Quality of life among patients treated for achalasia.

The present study is an attempt to capture the quality of life of achalasia patients after a successful treatment. It is also an effort to assess the extent of the subsequent restrictions achalasia may have imposed upon the patients' life-style. All achalasia patients who were successfully treated between 1984 and 1992 were identified. Qualified patients were supplied with a 12-item quality-of-life questionnaire that had been designed to assess the patients' perceptions of their swallowing function and their general health; the restrictions achalasia had imposed on five areas of performance, which encompassed social activities, family relationships, travel experiences, sports and housework activities, were also assessed. Sixty-six patients were offered the questionnaire and 52 (77.6%) returned a completed form. Forty-one of the group had pneumatic dilatation and the remaining 11 had cardiomyotomy. Some form of dysphagia was reported by 36 patients (69%) and a dietary modification was exercised by 29 (56%) of them. Heartburn was reported by 31 (59%) of the patients. Fifteen percent of the patients felt that the disease interfered with their social activities, 8% experienced difficulty in their family relations, 13% believed that the disease restricted travel and athletics, and finally, 9% stated that their symptoms placed restrictions on their ability to do housework. The group that received pneumatic dilation experienced less restriction in the performance areas of sports, travel, and housework. However, this difference was only significant in the area of sports (P = 0.04). It is concluded that: (1) The restoration of the normal swallowing mechanism is not often achieved after treatment for achalasia. The majority of patients who have been treated continue to have a component of difficulty for the rest of their lives. (2) These residual symptoms leave an impact on the patients' life-style. This impact is least important in the performance area of family relationship and most impressive in the area of sports. (3) Finally, those patients who have been treated with cardiomyotomy are more restricted in sport activities than those who received pneumatic dilatation.

An increased HLA DR2 frequency is seen in aplastic anemia patients.

The underlying etiology of aplastic anemia is unknown in the majority of patients, although medications, chemical exposure, or viral infections can be implicated in some. Genetic susceptibility to a variety of diseases has been shown and it has recently been suggested that aplastic anemia is more common in individuals who are HLA DR2+ than in the general population. To examine this question, we retrospectively analyzed the results of HLA-DR typing in 75 aplastic anemia patients who received antithymocyte globulin (ATG) therapy or an HLA-matched sibling bone marrow transplant at UCLA between 1978 and 1989. Thirty-one patients were DR2+, a 1.9-fold higher incidence than the expected number of 16.6 patients (P < .0005). Of the 37 patients who received ATG, 33 were evaluable for a response; 14 patients had either a complete (4 patients) or partial (10 patients) response, for an overall response rate of 42.4%. Of the 14 DR2+ patients who received ATG, 7 responded, for a 50% response rate, which is not significantly higher than the response rate for the DR2- patients (7 of 19 [36.8%]; P = .50). The median survival of patients who are DR2+ was slightly, but not significantly, longer than that of the DR2- patients in the ATG group (P = .19). Although the incidence of HLA DR2 was clearly increased in these patients with aplastic anemia, response rates to ATG were not significantly different in the DR2+ and DR2- patients.

Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus.

Autoimmune myelofibrosis is an uncommon disorder in which patients present with anemia and thrombocytopenia in conjunction with limited clinical manifestations of autoimmune disease or an exacerbation of previously established SLE. The presence of leukoerythroblastosis in a patient with SLE may suggest the presence of myelofibrosis. Conversely, the absence of splenomegaly in a patient with presumed idiopathic myelofibrosis may suggest an autoimmune etiology. Patients with autoimmune myelofibrosis universally have a positive ANA test and frequently have either elevated anti-DNA titers or a positive LE cell preparation. Because physical manifestations of autoimmune disease may not be evident at presentation, all patients found to have myelofibrosis should have an ANA test. Peripheral blood cytopenias in autoimmune myelofibrosis frequently respond to glucocorticoids but regression of bone marrow fibrosis may be incomplete. Hematologic response to treatment parallels that of the associated autoimmune disease.