Clinical and laboratory characteristics of systemic anaplastic large cell lymphoma in Chinese patients.

BACKGROUND: Systemic anaplastic large cell lymphoma (S-ALCL) is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK) has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL. RESULTS: The majority of Chinese S-ALCL patients were young male patients (median age 26, male/female ratio 1.7) and the median age was younger than previous reports regardless of ALK expression status. The only statistically significant different clinical characteristic in S-ALCL between ALK positive (ALK+) and ALK negative (ALK-) was age, with a younger median age of 22 for ALK+ compared with 30 for ALK-. However, when pediatric patients (≤ 18) were excluded, there was no age difference between ALK+ and ALK-. The groups did not differ in the proportion of males, those with clinical stage III/IV (49 vs 51%) or those with extranodal disease (53 vs 59%). Of 73 evaluable patients, the 3-year and 5-year survival rates were 60% and 47%, respectively. Univariate analysis showed that three factors: advanced stage III/IV, lack of expression of ALK, and high Ki-67 expression, were associated with treatment failure in patients with S-ALCL. However, ALK expression correlated with improved survival only in patients younger than 14 years, while not in adult patients. In multivariate analysis, only clinical stage was an independent prognostic factor for survival. Expressions of Wilms tumor 1 (WT1) and B-cell lymphoma 2 protein (BCL-2) correlated with the expression of ALK, but they did not have prognostic significance. High Ki-67 expression was also a poor prognostic factor. CONCLUSIONS: Our results show that ALK expression alone is not sufficient to determine the outcome of ALCL and other prognostic factors must be considered. Clinical stage is an independent prognostic factor. Ki-67 expression is a promising prognostic factor.

Randomized pilot test of a lifestyle physical activity intervention for breast cancer survivors.

OBJECTIVE: This paper will report the results of a pilot test of a 6-month, 21-session intervention to increase breast cancer survivors' physical activity by teaching them to incorporate short periods of moderate activity into their daily routines (lifestyle intervention). The effect of the intervention on physical performance, quality of life, and physical activity are reported. METHODS: Sixty breast cancer survivors were randomized to either a lifestyle intervention or a standard care control group. Physical performance, quality of life (Medical outcomes study short form-36 [SF-36]), and physical activity (7-day recall and motivation readiness), were assessed at baseline and 6 months. RESULTS: The lifestyle group had significantly better performance in the 6-min walk task than the controls (p=0.005) at 6 months. The intervention had positive effects on the bodily pain (p=0.020) and general health (p=0.006) subscales from the SF-36. The lifestyle group had a greater motivational readiness for physical activity at 6-month than standard care, but no significant differences were seen between the two in terms of number of minutes of moderate or more intense physical activity or number of days on which they did > or =30 min of moderate or more intense activity. CONCLUSIONS: Despite the small sample size, the lifestyle intervention showed promise for improving physical functioning and quality of life and increasing physical activity, and should be tested in a larger randomized trial. PRACTICE IMPLICATIONS: If the lifestyle approach is shown to be effective in a larger trial, it represents a highly feasible intervention that it can be delivered to cancer survivors by health care institutions or community organizations without dedicated exercise facilities and equipment.

Idiopathic erythrocytosis and other non-clonal polycythemias.

Idiopathic erythrocytosis (IE) is characterized by an increase of red blood cell mass without an identified cause. Its diagnosis is based on the exclusion of polycythemia vera (PV), secondary acquired polycythemias and various congenital primary and secondary polycythemias. The frequency of IE has been estimated to be 1.1 per 1000 subjects, which is higher than that observed in PV. Heterogeneous mechanisms underlying IE have been suggested, including 'early' PV and unrecognized secondary or congenital polycythemia. However, the transition of a patient initially classified as IE into PV is a rare occurrence, when more sophisticated diagnostic techniques are employed. IE is a stable disease with a low thrombotic risk and a low, if any, tendency to spontaneous progression to acute leukemia or myelofibrosis. Phlebotomy in patients with IE is controversial. Myelosuppressive drugs should be avoided since their use is associated with evolution into acute leukemia in about 10% of patients.

Red cell enzymes.

Mutations leading to red cell enzyme deficiencies can be associated with diverse phenotypes that range from hemolytic anemia, methemoglobinemia, polycythemia, and neurological and developmental abnormalities. While most of these mutations occur sporadically, some such as common glucose-6-phosphate dehydrogenase (G6PD) mutants are endemic and rarely cause disease. Common G6PD mutants likely reached their prevalence because they provide some protection against severe malarial complications. In this review G6PD, pyruvate kinase, 5' nucleotidase, and cytochrome b5 reductase deficiencies will be discussed in greater detail. Limitations of commonly used screening tests for detection of these disorders will also be emphasized, as well as emerging knowledge about non-enzymatic function of the glycolytic enzymes.

Recent advances in the molecular biology of congenital polycythemias and polycythemia vera.

This review will focus on the molecular basis of certain polycythemic disorders. Primary polycythemias are characterized by acquired somatic or inherited germ-line mutations expressed within hematopoietic progenitors that cause increased accumulation of red blood cells. Polycythemia vera (PV), an acquired condition, is the most common primary polycythemia; although some progress has been made in the understanding of PV, its molecular basis remains unknown. In contrast, recent advances in delineating the molecular defects of some inherited polycythemias have greatly furthered our knowledge of the regulation of erythropoiesis and hypoxia sensing; however, more work needs to be done.

Copper deficiency masquerading as myelodysplastic syndrome.

We describe a woman with severe neutropenia and dependency on red blood cell transfusions who had previously undergone Billroth II surgery and whose bone marrow (BM) showed morphologic characteristics typical of myelodysplastic syndrome (MDS) with ringed sideroblasts. She had transient reversal of anemia and severe neutropenia after therapy with erythropoietin and granulocyte colony-stimulating factor. Because of relapse while receiving growth factors, the patient was referred for allogeneic BM transplantation. A pretransplantation nutritional evaluation revealed severe copper deficiency, and her hematologic abnormalities resolved fully with copper therapy. This case shows that copper deficiency should be an integral part of the differential diagnosis of sideroblastic MDS, even in patients not requiring parenteral nutrition.