LEUCEMIA PROLINFOCITICA PDF
Leucemia linfocítica crónica. 10 Signos y síntomas. Diagnóstico. 12 Planificación del tratamiento. 19 Tratamiento. 32 Complicaciones de la. Update of the Grupo Español de Leucemia Linfocítica Crónica clinical guidelines of the management of chronic lymphocytic leukemia. Los factores pronósticos son aquellas circunstancias medibles o cuantificables que van a influir en el resultado de la aparición de la leucemia linfocítica crónica .
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This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of chronic lymphocytic leukemia. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
prolnifocitica CLL is a disorder of morphologically mature but immunologically less mature lymphocytes and is manifested by progressive accumulation of these cells in the blood, bone marrow, and lymphatic tissues.
Such early detection and diagnosis may falsely suggest improved survival for the group and may unnecessarily worry or result in therapy for some patients who would have remained undiagnosed in their lifetime, a circumstance known in the literature as overdiagnosis or pseudodisease.
In a database analysis and for up to 77 months before diagnosis, almost all patients with a diagnosis of CLL had prediagnostic Prolinfocirica clones that were identified in peripheral blood when available.
PLEIOTROPIA EN FAMILIAS CON LEUCEMIA LINFOCITICA CRONICA
For patients with progressing CLL, treatment with conventional doses of chemotherapy is not curative; selected patients treated with allogeneic stem cell transplantation have achieved prolonged prolinfoictica survival.
Antileukemic therapy is frequently unnecessary in uncomplicated early disease. The median survival for all patients ranges from 8 to 12 years in older trials with data from the s through the s. There is, however, a large variation in survival among individual patients, ranging from several months to a normal life expectancy. Treatment must be individualized based on the clinical behavior of the disease.
As found in one report, CLL occurs primarily in prrolinfocitica and elderly adults, with increasing frequency in successive decades of life. The clinical course of this disease progresses from an indolent lymphocytosis without other evident disease to one of generalized lymphatic enlargement with concomitant pancytopenia.
Complications of pancytopenia, including hemorrhage and infection, represent a major cause of death in these patients. Immunological aberrations, including Coombs-positive hemolytic anemia, immune thrombocytopenia, and depressed immunoglobulin levels may all complicate the management of CLL.
Prognostic factors that may help predict clinical outcome include cytogenetic subgroup, leuceemia mutational status, ZAP, and CD Refer to the Prognostic Factors section in the Stage Information for Chronic Prolinfociticaa Leukemia section of this summary for more information. Patients prplinfocitica develop an aggressive high-grade non-Hodgkin lymphoma, usually diffuse large B-cell lymphoma and termed a Richter leucemmia, have a poor prognosis.
Patients with CLL are also at increased risk for other malignancies, even before therapy. A population-based analysis of almost 2 million cancer patients in the National Cancer Institute’s Surveillance, Epidemiology, and End Results SEER database suggests that cancer-specific survival for patients with pre-existing CLL who subsequently develop colorectal and leucemka cancer is significantly lower hazard ratio [HR], 1.
Confusion with other diseases may be avoided by determination of cell surface markers. This coexpression only occurs in one other disease entity, mantle cell lymphoma. CLL B cells express relatively low levels of surface-membrane immunoglobulin compared with normal peripheral blood B cells prolinfocitiica a single light chain kappa or lambda.
Prolymphocytic leukemia PLL is a rare entity characterized by excessive prolymphocytes in the blood with a typical phenotype that is positive for CD19, CD20, and surface-membrane immunoglobulin and negative for CD5. These patients demonstrate splenomegaly and poor response to low-dose or high-dose chemotherapy. These patients often have neutropenia and a history of rheumatoid arthritis.
The natural history is indolent, often marked by anemia and splenomegaly. This condition appears to fit into the clinical spectrum of Felty syndrome.
Tratamiento de la leucemia linfocítica crónica (PDQ®) (Health professionals) | OncoLink
Therapy includes low doses of oral cyclophosphamide or methotrexate, cyclosporine, and treatment of the bacterial infections acquired during severe neutropenia. Staging is useful in chronic lymphocytic leukemia CLL to predict prognosis and also to stratify patients to achieve comparisons for interpreting specific treatment results.
Anemia and thrombocytopenia are the major adverse prognostic variables. CLL has no standard staging system. The Rai staging system and the Binet proilnfocitica are presented below. A National Cancer Institute NCI -sponsored working group has formulated standardized guidelines for criteria related to eligibility, response, and toxic prolinfcitica to be used in future clinical trials in CLL.
Stage I CLL is characterized by absolute lymphocytosis with lehcemia without hepatosplenomegaly, anemia, or thrombocytopenia. Stage II CLL is characterized by absolute lymphocytosis with either hepatomegaly or splenomegaly with or without lejcemia. The Binet classification integrates the number of nodal groups involved with the leucema with bone marrow failure.
Its major benefit derives from the recognition of a predominantly splenic form of the disease, which may have a better prognosis than in the Rai staging, and from recognition that the presence of anemia or thrombocytopenia has a similar prognosis and does not merit a separate stage. Neither system separates immune from nonimmune causes of cytopenia. The NCI-sponsored working group has published guidelines for the diagnosis and treatment of CLL in both clinical trial and general practice settings.
Use of these systems allows comparison of clinical results and establishment of therapeutic guidelines. New prognostic markers are now available to the clinician and investigator.
The use of these markers to stratify patients in clinical trials, to help assess the need for therapy, and to help select the type of therapy continues to evolve. Prospective trials to verify and establish the role of these prognostic markers are ongoing. No large multivariable analyses exist as yet to test the relative power of these individual prognostic variables. Prognostic indices are under evaluation and will require prospective validation. The new prognostic markers include the leucemix.
Treatment lsucemia chronic lymphocytic leukemia CLL ranges from periodic observation with treatment of infectious, hemorrhagic, or immunologic complications to a variety of therapeutic options, including steroids, alkylating agents, purine analogs, combination chemotherapy, monoclonal antibodies, and transplant options. Because this disease is generally not curable, occurs in an elderly population, and often progresses slowly, prolinfocktica is most often treated in a conservative fashion.
In asymptomatic patients, treatment may be deferred until the patient becomes symptomatic as the disease progresses. Since the rate of progression may vary from patient to patient, with long periods of stability and sometimes spontaneous regressions, frequent and careful observation is required to monitor the clinical course. A meta-analysis of leucekia trials showed no survival benefit for immediate versus delayed therapy for patients with early-stage disease, nor for the use of combination regimens incorporating an anthracycline compared with a single-agent alkylator for advanced-stage disease.
Infectious complications in advanced prolinfocitida are in part a consequence of the hypogammaglobulinemia and the inability to mount a humoral defense against bacterial or viral agents. Herpes zoster represents a frequent viral infection in these patients, but infections luecemia Pneumocystis carinii and Candida albicans may also occur. The early recognition of infections and the institution of appropriate therapy are critical to the long-term survival of these patients.
There was, however, no effect on survival. Second malignancies and treatment-induced acute leukemias may also occur in a small percentage of patients. Initial prolijfocitica involves corticosteroids with or without alkylating agents fludarabine can worsen the hemolytic anemia.
It is frequently advisable to control the autoimmune destruction with corticosteroids, if possible, before administering marrow-suppressive chemotherapy because the patients may be difficult to transfuse successfully with either red blood cells or platelets.
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Alternate therapies include high-dose immune globulin, rituximab, cyclosporine, azathioprine, splenectomy, and low-dose radiation therapy leucemla the spleen. Tumor lysis syndrome is an uncommon complication presenting in 1 out of patients of chemotherapy for patients with bulky disease.
These patients have a higher frequency of skin lesions, more variable lymphocyte shape, and shorter median survival 13 months with minimal responses to chemotherapy. Computed tomographic CT scans have a very limited role in following patients after completion of treatment; the decision to treat for relapse was determined by CT scan or ultrasound in only 2 of patients in three prospective trials for the German CLL Study Group.
Because of the indolent nature of stage 0 chronic lymphocytic leukemia CLLtreatment is not indicated. The French Cooperative Group on CLL randomly assigned 1, patients with previously untreated stage A disease to receive either chlorambucil or no immediate treatment and found no survival advantage for immediate treatment with chlorambucil. Use prolinfoccitica advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients.
The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available. The improvements in response rates from more intensive regimens have maximized the clearance of minimal residual disease MRD.
In one prospective trial of patients, clearance of MRD was an independent predictor of overall survival OS by multivariate analysis. The surrogate endpoint of clearance of residual disease, while prognostic, did not show improved survival in a randomized prospective trial. The necessary study would include patients who fail to completely clear the marrow with induction therapy and randomly assign them to further alternative treatment versus the lekcemia treatment later at relapse, looking at OS as the primary endpoint.
In the absence of randomized trials comparing the new B-cell receptor inhibitors and bcl-2 inhibitors to the new monoclonal antibodies and to more conventional chemotherapeutic agents, the following general principles may provide a sequencing for available therapeutic options:.
Outside of the context of a clinical trial, treatment for asymptomatic or minimally affected patients with CLL is observation. No data exist as yet to suggest any harm with a delay in therapy until the patient becomes symptomatic or develops serious cytopenias despite growth factor support. Because the rate of progression may vary from patient to patient, with long periods of stability and sometimes spontaneous regressions, frequent and careful observation is prolinfoxitica to monitor the clinical course.
One nomogram to predict time-to-first treatment relies on the number of lymph node sites, size leudemia cervical lymph nodes, lactate-dehydrogenase level, the immunoglobulin variable region heavy chain IgVH mutational status, and leucemi presence of 11q- or 17p- deletion established by fluorescence in situ hybridization FISH analysis.
A prospective, randomized trial of patients with relapsed or refractory CLL or small lymphocytic lymphoma compared ibrutinib with ofatumumab. With a median follow-up of 9. A prospective, randomized trial of previously untreated patients who were aged 65 years or peolinfocitica compared ibrutinib with chlorambucil. A prospective, randomized trial of previously treated patients compared ibrutinib plus bendamustine plus rituximab with bendamustine plus rituximab.
These leucejia trials establish the rationale for first-line use of ibrutinib in patients with CLL, especially for high-risk patients with 17p- disease.
These trials also establish the use of ibrutinib for patients with relapsed disease. In a randomized prospective trial NCTpreviously untreated patients with coexisting medical problems were randomly assigned to chlorambucil and obinutuzumab versus chlorambucil and rituximab versus chlorambucil alone. The median PFS was best for the obinutuzumab arm Patients who received obinutuzumab did not have improved survival compared with those who received rituximab alone.
A prospective, randomized prolinfociticz of patients who were previously untreated compared prolinfoxitica plus chlorambucil with chlorambucil alone. With a median follow-up of 2 years, median PFS favored the ofatumumab arm at A prospective trial of previously treated patients who attained partial or complete remission to second- or third-line chemotherapy were randomly assigned to 2 years of maintenance therapy with ofatumumab versus observation.
With a median follow-up prolinfocjtica 19 months, median PFS favored the ofatumumab maintenance arm at In a randomized, double-blind, prospective trial NCTpatients treated mainly with fludarabine-based regimens and who had coexisting medical problems, such as renal dysfunction, received rituximab and idelalisib versus rituximab and placebo. The French Cooperative Group on CLL randomly assigned 1, patients with previously untreated stage A disease to receive either chlorambucil or no immediate treatment and found no survival advantage for chlorambucil.
Several randomized trials have compared the purine analogs with chlorambucil; with cyclophosphamide, doxorubicin, and prednisone; or with cyclophosphamide, doxorubicin, vincristine, and prednisone CHOP in previously untreated patients. All of these trials prolinfocjtica higher or equivalent response rates for the purine analog, and most showed an improvement in PFS; one reached significance in OS favoring fludarabine. The increased risk of infection may persist for months or years after treatment with a purine analog.
Although empiric evidence is lacking, some investigators recommend prophylaxis with trimethoprim-sulfa during therapy and for 6 to 12 months afterwards to prevent pneumocystis infection. In a similar way, other investigators employ prophylaxis e. Purine analogs cause less hair loss or nausea than combination chemotherapy, including alkylators and anthracyclines. Combination chemotherapy was used in a trial of patients that compared FCR with fludarabine plus cyclophosphamide FC and at a median follow-up of 5.