Internists' attitudes towards terminal sedation in end of life care.

OBJECTIVE: To describe the frequency of support for terminal sedation among internists, determine whether support for terminal sedation is accompanied by support for physician assisted suicide (PAS), and explore characteristics of internists who support terminal sedation but not assisted suicide. DESIGN: A statewide, anonymous postal survey. SETTING: Connecticut, USA. PARTICIPANTS: 677 Connecticut members of the American College of Physicians. MEASUREMENTS: Attitudes toward terminal sedation and assisted suicide; experience providing primary care to terminally ill patients; demographic and religious characteristics. RESULTS: 78% of respondents believed that if a terminally ill patient has intractable pain despite aggressive analgesia, it is ethically appropriate to provide terminal sedation (diminish consciousness to halt the experience of pain). Of those who favoured terminal sedation, 38% also agreed that PAS is ethically appropriate in some circumstances. Along a three point spectrum of aggressiveness in end of life care, the plurality of respondents (47%) were in the middle, agreeing with terminal sedation but not with PAS. Compared with respondents who were less aggressive or more aggressive, physicians in this middle group were more likely to report having more experience providing primary care to terminally ill patients (p = 0.02) and attending religious services more frequently (p<0.001). CONCLUSIONS: Support for terminal sedation was widespread in this population of physicians, and most who agreed with terminal sedation did not support PAS. Most internists who support aggressive palliation appear likely to draw an ethical line between terminal sedation and assisted suicide.

Anemia of pregnancy.

A general work-up for anemia during pregnancy could follow a simplified flow diagram, such as the one shown in Figure 1. More specific laboratory tests should be selected as needed.

The mast cell and mast cell disease.

Mast cell disease or mastocytosis is a heterogeneous group of clinical disorders characterized by the proliferation and accumulation of mast cells in a variety of tissues, most often the skin. The signs and symptoms of mast cell disease are varied, dependent on the localization of mast cells in different organs and the local and systemic effects of mediators released from these cells. Although mast cell disease is most commonly identified in the skin, involvement of the skeletal, hematopoietic, gastrointestinal, cardiopulmonary, and central nervous systems may be seen. Clinical management of mastocytosis depends most heavily on knowledge of the diverse effects of mast cell mediators on various tissues and organs, the stimuli that can cause their release, and the different methods available for blocking the effects of these mediators.

Pericardial tamponade in chronic myelomonocytic leukemia.

We report four cases of malignant pleuropericardial effusions in patients with chronic phase myelomonocytic leukemia (CMML). Based on current literature, this event is rare and very poorly understood. Our cases shed light on three important clinical characteristics: (1) patients with CMML develop effusions during uncontrolled leukocytosis; (2) these effusions are very responsive to conventional chemotherapy; and (3) effusions may develop without clinical forms of disease in other sites of extramedullary hematopoiesis.

Isolated amyloidosis of the penile urethra and corpus spongiosum: a case report.

Localized amyloidosis of the penile urethra and corpus spongiosum is rare. The pathogenesis is obscure and optimal management of such patients has not been defined. We report a case that was treated with transurethral removal of amyloid tissue, which was obstructing the urethral outlet. Previously, the patient had been treated with dilation with temporary relief of the symptoms. He was disease-free 1 1/2 years postoperatively.

Sickle myonecrosis revisited.

This review focuses on the classification of sickle myonecrosis in the context of sickle cell vaso-occlusive crisis. Further, the potential and novel use of magnetic resonance imaging to further clarify the nature of sickle crisis is discussed.

Successful childbirth by a patient with congenital factor XI deficiency and an acquired inhibitor.

Acquired inhibitors in factor XI deficiency (FXI) are rare. The presence of an inhibitor during pregnancy poses a potential haemorrhagic risk to the fetus. We report an uncomplicated pregnancy and successful childbirth by a woman with congenital FXI deficiency and an acquired inhibitor, and discuss the persistence of residual FXI activity in the presence of an inhibitor.

Morphologic, biochemical, and cytogenetic studies of bone marrow and circulating blood cells in painters exposed to ethylene glycol ethers.

In a previous cross-sectional survey, up to 15% of shipyard painters were found to have mild anemia or granulocytopenia, mostly acquired since employment. Environmental studies had suggested a possible etiologic role for ethylene glycol ethers, solvents to which the men were heavily exposed and which have established myelotoxic potential. To exclude alternative hypotheses, examine possible common patterns of injury, and identify potential risk factors and markers for such an effect, the affected painters were further studied. The painters were matched with two groups of controls: exposed painters without evidence of hematologic abnormality on the previous survey and unexposed controls. Altogether 25 subjects were studied by histopathologic examination of bone marrow, cytogenetic studies of marrow cells, and peripheral lymphocytes and peripheral red cell studies of membrane and metabolic function. Except for an unexpected finding of a race-associated effect on marrow histology, insignificant differences were seen among the groups in terms of marrow morphology and cellularity, stem cell growth kinetics, and marrow or peripheral cytogenetics. Two metabolic abnormalities of peripheral red cells related to exposure or clinical status of the subjects were found. Pyruvate kinase, an established marker of acquired myelodysplasia, was significantly depressed in the subjects with previously abnormal counts. Although reduced glutathione levels and holoenzyme activities of glutathione reductase (GSHR) did not differ among groups, exposed subjects had decreased saturation of GSHR with flavin adenine dinucleotide which could be restored in vitro, suggesting riboflavin deficiency or impaired riboflavin metabolism. Thus, although a unique pattern of bone marrow injury by histologic or genetic assay attributable to ethylene glycol ethers was not defined, biochemical effects of possible mechanistic importance were identified. The relevance of these findings as subclinical disease markers remains to be established.

Disseminated intravascular coagulation and excessive fibrinolysis in a patient with metastatic prostate cancer. Response to epsilon-aminocaproic acid.

BACKGROUND: Disseminated intravascular coagulation (DIC) and primary fibrinolysis have both been reported in association with prostate carcinoma. The correct diagnosis of the coagulopathy can be difficult and the appropriate management controversial. METHODS: A case is presented of a man in whom DIC and soft tissue hemorrhage developed after prostatic biopsy. The results of therapy and a review of the literature are discussed. RESULTS: Fibrinogen levels continued to decrease despite high-dose estrogen therapy, but they rapidly returned to normal after therapy with epsilon-aminocaproic acid. Although routine coagulation tests were suggestive of primary fibrinolysis, the results of the D-dimer assay confirmed that the patient had DIC associated with excessive fibrinolysis. CONCLUSION: A review of the literature suggests that most cases of primary fibrinolysis are probably DIC with excessive secondary fibrinolysis. In cases in which bleeding is the primary manifestation of DIC and there is a significant reduction in alpha-2-plasmin inhibitor activity, a trial of epsilon-aminocaproic acid and low-dose heparin should be considered. The failure in this case of estrogen therapy to correct the coagulopathy, despite a later good tumor response, is consistent with the delay in which anorchid testosterone levels are obtained after initiating treatment.

Rationing health care: its impact and implications for hematology-oncology.

Rationing of health care in the United States currently exists via the covert mechanism of restricting significant segments of medical care for many of those who cannot afford it. Provision of universal health care would necessitate explicit rationing of certain interventions and technologies, even though an individual could afford them. The British and Canadian experiences provide lessons from which America can profit, and the Oregon health plan is an experiment in this direction. The progressive "graying" of America has raised the question of the need for intergenerational charity as a form of rationing. The implications of these rationing plans would result in a major restructuring of the practice of hematology-oncology.