Clinical and cytogenetic studied were done on 12 patients with lymphoblastic lymphoma. The ages of the patients ranged from 17 to 58 years (median, 24.5 years). Males predominated by a ration of 5:1. Ten of the 12 patients had a mediastinal mass at diagnosis; two thirds of the patients had involvement of the bone marrow (3 of 12 patients initially were in a leukemic phase), and 5 of 12 patients had involvement of the central nervous system (CNS) with lymphoma. In all patients, the malignant cells were characterized by nuclear convolutions. Cytogenetic studies done on four patients showed clonal chromosome abnormalities in two cases. Six of the 12 patients were treated with the combination chemotherapy regimen COPA and received CNS prophylaxis; their survival was slightly longer than that of the other patients, who were treated with a variety of treatment regimens. Two of the 12 patients achieved a complete remission. The median survival of all patients was 11.5 months but was shorter in patients with initial CNS involvement or leukemia. The poor results achieved thus far in the treatment of this disease demand a new therapeutic approach.
The preferred histopathological classification of Hodgkin's disease (HD) is that suggested by Lukes and Butler as modified at the Rye Symposium; the histologic subtypes are highly reproducible and correlate well with the anatomic sites of involvement, clinical stage, and survival. The accuracy of the bipedal lymphangiogram, 67gallium scan, and ultrasonography in predicting abdominal involvement by HD is 90% , 50%, and 88%, respectively. Staging laparotomy remains the most accurate method of detecting intra-abdominal disease and has added immensely to new concepts in the management of HD. These concepts suggest that patients with nodal disease limited to the celiac axis or upper para-aortic areas (substage III1) or pathologic stage (PS) IIIS+N-A, when treated with extended field radiotherapy alone have survival rates comparable to PS IIA patients. In contrast, patients in PS IIIA with lower abdominal nodal disease (substage III2), regardless of splenic involvement, have a prognosis comparable to PS IV disease. Thus, there may only be two stages of HD, those curable with extended mantle or smaller radiotherapy fields alone, and those requiring chemotherapy with or without supplemental radiotherapy.
Detailed clinical and cytogenetic studies were performed in five patients who had abnormal hematopoiesis and an acquired deletion of an F-group chromosome. Cytogenetic analyses, with banding techniques, of cells from bone marrow, spleen, or unstimulated peripheral blood showed a partial deletion of the long arm of one chromosome 20 [del(20)(q11)] in all five patients. Three patients had myeloproliferative disorders of uncertain classification, the fourth had possible preleukemia, and the fifth had acute myelomonocytic leukemia. Although the five cases showed certain similarities, the clinical and hematologic findings seen with the 20q- abnormality were not specific. None of the patients showed evidence of polycythemia vera or idiopathic acquired refractory sideroblastic anemia, two diseases previously associated with the 20q-. Our studies indicate that the 20q- abnormality is not limited to diseases primarily affecting erythropoiesis but that it can be found in the broader spectrum of myeloid disorders. In polycythemia vera, the 20q- has sometimes been regarded as a possible result of previous therapy with cytotoxic agents; however, four of our patients were untreated when the deletion was first noted.
The results of sequential staging procedures including laparotomy, radiotherapy, and combination chemotherapy are reported for 100 patients with poorly differentiated lymphocytic (PDL) and mixed cell (M€) non-Hodgkin's lymphoma (NHL). Twelve patients were found to have localized disease, pathologic stage (PS) I or 11; 88 patients had PS I11 or IV disease. Bone marrow biopsy showed a high incidence of involvement and advanced 34% of the patients from CS I, 11, and I11 to PS IV. Staging laparotomy has a very limited role in the evaluation of these patients. All of 12 patients with PS I and I1 NHL were treated with radiotherapy; at 5 years, they had 100% survival, 80% being disease-free. Fifteen patients with PS I11 disease were treated with total nodal radiotherapy (TNRT) alone and had a median diseasefree survival of 41 months. The remaining patients with PS I11 and IV disease were treated with chemotherapy consisting of vincristine afid prednisone (V 8c P); cyclophosphamide, vincristine (Oncovin@), procarbazine, and prednisone (COPP); cyclophosphamide, vincristine (Oncovin@), adriamycin, and prednisone (COPA); or "palliative therapy", consisting of chlorambucil and prednisone. Two-year and 4-year survivals for patients with diffuse lymphoma were 93% and SO%, respectively; for patients with +2 nodular lymphoma, 80% and 30%; for patients with nodular lymphomas, 76-93% and 5076, respectively. Treatment with COPP showed no advantage over V and P, "palliative therapy," or TNRT for patients with +2 nodular and nodular disease. The likelihood of cure appears most promising for patients in complete remission (CR) with diffuse lymphoma; patients in CR with nodular lymphoma show a high rate of relapse over 5 years of observation.We conclude that staging laparotomy in PDL and MC NHL is of limited value, and that the role of aggressive chemotherapy for patients with +2 nodular and nodular lymphoma needs to be redefined.Cancer 42:88-95, 1978. HE NON-HODGKIN'S LYMPHOMAS (NHL) cancer-related deaths in the United Statesz3T are the fifth most common cause of The clinical approach to be used by the
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