A 61–year-old man developed epidermolysis bullosa acquisita (EBA) in association with an IgM paraprotein and chronic lymphatic leukaemia (CLL). Direct immunocyto-chemistry and immunoelectron microscopy confirmed the presence of IgM at the dermoepidermal junction. IgG was not present. This appears to be a unique observation in EBA and is discussed in relationship to the possible pathogenesis of the disorder.
This randomized study was designed to determine whether response to VAD chemotherapy could be prolonged by using rh α‐2b‐interferon (IFN) at a dose of 3 mU three times per week, either concurrently with VAD (VIC) or as maintenance after completion of VAD (VIF). Maintenance IFN was given for 9 months in order for the duration of IFN therapy to be similar in both groups. 72 patients were randomized between December 1988 and August 1993. The majority of patients had poor prognostic features. The objective response rate was similar in each arm, 78% in VIF and 77% in VIC. Of the 56 responders, 33 have relapsed, three died in remission, and 18 proceeded to high‐dose therapy, withdrew for other reasons or were lost to follow‐up and were censored from analysis at the relevant time point. Only two patients remain in remission (both in partial remission). Median PFS was 15 months for both VIF and VIC, compared with 16.5 months for a historic control group treated with VAD alone (n.s.), The estimated median survival in VIF was 43 months and in VIC 22 months, compared with 45 months in the historic controls (n.s.). These findings indicate that neither maintenance nor concurrent IFN prolongs response to VAD.
AbstractThirty-four patients with acute myeloblastic leukaemia were treated with DAC, a schedule containing the nitrosourea CCNU (lomustine) 200 mg/m2 given on day one of treatment, together with a standard “3 + 7” remission induction schedule of daunorubicin (DR) and cytosine arabinoside (Ara-C). The results were compared with an historical control group of 24 patients who received 3 + 7 remission induction (DA). The DAC patients were older (median age 55 years) compared with the DA patients (median age 42 years), and had a higher frequency of poor prognosis features including secondary AML and prior myelodysplasia (11/34 DAC patients versus 1/24 patients receiving DA). Overall remission induction was the same for both groups (79%), but 89% of DAC patients who achieved remission did so with one course, compared with 37% of DA patients. The cytopenic phase following a single course of DAC was only slightly longer than that of a single course of DA (26 days vs. 19.5 days). DAC also gave a higher three year actuarial survival than DA (34% vs. 11%), and a lower relapse probability (44% vs. 74%).These results support the hypothesis that chemotherapy for AML may be favoured by including agents such as CCNU, which are active against both non-cycling and cycling leukaemic stem cells, in remission induction schedules.Key Words: Remission inductionacute myeloid leukaemiadaunomycinAra-CCCNUsurvival
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