Severe aplastic anemia associated with chronic mucocutaneous candidiasis. Immunologic and hematologic reconstitution after allogeneic bone marrow transplantation.

Chronic mucocutaneous candidiasis (CMC) is typically associated with the inability of T lymphocytes to proliferate and produce lymphokines in response to Candida antigen. A 7-year-old girl with CMC developed severe aplastic anemia and, after conditioning with cyclophosphamide, 200 mg/kg, underwent bone marrow transplantation from her HLA-identical sister. Engraftment was prompt and complete. The patient is surviving more than 3 years after transplantation with normal donor-derived hemopoiesis and immune function. Manifestations of CMC have resolved completely and she has not received antifungal therapy for more than 2 years.

National survey of neonatal transfusion practices: II. Blood component therapy.

Neonatal transfusion practices during 1989 of 452 institutions involved in transfusing infants were surveyed by questionnaire. Most respondents (77%) transfused fresh frozen plasma appropriately (ie, primarily to treat coagulation disorders). However, 11% stated that their most frequent use of fresh frozen plasma was solely to treat hypovolemia, a practice generally not recommended. Seventy-eight percent of respondents transfused platelets to treat bleeding infants with blood platelet counts of less than 50 x 10(9)/L; 84% gave platelets to sick, premature neonates with counts of less than 50 x 10(9)/L whether or not bleeding was evident. Only 35% of respondents transfused granulocytes for neonatal sepsis; most institutions used buffy coats isolated from units of blood--a product readily available, but of questionable efficacy when compared with leukapheresis granulocytes. Ninety-three percent of respondents provided blood components with low risk of transmitting cytomegalovirus: components from seronegative donors were used by 84%, leukocyte-reduced products by 6%, and a combination by 10%. Thirteen percent of respondents gave gamma-irradiated blood components to all and 46% gave them to some neonates to prevent graft vs host disease. Forty-one percent did not routinely irradiate. Ten percent of respondents used leukocyte reduction instead of gamma irradiation to prevent graft vs host disease, a practice currently not advocated. Thus, national transfusion practices for neonates are variable, controversial, and, occasionally, other than those usually recommended. Additional research and educational efforts are needed to ensure optimal transfusion therapy.

National survey of neonatal transfusion practices: I. Red blood cell therapy.

Neonatal blood component transfusion practices during 1989 were surveyed via a questionnaire developed by the Pediatric Hemotherapy Committee of the American Association of Blood Banks. Of 1790 questionnaires mailed, 452 were selected to form the database for this analysis because they were from institutions in which neonates were transfused. Nearly all institutions contained intensive care units directed by neonatologists and were involved in the management of high-risk infants. Results from institutions serving as the primary pediatric teaching hospital of a medical school were compared with those with no medical school affiliation. Thirty-six percent of primary pediatric teaching hospitals and 52% of hospitals with no medical school affiliation performed pretransfusion testing in excess of that required, resulting in additional blood loss in neonates. Sixty-six percent of primary pediatric teaching hospitals used fresh frozen plasma to adjust the hematocrit of red blood cell concentrates prior to transfusion (a practice increasing donor exposure), compared with only 29% of hospitals with no medical school affiliation. The usual indication for small-volume red blood cell transfusions in severely ill neonates was to maintain a desired hematocrit level, whereas for stable infants, red blood cell transfusions were given to treat symptomatic anemia, rather than to maintain a predetermined hematocrit. As found in 1985, neonatal transfusion practices in 1989 were variable. However, improvements have occurred since 1985 to suggest that further research and educational efforts may serve to promote even better neonatal transfusion therapy.

Aspirin and recurrent hyphema after blunt ocular trauma.

Recurrent hyphema after traumatic blunt injury to the eye is associated with a more serious prognosis than that occurring from the initial trauma, resulting in a higher risk of glaucoma, corneal staining, surgical intervention, poor visual acuity, and enucleation. Risk factors associated with the development of recurrent bleeding are not well defined, but recent evidence suggests a high association with concurrent aspirin ingestion. Of 25 consecutive patients with hyphemas (20 males and five females, ranging in age from 2 to 53 years), 12 took aspirin after the initial trauma and seven had recurrent hyphemas. Platelet aggregation determinations in these seven patients showed defects associated with aspirin. Only one of 13 patients without aspirin intake had recurrent bleeding.

Irrigation with effluents: effects of prewetting rate and clay content on runoff and soil loss.

Stability of soil surface aggregates and irrigation water quality significantly affect runoff and soil erosion. Slow wetting of aggregates prevents slaking. We hypothesized that wetting rate will affect a soil's susceptibility to seal formation and soil erosion, and that this susceptibility will differ between effluent- and fresh water (FW)-irrigated soils. Effects of prewetting rate (2, 4, 8, and 64 mm h(-1)) on runoff and interrill erosion from five Israeli soils exposed to 60 mm of simulated rain were studied in the laboratory. Soils were taken from fields irrigated with FW or effluents for >15 yr. In general, for effluent- and FW-irrigated samples, runoff and soil loss were greatest for the soil with 22.5% clay; at lower or higher clay contents, less runoff and soil losses were noted. Runoff and soil loss decreased with decreasing prewetting rate (PWR) mainly in soils with clay content > or =38%. Total runoff and soil loss were higher in effluent-irrigated soils than in FW-irrigated ones in the loamy sand (8% clay) only. Greater soil losses occurred from effluent-irrigated soils exposed to fast PWR (64 m h(-1)) compared with FW-irrigated soils. In general, PWR had similar effects on total runoff and soil loss for effluent- and FW-irrigated soils. Use of wetting rates < or =8 mm h(-1) to prevent aggregate slaking decreased runoff and soil loss from loam and clayey soils exposed to simulated rainfall of high kinetic energy (15.9 kJ m(-3)). Long term irrigation with effluents in soils containing >20% clay did not seem to adversely affect soil susceptibility to runoff and soil loss in soils exposed to simulated rainfall, beyond that observed in FW-irrigated soils.

Transfusion-associated noncardiogenic pulmonary edema. Report of a case and a warning regarding treatment.

Although noncardiogenic pulmonary edema (NCPE) is a recognized complication of blood transfusion, the precise etiology is not well understood. NCPE may be secondary to complement-mediated pulmonary capillary injury initiated by either donor or recipient anti-leukocyte antibodies. It is not caused by simple volume overload. Recent blood banking texts and published case reports continue to suggest diuretics as part of the initial therapy for this complication. We report a case of transfusion-associated NCPE in which empirical diuretic therapy clearly was detrimental and suggest that the use of diuretics for treatment of this condition is not warranted. Reversal of progressive hypoxemia is the mainstay of therapy. Hemodynamic monitoring is important in differentiating NCPE from pulmonary edema secondary to cardiac failure or volume overload and should be used as a guide for further therapy.

The accumulation of malonyldialdehyde, an end product of membrane lipid peroxidation, can cause potassium leak in normal and sickle red blood cells.

This study has examined the effect of malonyldialdehyde (MDA), an end product of lipid peroxidation, on the K+ leak in normal (AA) and sickle (SS) red blood cells (RBCs). In vitro MDA accumulation in human RBCs was accomplished by treating them with exogenous standard MDA. MDA accumulation assessed by the thiobarbituric acid reactivity of in vitro MDA-treated RBCs was comparable to the RBCs in hemolytic anemias. There was a significant K+ leak in AA RBCs after in vitro treatment with MDA. The effect of MDA on the K+ leak was greater in SS RBCs. The increase in cellular K+ leak was significantly positively correlated with the extent of MDA accumulation as assessed by thiobarbituric acid reactivity.

The effect of malonyldialdehyde on viscosity of normal and sickle red blood cells.

This study has examined the effect of MDA, an end product of lipid peroxidation, on the viscosity of AA and SS RBC suspensions. MDA accumulation in RBC was accomplished in vitro in human RBC by treating them with exogenous standard MDA. MDA accumulation assessed by the thiobarbituric acid reactivity of in vitro MDA-treated RBC was comparable to that of the RBC in vivo in hemolytic anemias. There was a significant increase in the viscosity of both AA and SS RBC suspensions after in vitro treatment with MDA. The increase in viscosity or RBC was significantly positively correlated with the extent of MDA accumulation.

A survey of Canadian neonatal blood transfusion practices.

In 1990, the Pediatric Hemotherapy Committee of the American Association of Blood Banks developed and distributed a questionnaire addressing neonatal blood transfusion practices. The same questionnaire was subsequently sent to Canadian university-affiliated hospitals (n = 92). This report describes the results of the Canadian survey. Seventy-two percent (n = 66) of institutions contacted responded. Of these 42% (n = 28) had sufficient experience with neonatal transfusions and provided sufficient data for analysis. Although the majority of stated practices did follow published guidelines, several areas of variability and/or suboptimal practices were identified. With respect to component selection and preparation, suboptimal practices included excessive pretransfusion testing, unnecessary routine washing of RBC concentrates for small-volume transfusions, routine volume reduction of platelet concentrates and the use of suboptimal granulocyte preparations. With respect to transfusion practices, a disturbingly high percentage of respondents indicated that frozen plasma would be given in situations generally considered inappropriate. There was a great deal of variability in the provision of blood components at low risk for CMV, in the use of gamma irradiation and in the platelet count used for prophylactic platelet transfusions. The data collected in this survey provide information concerning practices that require improvement, identify areas where further research is desirable and provide a basis for comparison with current and future neonatal blood transfusion practices.

Guidelines for auditing pediatric blood transfusion practices.

Although transfusion of blood products is an essential and potentially life-saving measure, not all blood transfusions are beneficial to patients. The associated risks, particularly transfusion-transmitted viral illnesses, such as hepatitis and acquired immunodeficiency syndrome, require that careful consideration be given before a decision is made to transfuse any blood product. Many institutions have established a local committee to monitor transfusion practices and audit such practices regularly. To assist in this task, the Pediatric Hemotherapy Committee of the American Association of Blood Banks has developed guidelines for the conduct of pediatric blood transfusion audits. These guidelines, summarized herein, cover transfusion of red blood cells, platelets, white blood cells, fresh-frozen plasma, albumin, and clotting concentrates. The use of cytomegalovirus low-risk and irradiated blood products is also discussed. Throughout the report, special attention is given to the transfusion needs of newborn infants.

Clinical significance of anti-Yt(b). Report of a case using a 51chromium red cell survival study.

Several published reports have documented the variable survival of Yt(a+) red cells (RBC) in patients with anti-Yt(a) as measured by 51Chromium (Cr)-labeled RBC survival studies. Similar studies with anti-Yt(b) have not been reported. A 51Cr-labeled RBC survival study was performed using Yt(b+) RBCs and a monocyte monolayer assay in a young hemodialysis patient who required chronic transfusion therapy and who had developed anti-Yt(b). The survival of the transfused RBCs was 100 and 93 percent at 1 and 24 hours, respectively, with a half life of 21 days at termination of the study (normal, 28 to 32 days). These results showed no evidence of rapid destruction of the Yt(b+) RBCs, indicating that this patient could be transfused safely with blood from Yt(b+) donors. Long-term survival of the 51Cr-labeled Yt(b+) RBCs was shortened moderately, however, a finding that correlated with a slightly abnormal monocyte monolayer assay test.

Use of L-asparaginase and cytosine arabinoside for refractory acute lymphocytic leukemia with particular reference to T-cell leukemia.

Ten patients with late-stage acute lymphocytic leukemia were treated with L-asparaginase and cytosine arabinoside. Complete remission was achieved in 8 patients including 5 of 5 patients with T-cell leukemia. Major toxicity included anaphylactic reactions in 3 of the 10 patients.

National acceptability of American Association of Blood Banks Pediatric Hemotherapy Committee guidelines for auditing pediatric transfusion practices.

In 1989, guidelines for the auditing of pediatric transfusion practices were developed by the Pediatric Hemotherapy Committee of the American Association of Blood Banks (AABB) and made available to AABB members. A survey of members who requested the guidelines was conducted to determine how consistent the guidelines were with local transfusion practices and how useful they were for the conduct of audits. The majority of respondents indicated that the recommended audit criteria agreed with local practices and that most of them could be applied to their transfusion practice audits with little or no modification. An exception was that criteria for the transfusion of platelets to premature infants were considered by some to be too liberal. However, after review of the comments and the published information available, the committee elected not to revise the guidelines pertaining to platelet transfusions for premature infants. Bearing in mind that audit criteria are intended to identify circumstances in which transfusions are acceptable as reasonable therapy without need for further justification, rather than to serve as indications for transfusions, the AABB Pediatric Hemotherapy Committee guidelines for auditing pediatric transfusion practices are fairly representative of national practice.