Dynamic model for predicting death within 12 months in patients with primary or post-polycythemia vera/essential thrombocythemia myelofibrosis.

PURPOSE: Current prognostic tools in myelofibrosis (MF) fail to identify patients at the highest risk of death and are limited by their applicability only to the time of diagnosis. We aimed to define an accelerated phase (AP) in MF by characterizing disease features that can identify patients with median overall survival of or= 10%, platelets less than 50 x 10(9)/L, and chromosome 17 aberrations (median overall survival, 10, 12, and 5 months, respectively). In the validation phase, chronic-phase patients who developed AP features during follow-up were found to have short subsequent survival times (median overall survival, 12, 15, and 6 months, respectively). AP was a necessary step in the progression to blast phase, with leukemic transformation being exceedingly rare (3% risk at 10 years) in patients who remained persistently in chronic phase. CONCLUSION: Blood or bone marrow blasts >or= 10%, platelets less than 50 x 10(9)/L, and chromosome 17 aberrations defined AP in patients with MF. Patients in AP should be candidates for intensive therapeutic interventions.

De novo deletion 17p13.1 chronic lymphocytic leukemia shows significant clinical heterogeneity: the M. D. Anderson and Mayo Clinic experience.

To determine the clinical fate of patients with de novo deletion 17p13.1 (17p-) chronic lymphocytic leukemia (CLL), we retrospectively studied the outcome of 99 treatment-naive 17p- CLL patients from the M. D. Anderson Cancer Center (n = 64) and the Mayo Clinic (n = 35). Among 67 asymptomatic patients followed for progression, 53% developed CLL requiring treatment over 3 years. Patients who had not progressed by 18 months subsequently had stable disease, with 3 of 19 patients progressing after follow-up of up to 70 months. Risk factors for progressive disease were Rai stage of 1 or higher and unmutated immunoglobulin variable region heavy chain (IgVH). The overall survival rate was 65% at 3 years. Rai stage 1 or higher, unmutated IgVH, and 17p- in 25% or more of nuclei were adverse factors for survival. The 3-year survival rates of patients with 1 or fewer, 2, and 3 of these factors were 95%, 74%, and 22%, respectively (P < .001). Response rates to therapy with rituximab (n = 6); purine analogues and rituximab (n = 25); and purine analogues, rituximab, and alemtuzumab (n = 16) combinations were 50%, 72%, and 81%, respectively. Patients with 17p- CLL exhibit clinical heterogeneity, with some patients experiencing an indolent course. Survival can be predicted using clinical and biologic characteristics.

The role of nurse practitioners in meeting the need for child and adolescent psychiatric services: a statewide survey.

The high prevalence of child and adolescent mental health disorders coupled with shortages in age-appropriate mental health services pose a significant problem likely to be exacerbated over time. A survey was designed to identify the current status of and need for child and adolescent psychiatrists (CAPs) and mental health services, as well as strategies and recommendations to address identified needs in the state of New York. Key informants from each county and New York City were surveyed by telephone (N = 58). Most respondents identified a shortage of child and adolescent psychiatry services and reported that when CAPs are unavailable, nurse practitioners (NPs) are currently among the top four professional groups who prescribe and/or monitor psychotropic medication. Almost half of the respondents (48%) identified employing NPs with advanced certification in child and adolescent psychiatry as a promising strategy to improve access to care. Addressing the shortage of CAPs can provide an opportunity for the nursing profession to advance its role in the provision of mental health services to youth.

The role of cytogenetic abnormalities as a prognostic marker in primary myelofibrosis: applicability at the time of diagnosis and later during disease course.

Although cytogenetic abnormalities are important prognostic factors in myeloid malignancies, they are not included in current prognostic scores for primary myelofibrosis (PMF). To determine their relevance in PMF, we retrospectively examined the impact of cytogenetic abnormalities and karyotypic evolution on the outcome of 256 patients. Baseline cytogenetic status impacted significantly on survival: patients with favorable abnormalities (sole deletions in 13q or 20q, or trisomy 9 +/- one other abnormality) had survivals similar to those with normal diploid karyotypes (median, 63 and 46 months, respectively), whereas patients with unfavorable abnormalities (rearrangement of chromosome 5 or 7, or > or = 3 abnormalities) had a poor median survival of 15 months. Patients with abnormalities of chromosome 17 had a median survival of only 5 months. A model containing karyotypic abnormalities, hemoglobin, platelet count, and performance status effectively risk-stratified patients at initial evaluation. Among 73 patients assessable for clonal evolution during stable chronic phase, those who developed unfavorable or chromosome 17 abnormalities had median survivals of 18 and 9 months, respectively, suggesting the potential role of cytogenetics as a risk factor applicable at any time in the disease course. Dynamic prognostic significance of cytogenetic abnormalities in PMF should be further prospectively evaluated.

Systemic mastocytosis in association with chronic lymphocytic leukemia: a rare diagnosis.

Systemic mastocytosis may be primary or be associated with other hematological malignancies, most commonly myeloid neoplasms. In contrast, the association of systemic mastocytosis with lymphoid neoplasms is comparatively rare, and in the absence of appropriate clinical vigilance the diagnosis may be missed. We report a case of aggressive systemic mastocytosis associated with chronic lymphocytic leukemia (SM-CLL), placing particular emphasis on the investigational algorithm used in establishing this diagnosis, and discussing key clinical considerations in the initiation of therapy.

Early results of a chemoimmunotherapy regimen of fludarabine, cyclophosphamide, and rituximab as initial therapy for chronic lymphocytic leukemia.

PURPOSE: Fludarabine and cyclophosphamide (FC), which are active in treatment of chronic lymphocytic leukemia (CLL), are synergistic with the monoclonal antibody rituximab in vitro in lymphoma cell lines. A chemoimmunotherapy program consisting of fludarabine, cyclophosphamide, and rituximab (FCR) was developed with the goal of increasing the complete remission (CR) rate in previously untreated CLL patients to >/= 50%. PATIENTS AND METHODS: We conducted a single-arm study of FCR as initial therapy in 224 patients with progressive or advanced CLL. Flow cytometry was used to measure residual disease. Results and safety were compared with a previous regimen using FC. RESULTS: The median age was 58 years; 75 patients (33%) had Rai stage III to IV disease. The CR rate was 70% (95% CI, 63% to 76%), the nodular partial remission rate was 10%, and the partial remission rate was 15%, for an overall response rate of 95% (95% CI, 92% to 98%). Two thirds of patients evaluated with flow cytometry had less than 1% CD5- and CD19-coexpressing cells in bone marrow after therapy. Grade 3 to 4 neutropenia occurred during 52% of courses; major and minor infections were seen in 2.6% and 10% of courses, respectively. One third of the 224 patients had >/= one episode of infection, and 10% had a fever of unknown origin. CONCLUSION: FCR produced a high CR rate in previously untreated CLL. Most patients had no detectable disease on flow cytometry at the end of therapy. Time to treatment failure analysis showed that 69% of patients were projected to be failure free at 4 years (95% CI, 57% to 81%).

Treatment of chronic lymphocytic leukemia with alemtuzumab: a review for nurses.

PURPOSE/OBJECTIVES: To review the use of the monoclonal antibody alemtuzumab in patients with advanced refractory B cell chronic lymphocytic leukemia (B-CLL) and nursing management during treatment. DATA SOURCES: Published articles, abstracts, book chapters, Web sites, and training material. DATA SYNTHESIS: Alemtuzumab can achieve disease remission in patients with chemorefractory B-CLL; however, management of high-risk patients presents certain challenges. Infusion-related events can be minimized by stepwise administration and appropriate prophylaxis. Cytopenia can be minimized by drug postponement and cytokine support or red blood cell or platelet transfusions. Patients also are at risk for infection because of lymphopenia, and anti-infective prophylaxis is mandatory at initiation of therapy until at least two months post-treatment. CONCLUSIONS: With satisfactory supportive measures in place, patients with chemorefractory B-CLL can experience the benefits of alemtuzumab therapy without excessive toxicity. IMPLICATIONS FOR NURSING: Nurses should be familiar with treatment and prophylactic protocols, be ready to offer supportive therapy to control side effects, and invest time in patient education.