Treatment of early clinically staged Hodgkin's disease with a combination of ABVD chemotherapy plus limited field radiotherapy.

The current management of early stage Hodgkin's disease (HD) is usually based on clinical staging, combined modality therapy and the use of less toxic chemotherapy regimens. This approach entails high cure rates, while ensures less long term toxicity with avoidance of laparotomy. The aim of this study was to assess the efficacy of a brief course of Adriamycin, Bleomycin, Vinblastine, Dacarbazine (ABVD) chemotherapy followed by limited field radiotherapy (RT) in favorable clinical stage (CS) I and IIA HD. Forty patients, aged 17-68 (median 34) years, with favorable CS I and IIA HD, without bulky mediastinal disease, have been treated with 4-6 (median 4) cycles of ABVD plus limited field RT. Twenty seven (67%) patients received 4 cycles of chemotherapy, while 13 received 5-6 cycles. Thirty five (87%) patients received limited field RT with dose 24-36 Gy and five (13%) received extended field with 36-46 Gy. All patients responded completely to chemotherapy. One patient experienced a relapse two months after the end of therapy. All patients are alive; 39 in continuous complete remission. With a median follow-up period of 44 months (range 18-101) the actuarial overall and progress free survival was 100 and 97% at 5 years. We did not observe any case of secondary leukemia or solid tumor. Pulmonary toxicity was mild in cases of mediastinal irradiation. Considering the short follow-up time and the small number of patients, the combination of a brief course of ABVD plus regional RT is a very efficacious treatment of favorable CS I and IIA HD with mild toxicity. However, long term survival data are needed, which could give confident answers regarding the risk of late therapy related complications, particularly second malignancies.

A complex pattern of antigen receptor gene rearrangements in a case of Philadelphia positive acute lymphoblastic leukaemia.

The paper describes a case of Philadelphia (Ph) positive acute lymphoblastic leukaemia (ALL) presenting with high white cell count and central nervous system involvement. Immunophenotypically the case was characterized as common ALL. The t(9;22) abnormality corresponded to a rearrangement within the breakpoint cluster region gene, while antigen receptor gene studies showed multiple rearrangements of the immunoglobulin heavy chain gene (IGH) concomitant with a single rearrangement of the T cell receptor beta chain gene (TCR beta). We speculate that this case represents the neoplastic transformation of a stem cell, the Ph abnormality being involved in the early steps of transformation. It is conceivable that the IGH but not the TCR beta gene was accessible to recombination within the malignant clone, thus generating the multiple rearrangements observed. If this is the case, these findings would appear to be compatible with the hypothesis that antigen receptor gene rearrangements may be partly dependent on the accessibility of the corresponding genetic loci.

Immunoglobulin abnormalities in malignant non-Hodgkin's lymphoma.

The association of monoclonal gammopathy and lymphoma was investigated in 100 consecutive, untreated cases of chronic lymphocytic leukaemia (CLL) and non-Hodgkin's lymphoma (NHL) of B-cell type, classified according to the criteria of Lukes & Collins (9). The overall incidence of monoclonal immunoglobulins (Ig) was 24%. The highest incidence (57%) was seen in plasmacytoid lymphocytic lymphoma and the lowest (7.9%) in cases of CLL. IgM was the predominant class of monoclonal Ig. It is concluded that the presence of monoclonal gammopathy in NHL reflects the stage of differentiation and maturation of the malignant B-cell clone.

Development of chronic lymphocytic leukaemia in a patient with polycythaemia vera.

A 73-year-old patient who had polycythaemia vera (PV), and who 6 years later developed chronic lymphocytic leukaemia (CCL) is described. 2 years after the appearance of CLL, the polycythaemic phase showed a remission following treatment with phlebotomy and busulfan. CLL did not make all the characteristics of PV disappear: leucocyte alkaline phosphatase and fibrosis of the bone marrow remained increased and the erythrocyte sedimentation rate remained low. The abnormal lymphocytes were B-cells and had the peculiarity that most of their nuclei showed a deep cleft, and some were bilobed. Chromosomal studies of the bone marrow showed the presence of a metacentric chromosome in the C-group, while the karyotype of the peripheral blood was normal. This case is described because of the rarity of the coexistence of these 2 diseases in the same patient.