[What is your diagnosis? Multifocal osteitis due to BCG].
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BCG Vaccine↗
Biomedical subjects
B Nelken
Publications and source records attributed to B Nelken.
Prognostic significance of the balanced t(1;19) and unbalanced der(19)t(1;19) translocations in acute lymphoblastic leukemia.
The recurring chromosomal 1;19 translocation in acute lymphoblastic leukemia (ALL) occurs in balanced t(1;19) (q23;p13) and unbalanced, -19, +der(19)t(1;19)(q23;p13) forms. The clinical features and outcome were compared for 30 patients with the t(1;19) and 36 patients with the der(19) forms. These were 45 children (less than 1-14 years) and 21 adults (15-54 years) (median age 9.0 years), 41 females, 25 males, with median white blood count (WBC) 20.9 x 10(9)/1. Patients were classified by karyotype thus: t(1;19) 11 cases; t(1;19) with additional change (+A) 19 cases; der(19) 17 cases; and der(19) +A, 19 cases. Non-random additional structural abnormalities included involvement of 1q, 6q, i(7q), i(9q), 9p, and 13q. The only significant difference in clinical or blast cell features between patients with the t(1;19) and the der(19) was the greater age of adults with t(1;19) (p less than 0.05). Projected median event-free survival and survival of all cases together was 22 months and greater than 112 months respectively. Neither age nor WBC contributed significantly to prognosis. For patients at all ages, prognosis of der(19) was better than t(1;19). This was statistically significant for event-free and overall survival in childhood (p = 0.02 and p = 0.01 respectively) and was independent of age (p = 0.04 and p = 0.008 respectively) and WBC (p = 0.03 and p = 0.04 respectively). Future studies should examine separately the outcome for patients with the balanced and unbalanced forms of the t(1;19).
Adolescent↗
[Fatal cerebral and pulmonary aspergillosis in acute leukemia in a child].
Immediately after induction therapy for acute lymphoblastic leukemia, a 2 1/2-year-old child developed invasive pulmonary aspergillosis revealed by pneumothorax, an unusual manifestation. Despite treatment with amphotericin B, status epilepticus occurred; this manifestation was related to diffuse ischemic cerebral lesions probably caused by cerebral aspergillosis. Outcome was fatal. Early invasive pulmonary aspergillosis is responsible for non-specific pneumonia. Thoracic CT scan and fiberoptic bronchoscopy are informative investigations. At recovery of bone marrow aplasia, the occurrence of hemoptysis and the discovery of excavated lesions on roentgenograms are suggestive of the diagnosis. Cerebral aspergillosis should be routinely considered whenever neurologic symptoms develop in a patient with agranulocytosis, fever, and pneumonia. The prognosis of invasive aspergillosis depends above all on the promptness of treatment; amphotericin B should be given intravenously whenever broad spectrum antimicrobial therapy fails to induce apyrexia in a patient with agranulocytosis.
Amphotericin B↗
[Oncologic complications and cytogenetic features of ataxia telangiectasia].
A very high incidence of cancers (10%) is recorded in patients homozygous for ataxia telangiectasia (AT). From 1970 to 1987, 35 children were investigated in the department of pediatric neurology in Lille. Three developed a malignancy (one hepatic tumor, one Hodgkin's disease and one non Hodgkin's lymphoma). Constitutional chromosome fragility and immune deficiency are the main features of AT. The first one is probably linked to the pathogenesis of malignancies. Moreover, cancer therapy has to take these features into account.
Ataxia Telangiectasia↗
[Precocious pseudo-puberty in granulosa cell tumor in children less than 1 year old. 2 cases].
Two cases of isosexual precocity in relation to granulosa cell tumor in 2 girls aged 6 and 9 months respectively are described. In the two cases clinical and biological signs of hyperoestrogenemia were found but in one, acute signs of tumoral rupture were the first symptom. Histologically, the two classical subtypes were found: macrofollicular ("adult" type) and "juvenile". These two children are alive without any sign of recurrence with a follow up of 12 and 18 months. The occurrence of granulosa cell tumors in such young girls seems extremely rare.
Estradiol↗
A new case of dic(9;12)(p13;p11) in acute lymphocytic leukemia.
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Child↗
Translocation t(1;19)(q23;p13) in acute lymphoblastic leukemia. A report on six new cases and an unusual t(17;19)(q11;q13), with special reference to prognostic factors.
We report clinical, immunologic, and cytogenetic characteristics of six patients with a t(1;19)(q23;p13) that was balanced in one case and of the unbalanced type [-19,der(19)t(1;19)(q23;p13)] in the remaining five cases. Intracytoplasmic immunoglobulins (cIg) were positive in the three cases where they were found. We also report on another patient, with a t(17;19) involving 17q11 and probably 19q13 regions, although involvement of 19p13 could not be excluded. In this patient, cIg were also present, thus raising the issue of whether such a rearrangement could be a variant of t(1;19). Clinically, five patients belonged to the high-risk acute lymphoblastic leukemia (ALL) group, because of high leukocytosis, central nervous system (CNS) disease at presentation, or massive organomegaly. Cytologically, all cases were FAB type L1. Except for the two cases allografted in the first complete remission (CR) all patients relapsed, three of them within 13 months. Two CNS relapses were seen in spite of adequate CNS prophylaxis. ALL with t(1;19) appears to be a poor-risk ALL subgroup and probably requires a reinforcement of therapeutic modalities that might include, when possible, allografting at first CR.
Adolescent↗
Cytogenetic studies in 30 patients with Burkitt's lymphoma or L3 acute lymphoblastic leukemia with special reference to additional chromosome abnormalities.
We performed cytogenetic analysis in 23 consecutive patients with Burkitt's ALL and 7 patients with Burkitt's lymphoma. Only one patient had a normal karyotype. Twenty-seven patients had a (8;14) translocation and 2 a (2;8) translocation. No (8;22) translocation was seen. In 12 patients (41%), the t(8;14) was the only chromosome rearrangement whereas in the 18 remaining cases (59%), the t(8;14) or t(2;8) was associated with other numerical or structural abnormalities. Chromosomes 1, 7 and 6 were rearranged in 10, 8, and 5 patients, respectively, usually in translocations, duplications, deletions (chromosome 6), or isochromosome of the long arm (chromosomes 1 or 7). The incidence of these additional rearrangements is discussed with regard to previously published reports and the chromosome localization of oncogenes.
Burkitt Lymphoma↗
Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis.
A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.
Humans↗
[Bone marrow monosomy 7 in children].
Three new cases of monosomy 7 are described. Two children, before onset of overt leukemia, had a preleukemic state: one with thrombopathy and myelodysplastic syndrome, the other with a moderate splenomegaly and an absolute monocytosis. In these two cases the leukemia was chemoresistant. The last child had a subacute myelomonocytic syndrome (juvenile type of chronic myelogenous leukemia) without high fetal hemoglobin value. She died from cachexia. The poor prognosis of monosomy 7 is underlined and such a chromosome deletion should be searched in myeloproliferative syndrome with monocytosis.
Blood Cell Count↗
[(9;11) (p21;q23) translocation: apropos of 2 new cases seen in acute undifferentiated monoblastic AML5a leukemia].
In a cytogenetic study of 54 patients with acute monoblastic leukaemias (AML5) two had a (9;11) (p21;q23) translocation. In addition to the clone, with t(9;11), both patients had a second clone with t(9;11) and trisomy 8. These two patients were aged 23 and 35 years at diagnosis and were classified as M5a in the FAB formulation. A complete remission was achieved in each case, persisting after 19 and 18 months respectively (autograft for one patient). Whereas chromosome 11 anomalies are involved in 13% of all AML5 cases, t(9;11) is less frequently encountered (3.7% in our experience).
Adult↗
Cytogenetics and their prognostic value in childhood and adult acute lymphoblastic leukemia (ALL) excluding L3.
Cytogenetic analysis was made at diagnosis in 174 cases of ALL (101 children less than 20 years and 73 adults), excluding Burkitt's ALL (L3). In 11 children (11 per cent) insufficient material was obtained. In the remaining 90, 50 (56 per cent) had a normal karyotype, 20 (22 per cent) a hyperdiploid karyotype, five (6 per cent) a hypodiploid karyotype, 12 (13 per cent) had a translocation (including seven cases of t(1;19] and three had a pseudodiploid karyotype without translocation. Ninety-eight per cent of patients reached complete remission (CR). Median actuarial CR duration was not attained, was 50 months, 13 months, and 11 months respectively in patients with hyperdiploid, normal, hypodiploid karyotype and in patients with a translocation, the difference between subgroups being significant. In a Cox model, cytogenetics were the strongest factor predicting CR duration (p = 0.03) followed by leukocytes (p = 0.04), whereas the presence of 'bulky disease' had a borderline value (p = 0.077). Of note was that 9/17 (53 per cent) patients with a hypodiploid karyotype or a translocation had no 'risk factors' before cytogenetic analysis. In adults, cytogenetic analysis was unsuccessful in 15 (20 per cent) of patients. In the remaining 58 cases, 19 (33 per cent) had a normal karyotype, 15 (26 per cent) had a hyperdiploid, one (2 per cent) had a hypodiploid karyotype, 19 (33 per cent) had a translocation (including 12 t(9;22], and four (7 per cent) had a pseudodiploid without translocation. 73 per cent patients reached CR. Median actuarial DFS was 12.5 months. No significant differences in CR rate and CR duration were seen between cytogenetic groups, but median CR duration was slightly longer in patients with a normal karyotype (17 months) and shorter in patients with t(9;22) (8.5 months). Only 3/12 of the latter had major risk factors before cytogenetic analysis. Cytogenetic analysis is important in ALL, especially in patients with otherwise standard risk factors, as it may reveal unexpected translocations or hypodiploidy, which may require a therapeutic reinforcement.
Adolescent↗