[Asperger syndrome - a too late diagnosed disorder?]. / Asperger-Syndrom - eine zu spät diagnostizierte Störung?

Asperger syndrome is a disorder within the autistic spectrum, which was first described by Hans Asperger in 1944. It belongs to the group of pervasive developmental disorders and is particularly characterized by qualitative impairments of social interaction and communication as well as distinct special interests and stereotyped patterns of behaviour. We present a patient, showing the typical behavioural symptoms of the Asperger syndrome, which were first diagnosed at the age of sixteen.

Adult patients with de novo acute myeloid leukemia and t(9; 11)(p22; q23) have a superior outcome to patients with other translocations involving band 11q23: a cancer and leukemia group B study.

Following reports of childhood acute myeloid leukemia (AML) showing that patients with t(9; 11)(p22; q23) have a better prognosis than those with translocations between 11q23 and other chromosomes, we compared response to therapy and survival of 24 adult de novo AML patients with t(9; 11) with those of 23 patients with other 11q23 translocations [t(11q23)]. Apart from a higher proportion of French-American-British (FAB) M5 subtype in the t(9; 11) group (83% v 43%, P = .006), the patients with t(9; 11) did not differ significantly from patients with t(11q23) in terms of their presenting clinical or hematologic features. Patients with t(9; 11) more frequently had an extra chromosome(s) 8 or 8q as secondary abnormalities (46% v 9%, P = .008). All patients received standard cytarabine and daunorubicin induction therapy, and most of them also received cytarabine-based intensification treatment. Two patients, both with t(9; 11), underwent bone marrow transplantation (BMT) in first complete remission (CR). Nineteen patients (79%) with t(9; 11) and 13 (57%) with t(11q23) achieved a CR (P = .13). The clinical outcome of patients with t(9; 11) was significantly better: the median CR duration was 10.7 versus 8.9 months (P = .02), median event-free survival was 6.2 versus 2.2 months (P = .009), and median survival was 13.2 versus 7.7 months (P = .009). All patients with t(11q23) have died, whereas seven (29%) patients with t(9; 11) remain alive in first CR. Seven of eight patients with t(9; 11) who received postremission regimens with cytarabine at a dose of 100 (four patients) or 400 mg/m2 (2 patients) or who did not receive postremission therapy (2 patients) have relapsed. In contrast, 7 (64%) of 11 patients who received intensive postremission chemotherapy with high-dose cytarabine (at a dose 3 g/m2) (5 patients), or underwent BMT (2 patients) remain in continuous CR. We conclude that the outcome of adults with de novo AML and t(9; 11) is more favorable than that of adults with other 11q23 translocations; this is especially true for t(9; 11) patients who receive intensive postremission therapy.

All-trans-retinoic acid in acute promyelocytic leukemia.

BACKGROUND: All-trans-retinoic acid induces complete remission in acute promyelocytic leukemia. However, it is not clear whether induction therapy with all-trans-retinoic acid is superior to chemotherapy alone or whether maintenance treatment with all-trans-retinoic acid improves outcome. METHODS: Three hundred forty-six patients with previously untreated acute promyelocytic leukemia were randomly assigned to receive all-trans-retinoic acid or daunorubicin plus cytarabine as induction treatment. Patients who had a complete remission received consolidation therapy consisting of one cycle of treatment identical to the induction chemotherapy, then high-dose cytarabine plus daunorubicin. Patients still in complete remission after two cycles of consolidation therapy were then randomly assigned to maintenance treatment with all-trans-retinoic acid or to observation. RESULTS: Of the 174 patients treated with chemotherapy, 120 (69 percent) had a complete remission, as did 124 of the 172 (72 percent) given all-trans-retinoic acid (P=0.56). When both induction and maintenance treatments were taken into account, the estimated rates of disease-free survival at one, two, and three years were 77, 61, and 55 percent, respectively, for patients assigned to chemotherapy then all-trans-retinoic acid; 86, 75, and 75 percent for all-trans-retinoic acid then all-trans-retinoic acid; 75, 60, and 60 percent for all-trans-retinoic acid then observation; and 29, 18, and 18 percent for chemotherapy then observation. By intention-to-treat analysis, the rates of overall survival at one, two, and three years after entry into the study were 75, 57, and 50 percent, respectively, among patients assigned to chemotherapy, and 82, 72, and 67 percent among those assigned to all-trans-retinoic acid (P= 0.003). CONCLUSIONS: All-trans-retinoic acid as induction or maintenance treatment improves disease-free and overall survival as compared with chemotherapy alone and should be included in the treatment of acute promyelocytic leukemia.

Computed tomographic enhancement of the liver, liver abscesses, spleen, and major vessels with perfluorooctylbromide emulsion. Influence of dosage and injection velocity in an animal model.

RATIONALE AND OBJECTIVES: Computed tomographic (CT) enhancement of the liver, liver abscess, spleen, and major vessels was investigated between 2 and 48 hours after intravenous administration of perfluorooctylbromide (PFOB emulsion) in an animal model of 63 rabbits. METHODS: Twenty-one animals received 3 g/kg PFOB as a fast bolus injection. Using a slow infusion rate, the same number of animals received either the same dose (3 g/kg) or half the dose (1.5 g/kg). RESULTS: Vascular enhancement was best after bolus injection of 3 g/kg emulsion. The density peak occurred after 2 hours. A continuous enhancement of approximately 100 Hounsfield units (HU) was observed up to 24 hours in the animals receiving 3 g/kg, independent of the injection velocity. A density peak of 70 HU was found 2 hours after the infusion of 1.5 g/kg. The density peak of the liver, the spleen, and the abscess wall was observed 48 hours after emulsion administration in all groups receiving 3 g/kg. The peak was approximately 150 HU for the liver, 400 HU for the spleen, and 150 HU for the abscess wall. In animals receiving only 1.5 g/kg perflubron, the peak density of the abscess wall was 132 HU after 12 hours, approximately 80 HU for the liver between 2 and 48 hours, and approximately 280 HU after 48 hours for the spleen. CONCLUSIONS: PFOB emulsion produces the highest vascular enhancement within the first 2 hours after the bolus injection of 3 g/kg. For spleen and abscess wall imaging, even the relatively low dose of 1.5 g/kg produced a satisfactory enhancement level for a significant length of time, whereas liver enhancement was best after administration of the higher dose.

[Computed tomographic studies on density kinetics after the bolus injection and infusion of perfluorooctylbromide (PFOB). Animal experimental studies]. / Computertomographische Untersuchungen zur Dichtekinetik nach Bolusinjektion und Infusion von Perfluorooctylbromid (PFOB). Tierexperimentelle Untersuchungen.

We investigated the enhancement of the liver, the spleen, and of induced abscesses and the abdominal vessels after administration of 3 g/kg bodyweight. Perfluorooctylbromide (PFOB) in an animal model. Twenty-one rabbits each received the contrast medium as bolus injection and as slow infusion over half an hour. CT was performed between 2 and 48 hours after contrast medium application. Peak enhancement of the liver, the spleen and the liver abscess membrane was found between 24 and 48 hours after PFOB administration, independently of the application mode. Peak enhancement of the abdominal aorta and the IVC was observed within two hours after bolus injection. In this rabbit model PFOB permits best delineation of the vessels after bolus injection within the first two hours, while CT imaging of the liver, the spleen and the liver abscess membrane is best between 24 and 48 hours after contrast medium application, independent of the injection velocity.

A clinical program of platelet cryopreservation.

A program of platelet cryopreservation has been developed at the Baltimore Cancer Research Center which now provides substantial proportion of the platelet transfusion requirements of alloimmunized patients. The program has gradually grown in size during the last eight years and in 1979 approximately 1600 units of frozen platelets were transfused including 225 transfusions of autologous platelets administered to 45 patients with leukemia. For many alloimmunized patients autologous frozen platelets represented the only source of histocompatible platelets. 5% dimethylsulfoxide is used as the cryoprotective agent and the platelets can be maintained in the frozen state for at least three years without significant reduction in effectiveness. There are essentially no side effects following transfusion and patient acceptance has been excellent. Post-transfusion increments have been highly consistent during the last five years, averaging two thirds of the recovery obtained with fresh platelets with accompanying shortening of the bleeding time. The technology is simple, cost effective and reproducible and is suitable for use in more general blood bank settings.

Frozen autologous platelet transfusion for patients with leukemia.

Platelets were removed from 25 patients with leukemia during remission and were frozen for subsequent transfusion. With 5 per cent dimethyl sulfoxide as a cryoprotective agent, we froze 3 to 5 units of pooled platelet concentrate by simply placing the platelets in the vapor phase of a liquid nitrogen freezer. Ninety-one transfusions of platelets stored for 13 to 400 days were administered. The mean freeze-thaw loss was 13 percent, and the corrected one-hour increment in platelet count was 13,700 per microliter, corresponding to a recovery of 53 per cent of the predicted value. In many patients most or all of the transfusion requirements were met with frozen platelets. Our results indicate that frozen platelets can circulate and function hemostatically. Autologous frozen platelets are of particular value in the management of alloimmunized patients and have become an integral part of our transfusion support program.

Failure of postfiltration leukapheresis plasma to increase granulocyte yields.

Autologous plasma collected either during or immediately after continuous flow filtration leukapheresis (CFFL) was infused prior to subsequent donations in an attempt to induce granulocytosis and to augment granulocyte yields. Granulocytosis did not occur in the 15 to 20 minutes following plasma administration and no increase in granulocyte yields could be demonstrated. In addition, the plasma infusion did not affect the timing or magnitude of the transient granulocytopenia which characteristically occurs during CFFL. Although infusion of postfiltration leukapheresis plasma does not appear to feasible or practical means of enhancing granulocyte collection, it may still serve as a valuable tool in the study of granulocyte kinetics.

Frozen autologous platelets in the supportive care of patients with leukemia.

Multiple units of platelet concentrate obtained by intensive plateletpheresis of patients with leukemia in remission were pooled and frozen using 4 to 5 per cent dimethylsulfoxide and retransfused during periods of thrombocytopenia. Plateletpheresis was well tolerated by all donors and an average platelet yield per unit of 0.99 X 10(11) (n = 155) was obtained. The results of 107 transfusions to 36 patients are presented. An average of 32.4 per cent of the platelets were lost during the freeze-thaw process. Freezing loss was lowest at a freezing rate of one degree C per minute, at a lower final concentration of platelets, and when polyolefin bags were used. The mean corrected posttransfusion count increment was 6,400/mul (range 600-19,000 xm2/10(11) platelets transfused). In vivo results did not correlate with freezing rate but were statistically significantly better at lower platelet (approximately 0.16 X 10(11) platelets/10 ml) concentrations. Eleven patients, including some who were refractory to random donor platelets were supported entirely with autologous platelets during reinduction therapy for leukemia. When administered prophylactically the autologous platelets seemed to prevent hemorrhage during periods of thromobocytopenia although in most patients bleeding times were not corrected posttransfusion. This study demonstrates that frozen autologous platelets can be used in the supportive care of thrombocytopenic patients. Further technical improvements are necessary before platelet freezing becomes practical for widespread use.