Clinical pathway for patients with Chronic Myeloid Leukaemia: The Euriclea Project.

BACKGROUND AND AIMS: The use of Tirosine Kinase Ihnibitors (TKIs) for the treatment of Chronic Myeloid Leukemia (CML) has definitely represented a turning point in the treatment of the onco-hematological diseases. Over the years, the interest of physicians, nurses, patients and caregivers has increasingly focused on the aspects of the humanization of care, the management of side effects and on the full and constant therapeutic adherence. The aim of the project was to define patient-oriented care processes, based on a proactive approach that can fully respond to the new health needs of CML patients. METHODS: A nursing expert Working Group (WG) was established. WG reviewed literature about CML patients assistance and then it was conducted a survey on organizational models for the treatment of CML patients, adopted by Italian haematologic and transplant centers.  Finally, the main issues regarding CML patients care were identified and discussed on a multiprofessional basis. RESULTS: Euriclea Project for care of CML patients with the description of a new and expanded nurse role was defined. The Nurse Case Manager or Nursing Clinical Experts were identified as key people for the management of the side effects of treatment, the promotion of the therapeutic adherence and the evaluation of efficacy and effectiveness of the process through the identification of specific indicators for structure, process and outcome. DISCUSSION: The focal areas of the care process were identified so as to define a different approach to the CML patient, through a holistic view of care and the multidisciplinary interventions.

Caring for Two: Pregnancy and Chronic Myeloid Leukemia.

A 27-year-old Australian-born woman named Mrs. B presented to her general practitioner in December 2010 with a three-month history of excessive tiredness. She has a supportive husband, and they have four children aged four months to five years old. Mrs. B was breastfeeding her youngest child and had initially attributed her fatigue to her busy home life. A blood test revealed an elevated white blood cell count (WBC) of 43.7 x 109/L (reference range: 4-11 x 109/L); hemoglobin and platelets were within normal range. Mrs. B was subsequently referred to a specialist hematology service.

Treating chronic myeloid leukaemia: NICE guidance.

Chronic myeloid leukaemia (CML) affects approximately 560 people annually in the UK. Although median age at diagnosis is 60 years (National Institute for Health and Clinical Excellence (NICE), 2012), CML occurs in all age groups (National Comprehensive Cancer Network (NCCN), 2012). It is a clonal myeloproliferative disorder which is characterized by a reciprocal translocation between chromosomes 9 and 22, resulting in the formation of the Philadelphia chromosome (Ph Chromosome) and the development of an unregulated tyrosine kinase (BCR-ABL) which is responsible for the pathogenesis of CML (NCCN, 2012).

Caregiving burden, stress, and health effects among family caregivers of adult cancer patients.

Unlike professional caregivers such as physicians and nurses, informal caregivers, typically family members or friends, provide care to individuals with a variety of conditions including advanced age, dementia, and cancer. This experience is commonly perceived as a chronic stressor, and caregivers often experience negative psychological, behavioral, and physiological effects on their daily lives and health. In this report, we describe the experience of a 53-year-old woman who is the sole caregiver for her husband, who has acute myelogenous leukemia and was undergoing allogeneic hematopoietic stem cell transplantation. During his intense and unpredictable course, the caregiver's burden is complex and complicated by multiple competing priorities. Because caregivers are often faced with multiple concurrent stressful events and extended, unrelenting stress, they may experience negative health effects, mediated in part by immune and autonomic dysregulation. Physicians and their interdisciplinary teams are presented daily with individuals providing such care and have opportunity to intervene. This report describes a case that exemplifies caregiving burden and discusses the importance of identifying caregivers at risk of negative health outcomes and intervening to attenuate the stress associated with the caregiving experience.

Managing chronic myeloid leukemia: a coordinated team care perspective.

Treatment of chronic myeloid leukemia (CML) has seen dramatic progress in recent years with the development of tyrosine kinase inhibitors (TKIs). To take maximum advantage of therapy with TKIs, compliance and good understanding of monitoring response to therapy are essential. We established a team that included a hematologist, a physician assistant (PA), and a nurse who work closely with a social worker, a pharmacist, and a research coordinator to assist patients throughout their journey with CML. The patient and the referring community oncologist were incorporated into this team. This coordinated team care approach takes advantage of each member's specific skills to provide patients with education about CML, encourage patients' strong involvement in tracking/monitoring results/response to therapy, and support patients with issues that arise throughout the long course of the disease. A low rate of noncompliance with clinic visits (3%) was an indirect measure of the impact of this approach. The inclusion of the referring oncologist in the team extended the tracking of monitoring results to the community practice. We conclude that a coordinated team care approach is feasible in the management of patients with CML. This approach provided patients with education and a good understanding of response to therapy and improved relations with the health care team.

Treatment of chronic myeloid leukemia following imatinib resistance: a nursing guide to second-line treatment options.

The introduction of the BCR-ABL inhibitor imatinib revolutionized the treatment of patients with chronic myeloid leukemia (CML). However, resistance to imatinib has become a clinically significant issue, limiting its long-term efficacy. Numerous mechanisms have been associated with imatinib resistance, including mutations to the BCR-ABL gene, increased production of BCR-ABL, and activation of BCR-ABL-independent pathways (e.g., SRC-family kinases [SFKs]). Resistance to imatinib has driven the development of second-line therapies, such as dasatinib, a dual BCR-ABL/SFK inhibitor more potent than imatinib at targeting BCR-ABL. Dasatinib is approved for the treatment of patients with imatinib-resistant and -intolerant CML and Philadelphia chromosome-positive acute lymphoblastic leukemia. Nilotinib, an analog of imatinib, more potent than its parent compound, is another approved agent for patients with imatinib-resistant or -intolerant CML in the chronic or accelerated phase. Nurses must be aware of what constitutes a requirement for treatment change and the mechanisms of resistance that inform the choice of second-line agents. Oncology nurses also must ensure that patients have been educated appropriately to understand imatinib resistance and second-line treatment options. This article explores the mechanisms and identification of resistance and treatment options for when resistance occurs, as well as nursing implications.

A once-daily dasatinib dosing strategy for chronic myeloid leukemia.

The BCR-ABL inhibitor imatinib is standard first-line therapy for patients with chronic myeloid leukemia (CML) and has revolutionized treatment of the disease. However, resistance and intolerance to the agent have emerged as major clinical complications. Dasatinib is the first and only dual BCR-ABL/SRC family kinase inhibitor approved by the U.S. Food and Drug Administration for the treatment of patients with CML in any phase or Philadelphia chromosome-positive acute lymphoblastic leukemia who are resistant to or intolerant of imatinib. The agent is well tolerated and has shown clinical activity in such patients. As with other oral tyrosine kinase inhibitors, nonadherence to the prescribed dasatinib treatment regimen could obstruct a successful outcome. A new recommended dose of 100 mg once daily has been approved for patients with chronic phase CML. That dosing regimen, combined with appropriate management of dasatinib-related adverse events, may help patients adhere to their prescribed treatment and achieve maximum therapeutic benefit. This article highlights recent changes to the dasatinib label, including results with the 100 mg once-daily starting dose for patients with chronic phase CML, and discusses nursing strategies for the successful management of adverse events in patients receiving dasatinib.

Análise crítica das recomendações formuladas por um painel de experts para o cuidado clínico de pacientes com Leucemia Mielóide Crônica / Critical analysis of the recommendations from an expert panel on the management of patients with Chronic Myeloid Leukemia

As recomendações sobre Leucemia Mielóide Crônica feitas em 2006 por um painel de experts, em nome do "European Leukemia Network", foram adotadas internacionalmente como um roteiro para a monitoração e tratamento da doença. Passados 18 meses de sua publicação, fazemos aqui um sumário dessas recomendações e em seguida apontamos diversas áreas em que as recomendações poderão ser aperfeiçoadas e atualizadas. Aspectos específicos relacionados à aplicação das recomendações no Brasil são também considerados.

The recommendations about chronic myeloid leukemia by a panel of experts in 2006 on behalf of the "European Leukemia Network" have been internationally accepted as a guide for monitoring and treatment of this disease. Eighteen months after its publication, we present here a summary of these recommendations, and point to areas in which they might be improved and updated. Specific aspects of the application of these recommendations in Brazil are also considered.

Overview of second-generation tyrosine kinase inhibitors for patients with imatinib-resistant chronic myelogenous leukemia.

Imatinib mesylate (Gleevec, Novartis Pharmaceuticals Corporation, East Hanover, NJ) has revolutionized the treatment of chronic myelogenous leukemia (CML) with marked improvement in survival in all three phases--chronic, accelerated, and blast. Most patients with CML now receive imatinib, which produces complete cytogenetic response in more than 80% of patients. About 10% of patients who initially respond to imatinib subsequently develop resistance. Mechanisms of imatinib resistance in CML include amplification, mutations, and additional chromosomal aberration. To date, more than 30 mutations have been identified in imatinib-resistant CML. Dasatinib and AMN107, second-generation tyrosine kinase inhibitors, are highly effective therapies for patients with CML experiencing imatinib resistance and mutation and offer new options for patients who do not achieve an optimal response to imatinib therapy. Studies found that dasatinib and AMN107 form tighter bonds, overcoming imatinib resistance and producing complete hematologic and cytogenetic remissions. Long-term observations are needed to determine the effectiveness of the treatment. Primary care providers need to follow patients receiving first- or second-generation tyrosine kinase inhibitors because unforeseen toxicity may surface, requiring accurate assessment, evaluation, and management. Oncology nurses will be actively involved in the symptom management of patients. Providing guidelines for symptom management and advanced knowledge of specific test results for monitoring CML may increase positive outcomes.

Primeros transplantes de médula ósea en Bolivia / First transplants of medula osea in Bolivia

El trasplante de médula óasea es un procedimiento que ha mejorado la sobrevivencia libre de enfermedad de patologías oncohematológicas. El presente trabajo describe los primeros transplantes de médula ósea en Bolivia.En noviembre de 2002 se realizó en Bolivia el primer transplante alogénico de hermano consanguineo HLA compatible en pacientes de sexo masculino de 24 años de edad, con diagnóstico le leucemia mieloide crónica en fase crónica. El acondicionamiento fue con thiotepa, ciclofosfamida y fludarabina, seguido de transplante de 2.7 x 10 células mononucleadas por Kg/peso. Para profilaxis de rechazo al injerto (GVHD) recibió metotrexate, ciclosporina y metilprednisolona. Presentó GVHD grado I al día +13. La recuperación de neutrófilos mayor a 500/ml y plaquetas mayores a 20.000/ml fue al día +14. Actualmente (marzo 2004)el paciente esta libre de enfermedaad en remisión molecular completa. Otro paciente dae 40 años de edad, se sexo masculino, con diagnóstico de Linfoma linfoblástico con bulky abdominal, de alto riesgo realizó tratamiento poliquimioterápico según protocolo LLA-BOL2000Adulto (adriamicina, vincristina, dexametasona, ciclofisfamida, citarabina, etopósido, metotrexate y prednisona) logrando remisión parcial de bulky abdominal. En julio de 2002 se realizó la obtención de células progenitoras de tejido hematopoyético que fueron congeladas con DMSO a -80°C; seguido de acondicionamiento con protocolo BEAM (BCNU, etopósido, ara-C y melphalan) y reinfusión de 1.9 x10 células mononucleadas por Kg/peso. La recuperación de neutrófilos mayor a 500 /ml se presentó el día +14 y plaquetas mayor a 20.000/ml el día +12. Actualmente después de 21 meses, el paciente se encuentra sano libre de enfermedad.

Management of molecular-targeted therapy for chronic myelogenous leukemia.

PURPOSE: To provide primary care providers with guidelines on the evaluation and management of treatment for patients with chronic myelogenous leukemia (CML) with emphasis on the molecular-targeted therapy imatinib mesylate. DATA SOURCES: Research-based articles in the medical literature, review articles, and clinical practice guidelines. CONCLUSIONS: CML is a clonal stem cell disorder causing 5,000 new cases annually. Research on molecular-targeted therapy confirms that specific protein kinases have broad consequences for the development of future drugs to treat CML. The most recent discovery is a new compound, imatinib mesylate, which has become commercially available. Currently, the standard of care for CML is this agent. This therapy has changed management strategies for treatment of patients with the diagnosis of CML. IMPLICATIONS FOR PRACTICE: Long-term observations and evaluations are needed for final determination of treatment effectiveness; thus, primary care providers will follow patients with CML in the community. Unforeseen toxicity may surface, requiring accurate assessment and evaluation. Primary care providers will be actively involved with providing symptom management for these patients.

Hematopoietic cell transplantation in the treatment of leukemia.

OBJECTIVES: To review the current evidence regarding the role and benefits of hematopoietic cell transplantation (HCT) in leukemia. DATA SOURCES: Review articles, original articles, internet web sites, and books. CONCLUSION: HCT is a potentially curative treatment modality for patients with hematologic malignancies such as leukemia. IMPLICATIONS FOR NURSING PRACTICE: Nurses working with patients undergoing HCT need awareness of the type of leukemia, source of the hematopoietic cell product, type of preparative regimen used, and the complications of the procedure will enable nurses to educate and intervene with patients and their family members throughout the transplant trajectory.

Diagnosis, treatment, and nursing care of patients with chronic leukemia.

OBJECTIVES: To provide an update on the impact of new information about the molecular biology of chronic leukemia and new treatment modalities available to patients. DATA SOURCES: Published articles, books, and research studies. CONCLUSION: There has been significant progress in the diagnosis and management of chronic myeloid and chronic lymphocytic leukemia. New therapies provide more options for patients and longer treatment periods. IMPLICATIONS FOR NURSING PRACTICE: With increasing treatment options and longer survival, patients with chronic myelogenous or chronic lymphocytic leukemia need increased education, support, and assistance with symptom management. Nurses caring for these patients must remain knowledgeable about new treatments and their management.

Update on the diagnosis of and therapy for acute promyelocytic leukemia and chronic myelogenous leukemia.

PURPOSE/OBJECTIVES: To present information about recent Food and Drug Administration (FDA)-approved indications regarding drug therapy for acute promyelocytic leukemia (APL) and chronic myelogenous leukemia (CML) and about the nursing care of patients receiving these therapies and to review the significance of the use of polymerase chain reaction (PCR) assays in the management of patients with these leukemias. DATA SOURCES: Published articles, book chapters, and production information. DATA SYNTHESIS: Two agents with newly approved FDA indications are changing the focus of leukemia treatment from the cytotoxicity of specific drugs to the reversal of arrested maturation of myeloid cells. All-trans-retinoic acid (ATRA) induces remission in patients with relapsed or refractory APL. Interferon alfa (IFN-alpha) administered to patients with chronic-phase, Philadelphia-chromosome-positive CML produces improved survival rates and delays disease progression as compared to standard therapy. PCR assays are used with both of these diseases to confirm diagnosis, monitor response to these agents, and predict disease progression. CONCLUSIONS: The care of patients with leukemia continues to change as new agents such as ATRA and are approved for treatment and new assays such as PCR become available for diagnosis and treatment monitoring. IMPLICATIONS FOR NURSING PRACTICE: The nursing care of patients with leukemia requires keeping knowledgeable about the latest information regarding the molecular biology of the disease, the mechanism of action of newly approved agents, and the clinical implications of developing tests. This knowledge allows nurses to assimilate new therapies into practice, which, in turn, enables them to help patients to understand and cope with treatment through patient education and innovative interventions for symptom management.