Acute leukaemia in a defined geographic area – Incidence,clinical history and prognosis

A consecutive series of patients (1978–1981) comprising all patients with acute leukaemia from a population of 475000 inhabitants was reviewed. Thus, 94 patients were diagnosed as having acute leukaemia. No patients were lost from follow-up. The incidence figures of ALL and AML differed significantly from those of Sweden as a whole. 9 patients were < 15 years old. The median age of adult patients was 64 years, 60.8% being ≥ 60 years old. Of adult patients with AML, 20% had a preleukaemic history (chronic myeloproliferative disorders, myelodysplastic syndromes and others). None of 6 patients with leukaemia as a metamorphosis of a chronic myeloproliferative disorder achieved a complete remission. The overall remission rate of the remaining adult patients was 25%. Treated patients, 15–39 years old, with AML without any preleukaemic history, had a complete remission rate of 80% compared to 12% for patients ≥ 60 years old with the same diagnosis. Of 60 patients with ‘primary’ AML, 14 were not treated, mainly because of advanced age and complicating diseases. Most of these patients died within a week of admission.     

Chronic lymphocytic leukaemia and lymphoma in an Iranian fat-tailed sheep: a case report

A 5-year-old Iranian fat-tailed sheep was referred to the Veterinary Clinic of Shiraz University in September 2003 with a history of emaciation, fever, decreased appetite, lethargy and cough. Small cutaneous and subcutaneous nodules were palpable, especially under the ribs on both sides of the thorax. Discrete cutaneous plaques and large scabby lesions were also observed. Very large mammary gland lymph nodes were noticed on palpation. Haematological and serum biochemical values were estimated through standard haematological and biochemical techniques. In this case a normocytic–normochromic anaemia, leukocytosis and lymphocytosis were found. The concentrations of blood urea nitrogen (BUN), creatinine, cholesterol and the activities of aspartate aminotransferase (AST) and gamma-glutamyl transferase (GGT) were higher than the values reported for sheep. Necropsy findings revealed that the lymph nodes were affected in most organs. Malignant lymphoma in the kidney, heart, spleen, mammary glands, liver and bone marrow was observed. The histopathological appearance of the affected tissues varied considerably, depending upon the degree of tumour infiltration. According to the history, clinical signs, laboratory findings, necropsy findings and histopathological examination the case was diagnosed as chronic lymphocytic leukaemia and lymphoma.     

A long-term time course of colorimetric assessment of the effects of imatinib mesylate on skin pigmentation: a study of five patients

BACKGROUND: Imatinib mesylate (IM), the first-line treatment of chronic myeloid leukaemia (CML), is a tyrosine kinase inhibitor that targets those proteins involved in BCR-ABL signal transduction in CML, c-kit (KIT) and platelet-derived growth-factor (PDGFR) receptor. The use of IM has been associated with cutaneous reactions. In the last 2 years numerous studies have focused the attention on hypopigmentations, depigmentations and photosensitivity developing after the initiation of IM therapy. OBJECTIVE: The aim of this study is to evaluate the effects of IM therapy on the skin pigmentation of five patients affected by CML. METHODS: Skin pigmentation measurements were performed with a Minolta CR-200 Chromameter. results: All the studied patients show the gradual lightening of the skin on unexposed areas over the treatment with IM. In particular, this explorative colorimetric study indicates the association between IM and skin depigmentation with a significant increase of luminance value (L*) (P = 0.001) and a significant decrease of the pigmentation value (b*) (P = 0.028). CONCLUSION: Even if we do not know the clinical significance of the skin depigmentation caused by IM, the regulatory role of KIT and its ligand stem cell factor in melanocyte development and survival seems to suggest an objective mechanism of action for IM in the pathogenesis of this cutaneous depigmentation.     

Mercaptopurine in childhood leukaemia: the effects of dose escalation on thioguanine nucleotide metabolites

The current U.K. trial protocol (UKALL XI) for childhood lymphoblastic leukaemia demands mercaptopurine (MP) dose escalation in children who tolerate daily 75  mg/m² MP (100% dose) without cytopenias. The previous trial (UKALL X) did not. MP metabolism was studied in a group of UKALL XI children ( n =21) who tolerated 100% dosages and who were matched in this respect with a similar group of UKALL X children. Red blood cell MP derived thioguanine nucleotide (TGN) concentrations were measured in both groups under comparable conditions; at 75  mg/m² MP there was no significant difference. MP dose escalation in the UKALL XI children produced higher TGN concentrations (TGNs at 100% vs 125% dosages, median difference 90  pmol/8×10⁸ RBCs, 95% CI 25 to 165  pmol, P <0.02). Assayed at the time of cytopenia induced dose reduction, the UKALL XI children had accumulated significantly higher TGN concentrations than the UKALL X children (median difference 78  pmol/8×10⁸ RBCs, 95% CI 20 to 144, P <0.02). These findings indicate that dose escalation in children tolerant of 100% MP dosages produces higher peak TGN concentrations.     

Cyclosporine A alleviates severe anaemia associated with refractory large granular lymphocytic leukaemia and chronic natural killer cell lymphocytosis

Large granular lymphocytic (LGL) leukaemia and chronic natural killer cell lymphocytosis (CNKL) are chronic indolent disorders often associated with neutropenia and constitutional symptoms. Severe anaemia occurs in about 20% of patients and is currently treated with corticosteroids followed by oral cyclophosphamide in non-responders. 30% of patients fail initial measures, and salvage therapy is inadequate. We describe three transfusion-dependent patients (two with T-LGL leukaemia, one with CNKL) refractory to corticosteroids, cyclophosphamide, and in one case fludarabine. Cyclosporine A (CSA) initiation resulted in prompt transfusion-independence and was well tolerated in all patients, making it an attractive alternative therapy for this disorder.     

The profile and incidence of cancer in Down syndrome

Background Down syndrome is one of the commonest causes of intellectual disability. As life expectancy improves with early and more intensive surgical and medical treatments, people with the disorder are more likely to exhibit classic morbidity and mortality patterns and be diagnosed with diseases such as cancer. Methods A profile of cancer cases among people with Down syndrome has been compiled, based on the analysis of a linked data set that included information from the Disability Services Commission of Western Australian and the State Cancer Registry. Results and conclusions Although the total age‐ and sex‐standardized incidence ratios (SIRs) for people with Down syndrome were similar to that for the general population, SIRs for leukaemia were significantly higher while the incidence of certain other types of cancers was reduced. Overall, there was a lower incidence of solid tumours in Down syndrome, possibly reflecting the age profile of the study cohort.     

Prevalence of hepatitis C virus antibody in an area endemic for hepatitis B virus and human T cell leukaemia virus

To clarify the prevalence of concurrent infection with hepatitis C virus (HCV), hepatitis B virus (HBV) and human T cell leukaemia virus (HTLV), we measured HCV antibody in the population of a district endemic for HBV and HTLV infection. Blood samples were collected in June 1990 from 579 inhabitants of four islands of Uwa Bay in the southwest of Ehime Prefecture in Japan. Anti-HCV antibody against C100-3 protein was detected using an enzyme-linked immunosorbent assay kit (Ortho Diagnostics). Thirteen of the 579 inhabitants (2.2%) were positive for anti-HCV, and this prevalence rate was not significantly different from the frequency of anti-HCV in Tokyo blood donors. A total of 11% (64 of 579) of the subjects were positive for HBsAg and 3.3% (19 of 579) were positive for anti-HTLV. These frequencies of HBsAg and anti-HTLV positivity were distinctly higher than the respective means of Japanese. All anti-HCV positive individuals were negative for HBsAg and anti-HTLV, while 54% (7 of 13) had increased alanine aminotransferase levels. These data suggest that the prevalence of HCV infection is not high even in an area endemic for HBV and HTLV infection.     

急性白血病非系限性分化抗原的动态研究

为探讨仅表达非系限性分化抗原的急性白血病的细胞起源，采用单克隆抗体免疫酶标和聚合酶链反应技术分析了１２例初诊时仅表达非系限性分化抗原的急性白血病化疗后免疫表型及免疫球蛋白重链（ＩｇＨ）和Ｔ细胞受体（ＴＣＲ）γ基因重排的变化。结果表明：初诊时仅表达ＣＤ３８抗原的５例急性白血病，化疗后３例出现Ｔ细胞相关抗原表达，并伴ＴＣＲγ基因重排；５例初诊时仅表达ＨＬＡ－ＤＲ或ＣＤ９的急性白血病，化疗后４例出现Ｂ细胞相关抗原表达，均伴有ＩｇＨ基因重排；２例无任何抗原表达者，化疗后１例表达Ｂ细胞相关抗原，另１例表达骨髓细胞相关抗原。提示免疫标志的动态研究，有助于初诊时免疫学无法分类急性白血病细胞起源的确定。