[Renal disorders in patients with immunoproliferative disorder].
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BACKGROUND: alpha-IFN is reported to be an effective treatment for a number of lymphoproliferative diseases. Little information is available at present on its effect in unaggressive immunoproliferative disorders. STUDY DESIGN AND RESULTS: In a prospective non randomized study, 57 patients with IgG or IgA MGUS, smouldering myeloma or stage I MM treated with alpha-IFN (3 MU 3 times a week for at least 6 months) were compared to 129 untreated similar patients. Four patients in the IFN group showed a monoclonal component reduction > 50% versus none in the control group, and 25% of patients suffered disease progression (MC increase > 50% and/or osteolytic lesions) in the IFN group as compared to 18% in the control group. CONCLUSIONS: alpha-IFN administration at the dose used is ineffective for the majority of patients with slowly proliferating immunoproliferative disorders; only a small subgroup of them may benefit from such a treatment.
Lymphocyte transformation with phytohaemagglutinin (PHA) was studied in 30 patients with immunoproliferative disorders (lymphoproliferative and plasma cell disorders).Lymphocyte transformation at 3 days was reduced in the lymphoproliferative disorders (chronic lymphocytic leukaemia (CLL), well-differentiated (lymphocytic) follicular lymphoma (FLL) and Waldenström's macroglobulinaemia (WM)), and normal in the plasma cell disorders (myelomatosis, primary systemic amyloidosis, α-chain disease and benign monoclonal gammopathy) and in idiopathic cold haemagglutinin disease. A case of plasma-cell leukaemia with increased numbers of abnormal cells in the circulation also showed reduced transformation. It is suggested that the presence in the circulation of abnormal lymphocytes (or plasma cells) accounts for the results in CLL and FLL, WM and plasma-cell leukaemia. In WM a correlation was found between the activity of the disease (expressed by the levels of IgM paraprotein) and the degree of blast transformation.The long-term (28 days) in vitro survival of lymphocytes using subconcentrations of PHA was also studied in 7 patients. The cell populations (PHA-non-responsive) of CLL and FLL, but not of WM, had a good in vitro survival, resembling in this respect the normal PHA-responsive population of lymphocytes, but they remained PHA-non-responsive after 4 weeks' culture. It is speculated that in CLL the long survival in vitro of the PHA-non-responsive (leukaemic) population corresponds to their long life-span in vivo.
Twenty-three patients, 9 with immunoblastic lymphadenopathy and 14 with atypical immunoproliferative disorders (AIPD), were studied in order to determine their clinical, radiographic, and pathologic features. Diffuse lymphadenopathy was present in all patients. Patients with immunoblastic lymphadenopathy (ILA) had an increased incidence of constitutional signs and symptoms. Radiographic findings in both groups were indistinguishable from those of malignant lymphomas. Of all the clinical characteristics examined to establish a prognostic significance, a positive history of allergies and the presence of polyclonal gammopathy were associated with a worse prognosis. Of the 22 patients treated, 15 initially received single agents and seven combination chemotherapy. The median survival of patients who received combination chemotherapy has not been reached at 38 months, while that of the patients on single agents is 10 months. Of 9 patients who achieved complete remission, 7 remain alive, while all 14 patients who failed to achieve a complete remission have already expired. Patients initially treated with combination chemotherapy tolerated treatment well and had a more favorable outcome with only three deaths (mostly secondary to tumor recurrence) in seven patients. Even though these entities are not considered malignant morphologically, their clinical behavior frequently resembles that of a lymphomatous disorder as confirmed by the short median survival of 22 months for AIPD and 24 months for ILA. Once progressive disease is documented, we recommend combination chemotherapy as initial therapy.
An immunoproliferative disorder with M component IgD/lambda and a strong decrease of the other Ig classes in the serum is reported. The peculiarity of this disorder consists in the shift from a predominant chronic lymphocytic leukaemic pattern, at the beginning, to a true myeloma with plasmacytic leukaemia IgD/lambda several months later. The possibility of the removal, in this case, of the initial precocious block with derepression or 'switch on' in transformation of the malignant lymphocyte clone and, consequently, change of a type of malignant lymphoma into another more evoluted and more malignant type is discussed.
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