Durable complete remission of macroglobulinemia after splenectomy: a report of two cases and review of the literature.

Two patients with macroglobulinemia (monoclonal IgM in the serum) and massive splenomegaly were incapacitated by progressive disease refractory to standard chemotherapy. In each case, palliative splenectomy was followed by a prompt, complete, and unexpected clinical remission with disappearance from the serum of the monoclonal IgM component. One patient remains free of disease 12 years after splenectomy. The other patient remained free of detectable macroglobulinemia for 13 years after splenectomy. A review of the literature revealed other cases of remission of macroglobulinemia attributable to splenectomy alone. Data in humans and animals suggest that the spleen may facilitate IgM secretion by normal and malignant B lymphocytes. Splenectomy should be considered a possible treatment option for patients with massive splenomegaly and macroglobulinemia who progress on chemotherapy.

Types of endoscopic instruments.

1. Rigid endoscopes provide excellent optics and are durable, but are not as versatile as flexible scopes. Flexible endoscopes provide accessibility to otherwise inaccessible areas, but have poorer optics. 2. There are several issues to consider when making the decision to use disposable or reusable items. This decision must be made jointly by the OR manager and the surgeon. The most important issue is patient outcome. 3. It is certain that the trend towards noninvasive procedures will continue as surgeons from additional surgical specialties adopt endoscopic techniques. The perioperative nurse must be ready to expand practice to facilitate the growing technology of endoscopic procedures.

Managing the trauma patient: focusing on perioperative education.

The key to planning and implementing safe, effective care to the trauma patient lies in initial and ongoing education, both hospital- and unit-based. Evaluation provides valuable feedback to assess the comprehensiveness of existing tools and the need for change. The perioperative nurse needs to know the pathophysiology of trauma, mechanisms of injury, continuity of care through the healthcare system, and trauma protocols of the facility, as well as the instrumentation and equipment used during trauma surgery. The goal of trauma orientation and continuing education is for the perioperative nurse to be able to use the nursing process to rapidly assess, diagnose, plan, implement, and evaluate nursing care for the trauma patient.

Role development of the clinical nurse specialist within the Indian Health Service.

The clinical nurse specialist is employed in many health care facilities to improve patient care. One service unit of the Indian Health Service has expressed interest in the development of this role. At the present time, there are no advanced practitioners within this facility. Development, as well as implementation of the role, must be based on the cultural and health care needs of the Indian people. This paper discusses development of the role of perioperative clinical nurse specialist, based on personal experience, at a service unit providing health care to a large tribe of American Indians in the Southwestern United States. Examples of care incorporating cultural practices, are used to illustrate the practice of the perioperative specialist within the Indian Health Service. The nursing process is used to organize the development of the role.

Biologic false-positive serologic tests for syphilis and other serologic abnormalities in autoimmune hemolytic anemia and thrombocytopenic purpura.

Autoimmune hemolytic anemia (AIHA), the first autoimmune disease to be recognized, is a manifestation of defective immune regulation. Although often associated with a lymphoid neoplasm or an overt immunologic disorder, AIHA frequently appears without apparent cause. Many patients with the "idiopathic" disease have been found to have various immunologic abnormalities in addition to the antibodies reacting with red cells. Although familial AIHA is uncommon, other autoimmune diseases and serologic abnormalities have been encountered in relatives of numerous patients. Few detailed family studies have been performed, but the available data suggest that predisposition to AIHA and to the associated immunologic disorders often is genetically transmitted. Less information is available about autoimmune thrombocytopenic purpura, in part because of the historic difficulty in recognizing autoantibodies that react with platelets. However, there is good evidence for genetically determined predisposing factors in some cases. Using the BFP reaction as an indicator, we add to the evidence that AIHA and autoimmune thrombocytopenia, like SLE, tend to occur in persons with a long-standing occult immunologic defect that often has a genetic basis. In our studies, 11 patients with AIHA or ITP had BFP reactions. The serologic abnormality in 4 had been known to precede the blood disorder by 6 to 44 years. Five of the patients had an additional disease believed to have an immunologic pathogenesis. Serologic abnormalities apart from the BFP reaction and the red cell or platelet antibodies were demonstrated in 9. Autoimmune diseases or serologic changes are known to have affected relatives of 5 patients, including 4 who had 1 or more relatives with BFP reactions. Serologic tests for syphilis were negative in 2 sibs with autoimmune thrombocytopenic purpura whose father had a chronic BFP reaction and thyroiditis, but all 3 had low levels of IgA and IgM. Lymphoproliferative disorders appeared in 3 of the patients with BFP reactions, and 2 had relatives with lymphoid neoplasms. A lymphoma occurred in a woman with cold agglutinin disease 21 years after the discovery of the erythrocyte antibody. Our observations support the view that an abnormality of cells of the immune system, often genetically determined, may predispose to serologic changes, immune deficiency, autoimmune diseases and neoplasia.

Genetic factors predisposing to autoimmune diseases. Autoimmune hemolytic anemia, chronic thrombocytopenic purpura, and systemic lupus erythematosus.

Genetic factors predisposing to autoimmune diseases were investigated in 10 families having more than one affected member. Seventy relatives and 23 spouses from two large kindreds (one in whom the proband had autoimmune hemolytic anemia and the other immune thrombocytopenic purpura) were examined for immunologically mediated disorders, autoantibodies, immunoglobulin abnormalities, and HLA genotypes. Significant differences between relatives and spouses were found for immune diseases (21 percent versus 0 percent; p = 0.02), antinuclear antibody titer of 1:80 or more (18 percent versus 0 percent; p = 0.04), single-strand DNA antibodies (18 percent versus 0 percent; p = 0.04), high-titer antinuclear antibody or antibodies to single-strand DNA or both (33 percent versus 0 percent; p = 0.001), and the combined frequencies of immune diseases and serologic abnormalities (44 percent versus 4 percent; p = 0.0004). Similar frequencies were found in 41 relatives from eight families in whom the proband had SLE. Segregation analyses using these abnormalities as genetic traits were most compatible with a Mendelian dominant model. Impressive odds (100:1) against linkage to HLA were calculated.

Bone marrow hemosiderin does not always reflect body iron stores.

Absence of hemosiderin from the bone marrow is sometimes encountered in patients who are not iron deficient. Two cases of autoimmune hemolytic anemia are described in which there was little or no hemosiderin in the marrow but large amounts in the spleen, which had been the major site of accelerated RBC destruction.

Thrombosis of the portal vein following splenectomy for myeloid metaplasia.

The records of 28 patients undergoing splenectomy for myeloid metaplasia were reviewed. Portal and mesenteric venous thrombosis was proved or suspected in five patients. The time interval between splenectomy and portal and mesenteric venous thrombosis varied from six days to three years. The incidental postmortem finding of portal vein thrombosis in one patient suggests that the incidence may be greater than clinically appreciated and may, at times, be asymptomatic. Postsplenectomy thrombocytosis was not found to be a significant risk factor, occurring in only one of the five patients in whom portal vein thrombosis developed. Recommendations for prevention include ligation of the splenic vein close to its junction with the inferior mesenteric vein at the time of splenectomy. In addition, prophylactic anticoagulation therapy or the use of antiplatelet drug therapy postoperatively, or both, should be considered.

Autoimmune hemolytic anemia with reticulocytopenia. A medical emergency.

In four cases of autoimmune hemolytic disease, rapidly developing anemia was associated with reticulocytopenia despite intensely erythroid bone marrow. Transfusions had been withheld because compatible blood could not be obtained, and each patient was virtually moribund on admission. Type-specific RBCs were administered promptly without reaction. From 2 to 84 carefully selected units were required to sustain life during the reticulocytopenic episodes, which lasted from a few days to more than six months. Transfusion in patients with autoimmune hemolytic anemia generally is unwise, because the autoantibody in the serum usually reacts with the RBCs of all potential donors, making a satisfactory cross match impossible. However, reticulocytopenia with profound anemia may present as a medical emergency in which prompt, careful transfusion is lifesaving.

Genetic factors predisposing to chronic lymphocytic leukemia and to autoimmune disease.

Among 320 relatives of 28 patients with chronic lymphocytic leukemia (CLL), 4 had CLL, 1 had lymphosarcoma, and 2 other adults had leukemia of unknown type. Autoimmune disease including hyperthyroidism, pernicious anemia, rheumatoid arthritis and systemic lupus erythematosus affected 18 relatives. HLA typing of members of two families demonstrated that within each family the patient with CLL shared a common haplotype with relatives with autoimmune disease, but the haplotype was different in the two kindreds. In contrast, CLL was encountered in only 1 and autoimmune disease in 4 of 396 relatives of a group of 28 control patients. These data together with information from earlier reports support the hypothesis that genetic factors distrubing the regulation of the immune system may predispose both to lymphoid neoplasms and to autoimmune disease. The concept is supported by an array of experimental studies in animals.