Symptomatic epilepsy associated with intracranial calcifications in children with acute lymphoblastic leukemia (ALL).

Acute and long-term sequels of central nervous system (CNS) prophylaxis with irradiation and intrathecal chemotherapy in children suffering from acute lymphoblastic leukemia (ALL) include vasculopathies, leucoencephalopathies, intracranial calcifications, intellectual and neurological impairment. We report two children at the age 5 and 8 years who manifested partial motor or complex seizures and intracranial calcifications 2-4 years after the diagnosis of ALL had been established. The occurrence of these disorders was much earlier than reported in the literature. Both children received prophylactic CNS treatment with irradiation and intrathecal methotrexate (MTX). Their brain CT scans and EEG had been normal before the first epileptic seizure was registered. Children are now seizure free on carbamazepine, and a boy with complex partial and myoclonic seizures is also on valproate and vigabatrine. Symptomatic epilepsy associated with intracranial calcifications and persisting EEG changes might occur as side effects of ALL treatment.

[Sideropenic anemia in infants and toddlers]. / Sideropenicna anemija u dojenackoj dobi i dobi malog djeteta.

Iron-deficiency anemia is the most common anemia in infants. In the routine pediatric care this problem is encountered every day. Numerous factors in infancy (low birth weight, rapid growth, insufficient nutrition) are involved in the development of iron-deficiency anemia, and they must be considered when establishing diagnosis, counselling parents, and prescribing oral iron preparations. Data on 119 patients aged up to two years treated in the Division of Gastroenterology and Nutrition, Pediatric Department, University Hospital Centre Zagreb Salata between 1994 and 1999, were analyzed. We were prompted to do so because of great frequency of iron-deficiency anemia as one of associated diagnoses, and unfortunately often the only diagnosis requiring hospitalization. Out of 119 patients with iron-deficiency anemia, nine (7.7%) patients (four premature newborns and three from twin pregnancy) had to receive transfusion of erythrocyte concentrate due to very bad general condition and low red blood count, accompanied by clinical signs of anemic hypoxia. We also analyzed prenatal and perinatal history, socioeconomic living conditions of these children, i.e. their nutrition, and if they had previously received oral iron preparations. Some of the results, such as inadequate alimentation with flour, as well as insufficient prophylaxis of iron deficiency, which were found in most cases of severe anemia, point to the need of paying greater attention to this problem, better parents education, and more adequate screening for iron deficiency anemia.

Jumping translocations involving 11q in a non-Hodgkin lymphoma.

This paper presents the results of a cytogenetic analysis in an 11-year-old boy with non-Hodgkin lymphoma. The investigation was performed on slides obtained from short-term culture of lymph node cells. The analyses revealed an abnormal clone with loss of Y, gain of an X chromosome, t(3;22), trisomy 11, and three cytogenetically-related subclones with jumping translocations involving 11q13 as the common breakpoint region. This region is an unusual site of chromosome breakage in jumping translocations, and has not been reported thus far. Contrary to most published reports, the jumping translocation in our patient is associated with long survival.

[Case report of a female patient with autoimmune hemolytic anemia and cold agglutinin antibodies]. / Prikaz bolesnice s autoimunom hemolitickom anemijom s hladnim protutijelima.

We report a case of acute transient cold agglutinin disease, the etiology of which we could not determine with the available methods. Cold autoagglutinins had anti I specificity, high titers of the autoantibody (> 1:1,000) and the thermal range was relative wide. Our patient had severe haemolysis and immunosuppressive therapy with methylprednisolone and cyclophosphamide was administered. It is a question how much these immunosuppresive agents influenced the recovery, and in what extent it was a self limited disease with spontaneous recovery.

Chromosomal analysis of two neuroblastomas.

We report the results of cytogenetic analysis in two children with neuroblastoma. The analysis was performed on slides obtained from a 24-hour bone marrow (BM) culture or by a direct method of the primary tumor tissue. The structural and numeric karyotype aberrations were established, including structural aberrations of chromosome 1. The finding of i(1q) and t(1;5)(p22;q13) is of interest because these chromosome aberrations are rare in this type of disease.

Cytogenetic analysis in children with acute nonlymphocytic leukemia.

In this work we present the results of cytogenetic analysis of the malignant cells in 27 children with acute nonlymphocytic leukemia (ANLL). The aim of our investigations was to determine the frequency and types of chromosome aberrations in our population of children with ANLL. Successful cytogenetic analysis was carried out in 24 (89%) patients. Aberrant karyotypes of malignant cells were established in 58% of the cases. The most frequent chromosomal abnormality was t(8;21), identified in 5 (20.8%) patients, i.e., 4 of 10 M2-ANLL. Aberration frequency of chromosome 11 was 16.6% and was identified in 3 of 8 M5-ANLL. Trisomy 8 and monosomy 7 were identified in one patient each with M3 and M2-ANLL, respectively. del(13), a rare chromosome aberration in hemoblastoses, was found in a child with M1,t(8;21) and the loss of chromosome Y. Translocation t(1;11;21) with a break in regions 1q23, 11q23, and 21q22, is unusual and was identified in a boy with M2-ANLL; it can be considered as a variant form of the t(8;21).

[Results of treatment in non-Hodgkin's lymphoma in children using the YU-77, YU-84 and YU-87 protocols]. / Rezultati lijecenja ne-Hodgkinova limfoma u djece protokolima YU-77, YU-84 i YU-87.

Fifty-eight children with non-Hodgkin's lymphomas (NHL) were treated with three different chemotherapeutic protocols at the Division of Hematology and Oncology, Department of Pediatrics Salata during the period from 1977-1989. Twelve (20%) patients had lymphocytic and 46 (80%) lymphoblastic type of NHL. There was no statistical difference in the incidence of the 1st complete remission induced by different chemotherapeutic protocols (YU-77 86% YU-84 72% and YU-87 81%). However, the 1st relapse occurred in 8% of the patients treated with YU-87 protocol, compared to 30% in those treated with YU-84 and 58% treated with YU-77 (p < 0.01). The probability of 36-month survival was highest in patients treated with the most aggressive chemotherapeutic protocol YU-87 (81%), whereas it was 63% in those treated with YU-84 and 32% treated with YU-77 (p < 0.01). All patients that did not enter the 1st complete remission died during the first year of treatment. The median survival of patients that achieved the 1st complete remission was 28.3 months, while the probability of 36-month survival was 62% (p < 0.001). The survival strongly correlated with the protocol employed and clinical stage, but was independent of the histologic type of NHL.

Heterochromatic variability in children with acute lymphoblastic leukemia.

An analysis of the C-segment variability of chromosomes 1, 9, and 16 was carried out in 38 children with ALL, and 90 control subjects. When studying location variants, no differences were found between group of patients and the normal controls. A larger quantity of structural heterochromatin was, however, observed on chromosomes 1, 9, and 16, and a higher frequency of homologous chromosomes heteromorphism in children with ALL when compared with the control group.

Premature chromosome condensation in children with acute lymphocytic leukemia (L1) and malignant histiocytosis.

In this study we report the observation of premature chromosome condensation (PCC) in two children with acute lymphocytic leukemia L1 and one child with malignant histiocytosis. Cytogenetic analysis was performed on peripheral blood or bone marrow cells cultivated for 24 hours without mitogen. In all three reported cases the modal karyotype was normal, while 12.9%, 5.5%, and 5% of spreads with PCC was observed, respectively.

Subsets of childhood acute lymphoblastic leukaemia in Croatia.

As a contribution to epidemiological studies on distribution of acute lymphoblastic leukaemia (ALL) subsets in different countries, we investigated blast cell immunophenotype in 54 children with ALL from the western part of Yugoslavia. The subtype incidences were: common, 75.9%; null, 7.4%; T, 11.1%; B, 1.9%; and unclassifiable, 3.7%. This resembles the ALL pattern registered in developed countries. Hence, differences in socioeconomic status between our population and developed European countries do not result in an appreciably altered incidence of childhood leukaemia subtypes.

Cytogenetic analysis of a child with malignant histiocytosis.

This report presents the results of cytogenetic analysis of a child with malignant histiocytosis. The analysis was carried out on slides obtained following short-term cultures of peripheral blood cells. The malignant cells had a deletion of the long arm of chromosome #7, 46,XX,del(7)(q22).

Heterochromatic segment length of Y chromosome in 55 boys with malignant diseases.

By investigating heterochromatic segment variability in a group of boys with malignant disease a significantly greater value of the Yc:F index in relation to the control subjects was established.

Marker chromosome 1q+ in acute lymphocytic leukemia.

This paper presents the results of a cytogenetic analysis on a patient with acute lymphocytic leukemia (ALL) type L2 according to the FAB classification. Of the metaphases examined, 69.3% belong to the aberrant clone of pseudodiploid karyotype. Marker chromosome 14q+ has been identified in all the cells of the clone. Duplication was found in 30% of the metaphases, and in 15% triplication of the proximal segment of the long arm of chromosome #1 (q11-q21). In one metaphase the long arm of chromosome #1 is made up of segment q11-q21 four times repeated. Aberrations of chromosome #1 support the idea that heterochromatic region may be related to the higher degree of the cell malignity.

Variability of chromosomes 1, 9, and 16 in children with malignant diseases.

Heterochromatic segments of chromosomes #1, #9, and #16 were analyzed in 38 children with malignant disease and 42 healthy persons. The analysis was carried out on C-banded metaphases obtained by peripheral blood culture. Using a quantitative method of analysis, an association was established between C-segment length of chromosome #9 and malignant disease in children. A disturbed quantitative relation of C-heterochromatin of chromosomes #1, #9, and #16 was also found in the group of children with malignant disease.

[Treatment of bone marrow aplasia in phase I of acute lymphatic leukemia treatment in children]. / Pokusaji suzbijanja i lijecenja aplazije kostane srzi u I fazi lijecenja akutne limfaticne leukemije u djece.

In the first phase of treatment of acute lymphatic leukemia in children (ALL) with aggresive cytostatic protocols, the doctor is, in some patients, forced to modify the antitumor therapy over a certain period of time because of bone marrow depression. The authors attempted to pull patients with ALL through this critical phase of the disease - by administering "profilactically" Lithium Carbonate (Li2CO3) ( in order to stimulate granulopoiesis) or, if anaemia, leukopenia and thrombocytopenia had already occurred, by administering concentrates of erythrocytes, leukocytes and platelets - without discontinuing the administration of cytostatics. The results of these attempts are reported.

[Congenital hemolytic anemia]. / Prirodene hemoliticke anemije.

The cases of 91 patients with congenital hemolytic anemia (H.a.) are reported. The causes of congenital H.a. and the laboratory test by which the diagnosis and precise subclassification of the disease were facilitated are indicated. Emphasis is placed on the therapeutic significance of splenectomy in those patients In whom laboratory tests with a radioactive marker [Cr 51]indicated that splenectomy could have a beneficial effect.