Sole rearrangement but not amplification of MYC is associated with a poor prognosis in patients with diffuse large B cell lymphoma and B cell lymphoma unclassifiable.

Rearrangement of MYC is associated with a poor prognosis in patients with diffuse large B cell lymphoma (DLBCL) and B cell lymphoma unclassifiable (BCLU), particularly in the setting of double hit lymphoma (DHL). However, little is known about outcomes of patients who demonstrate MYC rearrangement without evidence of BCL2 or BCL6 rearrangement (single hit) or amplification (>4 copies) of MYC. We identified 87 patients with single hit lymphoma (SHL), 22 patients with MYC-amplified lymphoma (MYC amp) as well as 127 DLBCL patients without MYC rearrangement or amplification (MYC normal) and 45 patients with DHL, all treated with either R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) or intensive induction therapy. For SHL and MYC amp patients, the 2-year progression-free survival rate (PFS) was 49% and 48% and 2-year overall survival rate (OS) was 59% and 71%, respectively. SHL patients receiving intensive induction experienced higher 2-year PFS (59% vs. 23%, P = 0·006) but similar 2-year OS as compared with SHL patients receiving R-CHOP. SHL DLBCL patients treated with R-CHOP, but not intensive induction, experienced significantly lower 2-year PFS and OS (P < 0·001 for both) when compared with MYC normal patients. SHL patients appear to have a poor prognosis, which may be improved with receipt of intensive induction.

The impact of race, age, and sex in follicular lymphoma: A comprehensive SEER analysis across consecutive treatment eras.

The impact of race/ethnicity and the additional factors of age, sex, and socioeconomic status (SES) on follicular lymphoma (FL) outcomes have not been comprehensively studied and are not well defined. We examined population-based FL data from >18,000 patients in SEER-13 (1992-2009) investigating race/ethnicity and the impact of relevant factors including sex, age, and SES. Further, we compared data over two consecutive periods: Era-1 (1992-2000, n = 8,355) and Era-2 (2001-2009, n = 10,475). We identified 18,830 FL patients (White: n = 15,116; Hispanic: n = 1,627; Asian/Pacific Islander (A/PI): n = 1,002; and Black: n = 846). Median ages (years) differed significantly by race/ethnicity: White: 62.1, Hispanic: 57.3, A/PI: 60.7, and Black: 56.8 (P < 0.01 each race versus White). Overall survival (OS) was superior in Era-2 versus Era-1 for all patients (5-year: 76.7% versus 67.4%, respectively, P < 0.001). Further, survival was significantly improved for all age groups <80 years, for males (P = 0.0019), and females (P < 0.001) across eras. Females had superior OS compared with males in Era-1 (P = 0.004), but not in Era-2. Additionally, all races, except A/PI, had improved 5-year OS rates from Era-1 to Era-2. Finally, OS improved across eras for lower and higher SES populations; however those with higher SES were superior to lower SES patients in both eras. In conclusion, and in the largest comprehensive evaluation of US-based FL patients to date, we show that despite improvements in OS for FL over time, critical disparities across races/ethnicities, sex, and age groups remain in the modern era and warrant further studies.

Extramedullary chronic lymphocytic leukemia: systematic analysis of cases reported between 1975 and 2012.

The prognostic significance of extra-medullary chronic lymphocytic leukemia (EM-CLL) is unknown. We conducted a Medline database systematic search analyzing English language articles published between 1975 and 2012 identifying 192 cases. Patients with EM-CLL were more commonly treated than not (p < .001). Skin and central nervous system (CNS) were the most commonly reported sites of organ involvement. Survival after diagnosis of EM-CLL appeared to depend on the site of EM involvement. Prospective evaluation and further studies of EM-CLL are warranted.

Erlotinib eradicates brain metastases from epidermal growth factor receptor mutant non-small cell lung cancer.

Erlotinib is active in patients with lung cancer; especially those who demonstrate a mutation in exons 18-21 in the epidermal growth factor receptor (EGFR) gene. Patients with lung cancer and brain metastases have poor prognosis as systemic chemotherapy is ineffective in treating the central nervous system (CNS) metastases due to its inability to cross the blood brain barrier. Herein, we report a case of a 61 year old female who presented with stage IV adenocarcinoma of the lung with bilateral cerebral and cerebellar CNS involvement. The patient's tumor harbored a mutation in exon 19 in the EGFR gene. Treatment with erlotinib was started as soon as the molecular studies were available with remarkable and complete radiographic response in the CNS disease, and complete resolution of the previously detected metastases. The patient did not receive any other CNS intervention and radiation was not given due to the lack of CNS symptoms.

Validation of a food frequency questionnaire for determining calcium and vitamin D intake by adolescent girls with anorexia nervosa.

Assessing calcium and vitamin D intake becomes important in conditions associated with low bone density such as anorexia nervosa. Food records that assess intake over a representative time period are used in research and sometimes clinical settings. However, compliance in adolescents can be suboptimal. This study was undertaken to determine the validity of a food frequency questionnaire (FFQ) for assessing calcium and vitamin D intake in adolescent girls with anorexia nervosa and healthy girls compared to validated food records assessing intake during a 4-day period, the hypothesis being that intake would be adequately predicted by the FFQ. Thirty-six girls with anorexia nervosa and 39 healthy girls aged 12 to 18 years completed both the food record and the FFQ. An additional 31 subjects (20 with anorexia nervosa, 11 controls) completed the FFQ, but not the food record, and one girl with anorexia nervosa completed the food record, but not the FFQ. Subjects demonstrated greater compliance with the FFQ (99%) than the food record (71%). Daily calcium and vitamin D intake calculated using the food record and FFQ did not differ, although the FFQ tended to under-report vitamin D intake corrected for energy intake. Using quartile analysis, no gross misclassification was noted of calcium or vitamin D intake calculated using the food record or FFQ in girls with anorexia nervosa. Strong correlations were observed for daily vitamin D intake derived from the FFQ vs the food record, particularly in girls with anorexia nervosa (r=0.78, P<0.0001). Less robust correlations were observed for calcium intake (r=0.65, P<0.0001). The FFQ used in this study can be effectively used to assess daily calcium and vitamin D intake in adolescent girls with anorexia nervosa.

Determinants of height in adolescent girls with anorexia nervosa.

BACKGROUND: Anorexia nervosa, a condition characterized by marked caloric restriction and low insulin like growth factor-1 levels, would be expected to cause short stature. However, this disorder is also associated with hypogonadotropic hypogonadism and high growth hormone levels. Delays in growth-plate closure from associated hypogonadism may result in a longer period of time available for statural growth with protective effects on stature. In addition, growth hormone may have direct effects on the growth plate independent of insulin-like growth factor 1 to increase statural growth. OBJECTIVES AND METHODS: To determine the impact of undernutrition, hypogonadism, and acquired growth hormone resistance on height in adolescents with anorexia nervosa (aged 12-18 years), we examined 208 girls: 110 with anorexia nervosa and 98 controls of comparable chronological age. Sixty-three girls with anorexia nervosa and 79 controls were followed prospectively over 1 year. Mean duration of illness was 11.6 +/- 13.2 months. In a subset, overnight growth hormone sampling was performed every 30 minutes for 12 hours, and fasting insulin-like growth factor 1 levels were obtained. RESULTS: The difference between height and target height and between predicted adult height and target height did not differ between the groups, indicating preservation of height potential. The groups had comparable bone age, but bone age was lower than chronological age in girls with anorexia nervosa. Girls with anorexia nervosa had lower insulin-like growth factor 1 levels and higher nadir growth hormone levels than those of controls. Nadir growth hormone levels predicted height SD scores and predicted adult-height SD scores in controls but not in the girls with anorexia nervosa. In girls with anorexia nervosa, insulin-like growth factor 1 and duration of illness predicted height measures. Height SD scores of <0 were more likely after 32 months of illness and at insulin-like growth factor 1 levels of <134 ng/mL. Delayed baseline bone age predicted subsequent increases in height SD scores in immature girls with anorexia nervosa. CONCLUSIONS: Our data suggest that preservation of height potential in this cohort of girls with anorexia nervosa may be a consequence of delayed bone age. Hypogonadism may negate the deleterious effects of undernutrition on stature by allowing for a longer duration of growth.