Hemolysis during percutaneous mechanical thrombectomy can mimic a hemolytic transfusion reaction.

BACKGROUND: Interventional radiologists have developed percutaneous mechanical thrombectomy (PMT) devices to remove intravascular thrombi. Hemolysis, secondary to thrombus destruction from these devices, has been described in radiology journals, but similar reports appear to be lacking in the transfusion medicine literature. Two cases of hemolysis after PMT are described that involved the transfusion service, one of which was reported as a hemolytic transfusion reaction. CASE REPORTS: The first patient received 4 units of red cells (RBCs) during a thrombectomy and subsequent placement of a transjugular intrahepatic portosystemic shunt. The patient developed hemoglobinuria, and it was reported to the blood bank as a possible hemolytic transfusion reaction. After RBC mismatch and bacterial contamination were excluded, the hemolysis was attributed to thrombectomy-related mechanical hemolysis. In the second case, a hemolyzed sample was sent to the blood bank for a type and cross-match. Upon requesting that the sample be redrawn, it was learned that the sample was obtained after PMT. CONCLUSION: Patients who have undergone PMT can have clinical and laboratory findings suggestive of hemolytic transfusion reactions. Although interventional radiologists are familiar with these side effects, the blood bank profession needs to be aware that these procedures cause nonimmune hemolysis and must consider this possibility when evaluating transfusion reactions in these patients.

A fifty-year follow-up of ocular defects in congenital rubella: late ocular manifestations.

Fifty patients born with congenital rubella in 1939-1945 were reviewed in 1967. In 1991 this cohort was again studied and their ophthalmic features are presented here. The presence of two new cataracts and one case of possible choroidal neovascularisation are again noted; apart from these three eyes, the group showed no evidence of other delayed manifestations of the congenital rubella syndrome. A review is given of the ocular complications of the congenital rubella syndrome, with particular emphasis on the late complications.

Gallium-67 and technetium-99m-methylene diphosphonate skeletal scintigraphy in determining prognosis for children with stage IV neuroblastoma.

Thirty-five children (aged 0-9 yr) who had presented with Stage IV neuroblastoma were studied to see if avidity for 67Ga or 99mTc-methylene diphosphonate (MDP) uptake in both primary and secondary sites at diagnosis conferred any prognostic significance. Twenty-three percent of the patients were disease free and off treatment at the time of study. Crude survival did not differ between groups. Duration of survival and the likelihood of completing treatment were related to the scintigraphic appearance at the time of diagnosis, after adjustment for potential confounding effects, using Cox's proportional hazards regression and multiple logistic regression. After adjustment for confounding influences, neither 67Ga avidity nor uptake of 99mTc-MDP was associated with a significantly worse prognosis, both in terms of adjusted survival and likelihood of completing treatment. Patients with 67Ga-avid scans at diagnosis did not demonstrate significantly worse survival (HR 1.47, 95% CI 0.43-5.11) than those without 67Ga avidity. They were somewhat less likely to complete treatment (OR 0.23, 95% CI 0.03-1.63), but this did not reach statistical significance. Similarly, although patients with 99mTc-MDP positive scans demonstrated somewhat worse survival (HR 2.47, 95% CI 0.45-13.54), this result did not reach statistical significance, nor were they less likely to complete treatment (OR 0.69, 95% CI 0.07-6.67) than those with 99mTc-MDP negative scans. Uptake of 99mTc-MDP into extraosseous sites was also not associated with worse survival (HR 1.45, 95% CI 0.58-3.62) nor with decreased likelihood of completing treatment (OR 0.78, 95% CI 0.12-5.09). Other than indicating disease stage, these results do not support the hypothesis that the scintigraphic appearance at diagnosis confers prognostic information in children with advanced neuroblastoma.

A fifty-year follow-up of congenital rubella.

50 patients with congenital rubella, born in 1939-43, were reviewed in 1967. Here we report their outcome in 1991. Since 1967, there have been 7 deaths (3 cardiovascular, 3 malignant disease, 1 AIDS). 40 had full clinical assessment. The prevalence of diabetes mellitus is similar to that in 1967: 4 of the 5 reported diabetic then, remain so, and there is 1 new case. 1 subject has malignant melanoma and 3 have died from cancer. Although the incidence of malignant disease is not increased, the death rate is (standardised mortality rate 6.0, 95% CI 1.24-17.57). Longer follow-up will be required to confirm this observation.

Bulbar and pseudobulbar palsy complicating therapy with high-dose cytosine arabinoside in children with leukemia.

Three of 38 children given high-dose cytosine arabinoside therapy developed a previously undescribed complication. Neurological problems are a frequent occurrence in patients given this therapy, particularly cerebellar ataxia, but the development of bulbar and pseudobulbar palsy has not been reported. In two of these cases, it was sufficiently marked for the course of treatment to be curtailed and occurred at a relatively low cumulative dose of the drug. Neurotoxicity can occur at any time using high-dose cytosine therapy.

Japanese encephalitis after a two-week holiday in Bali.

Japanese encephalitis is described in a 10-year-old girl after a short holiday in Bali. Four days after returning to Australia the patient presented with a high fever, stupor and rapidly-developing focal neurological signs. Recovery occurred gradually over a period of three months and she has returned to school. Japanese encephalitis viral infection was confirmed by a marked rise in specific haemagglutination-inhibition antibodies and the presence of immunoglobulin M antibodies to the flavivirus group. It is important to be aware of the possibility of arboviral infection in patients with encephalitis. In view of the recent outbreaks of Japanese encephalitis in Asia, travellers to the region should be warned to protect themselves from mosquito-bites.

Growth and growth hormone therapy of children treated for leukaemia.

A total of 37 children (24 male, 13 female) who had been treated for leukaemia with chemotherapy and 24 Gy cranial irradiation, and who were disease free for at least 18 months, were commenced on somatrem at a mean of 7.6 years (range, 4.8-12.1 years) after leukaemia diagnosis because of growth rate below the 25th centile for bone age. Peak GH response to provocation (exercise, arginine, insulin hypoglycaemia) was less than 20 milliunits/litre in 27 children (deficient group) and 20 milliunits/litre or more in 10 children (non-deficient group). The mean height SD decrease from diagnosis of leukaemia to commencement of somatrem was 1.98, 86% of the children decreasing by more than 1 SD. Those who were tall for age at leukaemia diagnosis and females were more severely affected. Mean (+/- SD) height velocity increased on somatrem from 2.7 +/- 1.1 to 6.6 +/- 2.2 cm/year during the first 6 months (n = 25), and to 6.0 +/- 1.7 cm/year during the first 12 months (n = 19). No difference in growth response was seen between the sexes or between the deficient and non-deficient groups. Catch-up growth occurred for the first 6 months only. It is concluded that children with a low growth rate after treatment of leukaemia should be considered for GH therapy irrespective of the results of GH provocative tests.

Unusual skin rash following withdrawal of oral 6-mercaptopurine in children with leukemia.

Sixteen episodes of a distinctive, papular rash occurred in eight patients following withdrawal of 6-mercaptopurine (6MP) and methotrexate (MTX) used as maintenance therapy for acute lymphoblastic leukemia (ALL). The rash also developed in one of the eight patients when only 6MP was discontinued. The eruption occurred mainly on the face, and in this site resembled the perioral dermatitis seen following withdrawal of topical fluorinated steroids. The rash generally began within 3 weeks of stopping 6MP and lasted 3 to 4 weeks. It failed to improve with the use of topical corticosteroid. We conclude that this rash is caused by the withdrawal of oral 6MP.

Growth failure and growth-hormone deficiency after treatment for acute lymphoblastic leukaemia.

In a study of 77 children who had been treated for acute lymphoblastic leukaemia (ALL) with an LSA2L2 (Memorial Sloan-Kettering) chemotherapy protocol plus radiotherapy (24 Gy) as cranial prophylaxis, growth was examined 3.0-9.5 years after diagnosis. The children's growth slowed and they crossed height percentiles towards the end of or after treatment. The Z-score, which reflects the deviation of height measurements from the population mean, was used to assess height change. The mean Z-score was 0.16 at diagnosis, -0.30 2 years later, -0.71 4 years later, and -1.37 6 years later. Height for age had fallen by more than 1 standard deviation of the population mean in 32% of survivors 4 years after diagnosis and in 71% 6 years after diagnosis. Younger children and those tall for age at diagnosis were more severely affected. Growth-hormone (GH) response to standard provocation tests was measured in 46 patients; 30 had partial or complete GH deficiency. Mean pulsatile GH secretion was low in the 34 patients tested. Cranial irradiation is probably the most important causative factor in the development of GH deficiency in survivors of ALL.

Coxsackie B, mumps, rubella, and cytomegalovirus specific IgM responses in patients with juvenile-onset insulin-dependent diabetes mellitus in Britain, Austria, and Australia.

Patients from England, Austria, and Australia with recently diagnosed juvenile-onset insulin-dependent diabetes (type 1) mellitus (IDDM) and matched controls were tested for specific IgM responses to Coxsackie B1-5 viruses. 37 of 122 (30%) patients aged less than 15, but only 15 of 204 (6%) controls, were positive (p less than 0.005). Differences in Coxsackie B virus specific IgM responses between patients and controls were statistically significant for patients in England and Austria (p less than 0.005). Coxsackie B virus specific IgM responses were detected in only 3 of 31 patients aged greater than 16. Virus-specific IgM responses were directed against a single serotype, usually Coxsackie B4 or 5, in 23 of 37 (62.5%) children aged less than 15; 10 of 13 (77%) of children aged less than 7 had monotypic responses. Among families of Austrian patients with IDDM, 8 of 79 (10%) siblings had Coxsackie B virus specific IgM responses, 1 of whom subsequently had IDDM, but none of the 80 parents was positive. In contrast, there was no evidence of recent infection by mumps, rubella, or cytomegalovirus (CMV), since mumps-virus specific IgM was present in only 2 of 100 children with IDDM and 5 of 139 controls; no rubella or CMV specific IgM responses were detected in 60 sera from patients with IDDM.

Epidemiology of congenital rubella and results of rubella vaccination in Australia.

Congenital rubella was not notifiable in Australia until recently, but the national incidence of childhood deafness has been accurately documented since 1949. On the basis of these data and a study of congenital rubella in Western Australia, it is estimated that the national incidence of congenital rubella has decreased from a mean of at least 120 cases annually (one in 2,000 live births) to approximately 20 or less since 1977. This decrease has occurred since the introduction of rubella vaccination programs in 1971. The aim of these programs was to reduce the incidence of congenital infection by vaccinating girls (aged 10-14 years) at school without assessing their immunity. Nonpregnant seronegative women were also offered vaccination in family planning clinics as were postpartum seronegative women in obstetric units. By 1983, 96% of 8,226 pregnant women were seropositive for rubella antibody, as compared with only 82% of a similar group of women in 1971. This improvement in the immune status of pregnant women appears to be the result of rubella vaccination. Rubella vaccination also appears to have been successful in preventing congenital infection.

Impact of rubella vaccination in Australia.

Rubella vaccination of schoolgirls aged 10-14 years started 13 years ago in Australia; the girls were vaccinated without prior assessment of their immunity. Non-pregnant seronegative women were also offered vaccination in family-planning clinics and post partum in obstetric units. Serological follow-up of 191 schoolgirls 5 years after vaccination and 56 girls 8 years after vaccination showed that all had detectable rubella antibody, whereas 24% of 239 unvaccinated young men of similar age (18-23 years) were seronegative. In 1983, 96% of 8226 pregnant women aged 12-53 years (mean 25.3 years) had detectable rubella antibody, and since 1977 there has been a striking reduction in the incidence of deafness due to congenital rubella. These results indicate that the rubella vaccination programme in Australia is having a significant impact on both the serological status of pregnant women and on the incidence of congenital infection.

Population studies of diphtheria immunity using antitoxin radioimmunoassay.

A quantitative method for testing serum diphtheria antitoxin levels was set up using a diphtheria antitoxin radioimmunoassay (RIA). The results of this RIA correlated well with the Schick test in 554 subjects and with intradermal neutralization tests in guinea-pigs in a small group of subjects. The RIA was suitable for use on blood collected by fingerprick on to a disc of standard chromatography paper. These discs could be stored at room temperature for at least 1 month. If storage for more than 6 months was required -20 degrees C was found to be better. Experience with this RIA in a total of 2349 subjects indicated that it is more accurate, rapid and less costly than Schick testing. The RIA should prove to be the preferred method for testing diphtheria immunity in population surveys.

Pancreatic islet and other autoantibodies in juvenile and adult onset diabetics in Australia.

The prevalence of serum antibodies to the cytoplasm of the pancreatic islet cell (PICA), and of thyroid microsomal (TMA), gastric parietal cell (GPCA) and anti-nuclear (ANA) antibodies was studied in 135 newly diagnosed diabetics presenting to a hospital for adults and 83 children with recent onset diabetes presenting to a children's hospital. The study also included another 144 diabetic children whose disease had been present longer, and 200 control children. There was a high prevalence (87%) of PICA in the children whose diabetes had just been diagnosed in comparison with control children (1%):(P less than .0001). Diabetic children also had a high prevalence (21%) of one or more of the other autoantibodies in comparison with the control children (9%):(P less than .001). Only 26% of the 58 insulin dependent adults had PICA but 33% had other autoantibodies. Two (3%) of the 77 adult diabetics who did not require insulin had PICA; 8% had other autoantibodies.