen in any of these autopsies. Histologically, the disease is systemic, with specific lesions in the lymph nodes. The spleen, liver, bone marrow, skin and lung are also involved, but the changes are less characteristic than in the lymph nodes. In the patients in whom sequential biopsies were performed, a trend toward restoration of the nodal architecture was observed. AILD is a clinical-pathologic entity in a spectrum of yet to be defined immune reactions. The clinical, laboratory and pathologic manifestations of AILD are consistent with an autoimmune disorder, in which a deficiency of the T-cell regulatory functions probably predisposes to an abnormal proliferative and autoaggressive reaction of the B-cell system. Surgical staging procedures do not appear to be indicated. Intensive cytotoxic treatment may be hazardous in some patients, precipitating their death, but long survival after such therapy has been observed in others. Supportive therapy and small doses of steroids appear to be a safer therapeutic approach.
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Nineteen patients with localized non-Hodgkin's lymphoma large cell type classified by the international formulation in group D (5) and group G (14) were treated with extended field radiation therapy. These patients have been followed for 168 months with range from 12 to 168 months from initiation of treatment. In G category of the formulation, the overall survival is 83 per cent at 5 and 10 years and recurrence-free survival is 75 per cent at 10 years. Sixteen of the patients were stage I and three were stage II. Two patients died without evidence of recurrence. Four patients recurred and two of these died of disease. Thirteen of the 19 patients are alive and recurrence free. Bulk of disease had no apparent influence on the response to irradiation. We believe that the early pathological stage large cell lymphoma of the G and D type international formulation are appropriate candidates for radical radiation therapy and benefit from this approach to treatment.
A case of Castleman's disease of the plasma-cell type is reported in which central nervous system (CNS) involvement was diagnosed by cerebrospinal fluid (CSF) cytology. The patient had multicentric disease with constitutional symptoms, immunologic abnormalities and peripheral blood cytopenias requiring cytotoxic agents and steroids for treatment. CNS symptoms and diagnostic cytologic findings in CSF occurred in the absence of morphologic lesions demonstrable by computed tomography or magnetic resonance imaging of the brain.
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