Occurrence of acute nonlymphoblastic leukemia in two girls after treatment of recurrent, disseminated Langerhans cell histiocytosis.

The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.

[Results and experiences with a modified BFM protocol for treatment of recurrences in children with acute lymphoblastic leukemia in East German areas]. / Ergebnisse und Erfahrungen mit einem modifizierten BFM-Protokoll zur Rezidivbehandlung von Kindern mit akuten lymphoblastischen Leukämien (ALL) in den ostdeutschen Ländern.

Between 1988 and 1990, 55 patients with first relapses of acute lymphoblastic leukemia (ALL) were treated with a modified BFM-protocol (ALL REZ I/88). The patients were divided according to time and site of relapse: relapses with bone marrow involvement up to 6 months after stopping front line therapy (group A), relapses with bone marrow involvement beyond 6 month after therapy (group B) and isolated extramedullary relapses at any time (group C). During therapy the patients received alternating courses of polychemotherapy including infusions of intermediate dose methotrexate (1 g/m2 in 36 hours). The maintenance treatment consisted of daily oral thioguanine and biweekly intravenous (IV) MTX. The overall second remission rate was 89% (group A: 90%, group B: 86%, group C: 93%) and the probability of event free survival (EFS) at 4 years is 0.28 +/- 0.13 (group A: 0.22 +/- 0.12, group B: 0.24 +/- 0.18, group C: 0.57 +/- 0.15). We conclude, that with the treatment regimen applied, long lasting second remission can be achieved in about one third of patients even after intensive front line therapy. The most unfavourable prognoses were seen in patients with early bone marrow relapses (group A).

[Experiences with modified BFM protocols in the treatment of children with acute lymphoblastic leukemia (ALL) in East Germany 1981-1991]. / Erfahrungen mit modifizierten BFM-Protokollen bei der Behandlung von Kindern mit akuten lymphoblastischen Leukämien (ALL) in den ostdeutschen Ländern von 1981 bis 1991.

Between September and August 1991 818 previously untreated children and adolescents up to 18 years of age with acute lymphoblastic leukemia were entered into two modified BFM-protocols. Patients with B-ALL were excluded. From 1981 to 1987 524 patients were entered into the randomized multicenter study ALL VII/81 (modified ALL-BFM 81 protocol). Patients were divided into three risk groups standard (SR), medium (MR), high risk (HR) using the BFM risk factor. In a connecting study from 1988 to 1991 294 patients were registered on the stratified and randomized multicentric trial ALL VIII/87 (modified ALL-BFM 86 study). The main modification in study ALL VII/81 concerned the duration of treatment. Patients were randomized into two groups. The first group received as a late reinduction protocol III and then therapy was stopped. The second group received 6-MP and MTX for another six months. The other whole treatment strategy of ALL-BFM 81 was adopted. In protocol ALL VIII/87 the only modification was the reduction of the MTX dosage from 5 g/m2 to 1 g/m2 with an infusion time of 24 hours (leucovorin rescue 15 mg/m2 after 48 and 54 hours). The probability of the event-free-survival (EFS) for study ALL VII/81 was 59%. CNS events were significantly more frequent in standard risk patients with intermediate dose MTX (4 x 0.5 g/m2) compared with the irradiation group (18 Gy). The EFS for SR patients amounts to 61%, for MR patients to 59% and for HR patients to 36%. There was no significant difference of EFS for the two groups with different duration of therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

[Capillary resistance in premature and newborn infants and its relation to hemostatic potential]. / Die Kapillarresistenz bei Früh- und Neugeborenen und ihre Beziehung zum Hämostasepotential.

Capillary resistance, platelet count, platelet adhesiveness and aggregation and thrombelastogramm (TEG) were studied in 40 healthy newborn infants (term eutrophic, preterm eutrophic and hypotropic). The capillary resistance was normal in all cases. The physiologic decrease of platelet aggregation in newborns and the prolonged clotting time in TEG (hypotrophic infants) were without influence on the capillary resistance. An important factor of the normal capillary resistance in newborns is normal platelet adhesiveness.

Improved treatment results in childhood acute nonlymphoblastic leukemia with the BFM-AML protocol 78 in a multicenter study in the GDR.

Eighty-seven children with acute nonlymphoblastic leukemia were treated with the AML protocol BFM 78 between June 1979 and February 1986 in a multicenter study in the GDR. Seventeen children (20%) died from early complications, eight did not respond to therapy. Fifty-eight patients (70%) achieved a complete remission. Twenty-three patients relapsed. The life table analysis revealed after 5 years a probability for event-free survival of 36% (SD = 6%) and an event-free interval of 51% (SD = 8%). Six patients were transplanted in first remission. Two of them died; one (M 1) on day + 19 from encephalopathy and one (M 4) on day + 60 from acute GVHD. The overall results are in good correlation with the original BFM study, but there are differences in the subtypes. Results are superior to other AML protocols in our group.

[Drug effect on the rotation thromboelastogram in newborn infants]. / Die medikamentöse Beeinflussung des Rotationsthrombelastogramms bei Neugeborenen.

The impact of the antibiotics carbenicilin and gentamycin on the aggregation and adhesiveness of thrombocytes and on the rotation thrombelastogramme is investigated in healthy newborns. Thrombocyte aggregation was markedly inhibited by carbenicilline; gentamycine had no influence. Thrombocyte adhesiveness was less influenced by both medicaments. The parameters of TEG, viz. reaction time, clotforming time, thrombus elasticity and index were compared before and after rotation and with and without medicaments. Under carbenicilline a prolongation of the clotforming time and a decrease of thrombus elasticity could be shown to exist after rotation. The impact of medicaments on the rotation thrombelastogramme can only be conceived by comparing the values before and after rotation in each case with and without medicament and not simply evaluating it, so that the rotation thrombelastogramms cannot be recommended to be used for assessing the disturbance of thrombocyte function induced by medicaments.