Chapter 105. Malignancies of Lymphoid Cells (Part 8)

Chapter 105. Malignancies of Lymphoid Cells (Part 8)Approach to the Patient: Lymphoid Cell MalignanciesRegardless of the type of lymphoid malignancy, the initial evaluation of the patient should include performance of a careful history and physical examination. These will help confirm the diagnosis, identify those manifestations of the disease that might require prompt attention, and aid in the selection of further studies to optimally characterize the patients status to allow the best choice of therapy. It is difficult to overemphasize the importance of a carefully done history and physical examination. They might provide observations that lead to reconsidering the diagnosis, provide...
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Chapter 105. Malignancies of Lymphoid Cells (Part 8) Chapter 105. Malignancies of Lymphoid Cells (Part 8) Approach to the Patient: Lymphoid Cell Malignancies Regardless of the type of lymphoid malignancy, the initial evaluation of thepatient should include performance of a careful history and physical examination.These will help confirm the diagnosis, identify those manifestations of the diseasethat might require prompt attention, and aid in the selection of further studies tooptimally characterize the patients status to allow the best choice of therapy. It isdifficult to overemphasize the importance of a carefully done history and physicalexamination. They might provide observations that lead to reconsidering thediagnosis, provide hints at etiology, clarify the stage, and allow the physician toestablish rapport with the patient that will make it possible to develop and carryout a therapeutic plan. For patients with ALL, evaluation is usually completed after a completeblood count, chemistry studies reflecting major organ function, a bone marrowbiopsy with genetic and immunologic studies, and a lumbar puncture. The latter isnecessary to rule out occult CNS involvement. At this point, most patients wouldbe ready to begin therapy. In ALL, prognosis is dependent upon the geneticcharacteristics of the tumor, the patients age, the white cell count, and the patientsoverall clinical status and major organ function. In CLL, the patient evaluation should include a complete blood count,chemistry tests to measure major organ function, serum protein electrophoresis,and a bone marrow biopsy. However, some physicians believe that the diagnosiswould not always require a bone marrow biopsy. Patients often have imagingstudies of the chest and abdomen looking for pathologic lymphadenopathy.Patients with typical B cell CLL can be subdivided into three major prognosticgroups. Those patients with only blood and bone marrow involvement byleukemia but no lymphadenopathy, organomegaly, or signs of bone marrow failurehave the best prognosis. Those with lymphadenopathy and organomegaly have anintermediate prognosis, and patients with bone marrow failure, defined ashemoglobin discern. The prognosis is adversely affected when either or both of theseabnormalities are due to progressive marrow infiltration and loss of productivemarrow. However, either or both may be due to autoimmune phenomena or tohypersplenism that can develop during the course of the disease. These destructivemechanisms are usually completely reversible (glucocorticoids for autoimmunedisease; splenectomy for hypersplenism) and do not influence disease prognosis. Two popular staging systems have been developed to reflect theseprognostic groupings (Table 105-7). Patients with typical B cell CLL can havetheir course complicated by immunologic abnormalities including autoimmunehemolytic anemia, autoimmune thrombocytopenia, and hypogammaglobulinemia.Patients with hypogammaglobulinemia benefit from regular (monthly) γ globulinadministration. Because of expense, γ globulin is often withheld until the patientexperiences a significant infection. These abnormalities do not have a clearprognostic significance and should not be used to assign a higher stage. Table 105-7 Staging of Typical B Cell Lymphoid Leukemia Stage Clinical Features Median Survival, Years RAI System 0: Low Lymphocytosis only in blood and >10risk marrow I: Lymphocytosis + lymphadenopathy 7Intermediate + splenomegaly ± hepatomegalyrisk II III: High Lymphocytosis + anemia 1.5risk IV Lymphocytosis + thrombocytopenia Binet System A Fewer than three areas of clinical >10 lymphadenopathy; no anemia or thrombocytopeniaB Three or more involved node areas; 7 no anemia or thrombocytopeniaC Hemoglobin ≤10 g/dL and/or 2 platelets