Effects of azithromycin in bronchiolitis obliterans syndrome after hematopoietic SCT--a randomized double-blinded placebo-controlled study.

Bronchiolitis obliterans syndrome (BOS) is an important complication after hematopoietic SCT (HSCT). Recent observations suggested that azithromycin might improve lung function in BOS after HSCT. We conducted a randomized double-blinded placebo-controlled study on azithromycin in patients with BOS after HSCT. The treatment group (n=10) received oral azithromycin 250 mg daily while the control group (n=12) received placebo daily for 12 weeks. Respiratory symptoms were assessed by the St George Respiratory Questionnaires and spirometry at baseline (drug commencement), 1, 2, 3 (drug cessation) and 4 months (1 month after drug cessation). There was no significant difference in the baseline demographic characteristics between the treatment and the control groups in age, gender, time from HSCT to BOS, time since diagnosis of BOS, chronic GVHD, baseline respiratory symptom scores and baseline forced expiratory volume in 1 s (FEV(1)). Throughout and after 3 months of treatment, there were no significant changes in respiratory symptom scores and FEV(1) measurements between the treatment and the control groups. In conclusion, there was no significant benefit of 3 months of oral azithromycin on the respiratory symptoms and lung function in patients with relatively late BOS after HSCT in this randomized placebo-controlled study.

Hepatitis C in haematological patients.

There is no consensus guideline concerning the management of chronic hepatitis C patients during chemotherapy, and immunosuppression. However, there are some suggestions in literature that hepatitis C viral load increases during chemotherapy and there is a risk of rebound immunity against hepatitis C after discontinuation of immunosuppression with a consequent liver injury. A close monitoring of liver function of these patients is prudent during treatment of haematological malignancy. Antiviral treatment is deferred after the completion of chemotherapy and recovery of patients' immunity to minimize the toxicity of treatment. A combination of pegylated interferon and ribavirin is the standard therapy in hepatitis C infected haematological patients.

An update in management of noncutaneous T-cell lymphomas.

T-cell lymphoma is a heterogeneous group of diseases. Except for ALK positive anaplastic large cell lymphoma, T-cell lymphoma responds to conventional chemotherapy unfavourably, and most patients carry poor prognosis. In recent years, efforts have been made to improve the outcome of T-cell lymphoma patients. Novel agents, high-dose therapy, and allogeneic stem cell transplantation are studied, and various results are reported in literature. This paper looks into the prognostication and treatment approach of different entities of noncutaneous T-cell lymphoma and would focus on the latest updates in its management.

A case of mixed adult Wilms' tumour and angiosarcoma responsive to carboplatin, etoposide and vincristine (CEO).

Here we report an unusual case of mixed Wilms' tumour and angiosarcoma in a 38-year-old female patient who presented with haematuria and right lower back pain. A computed tomographic (CT) scan confirmed a massive renal tumour associated with extensive retroperitoneal lymph node involvement, bony metastases and a right hip fracture. She was initially managed with palliative nephrectomy, which was followed by rapid postoperative deterioration. Histopathology revealed differentiated adult Wilms' tumour with renal angiosarcoma, whereas the pathology of the para-aortic lymph node and bone metastasis revealed angiosarcoma only. In view of her cachexia and cytopaenia, emergency chemotherapy was initiated using a modified regimen of carboplatin, etoposide and vincristine (CEO) in preference to the more traditional but less well-tolerated VAC (vincristine, actinomycin D, cyclophosphamide). Four cycles of this protocol yielded a dramatic response on re-staging CT scan. This case suggests that highly angiogenic tumours such as angiosarcoma may be effectively palliated using agents usually reserved for refractory Wilms' tumour, and supports the view that adult Wilms' tumour is more sensitive to such agents.

Hodgkin's lymphoma in Chinese migrants to British Columbia: a 25-year survey.

BACKGROUND: Compared with the West, Hodgkin's lymphoma in Oriental countries is characterized by a lower incidence rate and a higher proportion of mixed cellularity histology. Both environmental and genetic factors may be involved. PATIENTS AND METHODS: The incidence and pattern of pathology of Hodgkin's lymphoma in the migrant Chinese population (0.4 million) in British Columbia (population 3.2 million) were studied. From a computerized database, all Hodgkin's lymphoma cases diagnosed in British Columbia from 1970 to 1997 were identified. Chinese descent was determined using patient surname by standard methodology and verified from the treatment record or by patient interview. The corresponding figures from the Chinese population in Hong Kong were used for comparison. For incidence rates, the age-specific incidence of Hodgkin's lymphoma in Hong Kong was obtained from the government cancer registry. For comparison of histology subtypes, 200 Hodgkin's lymphoma records from a Hong Kong regional referral center for the same time period were reviewed. Crude and age-standardized incidence rates were calculated by 5-year intervals in terms of age and calendar year, and relative rates were compared between the three populations. RESULTS: From 1970 to 1997, Hodgkin's lymphoma was diagnosed in 34 Chinese patients in BC, with 24 cases diagnosed from 1970 to 1994. Thus, the crude and age-adjusted incidence rates from 1970 to 1994 were 0.91 and 1.14 per 100,000 per year in the British Columbia Chinese migrant population. Within the same period, 1862 cases of Hodgkin's lymphoma were diagnosed in British Columbia, giving a provincial background crude and age-adjusted incidence rates of 5.2 and 4.87 per 100,000 per year. The number of cases in the Hong Kong Chinese population (1970-1994) was 404, giving crude and age-adjusted incidence rates of 0.32 and 0.31 per 100,000 per year, respectively. Corrected for age and calendar year trends, the observed 25-year incidence of Hodgkin's lymphoma in British Columbia Chinese was significantly lower than expected from the British Columbia background population [24 observed versus 71 expected cases; standardized incidence ratio (SIR) = 0.34; 90% confidence interval (CI) 0.24-0.48; P <0.0001]. On the other hand, it is higher than that expected by extrapolating from the Hong Kong Chinese population (24 observed versus 8.5 expected cases; SIR = 2.81; 90% CI 1.94-3.95; P <0.0001). The difference is mainly accounted for by young patients with nodular sclerosis type disease in the migrant population. CONCLUSIONS: Although any conclusion about the impact of migration on Hodgkin's lymphoma incidence and types in the Chinese population must be considered tentative due to the small number of observed cases and confounding variables such as age, changing diagnostic standards and secular trends in Hodgkin's lymphoma rates, our data demonstrate a tendency for the Chinese population of British Columbia to take on a Western pattern of Hodgkin's lymphoma. This observation provides additional evidence that both genetic and environmental influences play a role in the pathogenesis of this lymphoma, and that environmental factors can exert their influence over a relatively short period of time.

Can defects in transferrin receptor 2 and hereditary hemochromatosis genes account for iron overload in HbH disease?

Iron overload was found to be the major cause of disability in Chinese HbH disease patients although they were not on regular blood transfusion. The transferrin receptor 2 (TFR2) and hereditary hemochromatosis (HFE) genes were examined to see if inheritance of these gene defects may be a possible cause of iron overload in 45 HbH patients. A novel intronic (IVS6 (+6) T-->A) mutation of the TFR2 gene was identified in one patient, and six others were found to carry a known missense mutation (exon 5, I238M) that was also present in one normal control subject. One HbH patient and one normal control carried the H63D mutation of the HFE gene. Since only eight out of 45 iron-overloaded HbH patients carry a defect in the TFR2 or HFE gene in the heterozygote state and their iron loading status was comparable to the matched controls without such defects, it would appear that the accumulation of excess iron in HbH disease is more likely a result of increase dietary absorption secondary to ineffective erythropoiesis.