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Biomedical subjects

C Kaplinsky

Publications and source records attributed to C Kaplinsky.

At least 37 records · Page 2Linked to original sources
PubMed1994

Involvement of 11p15 and 3q21q26 in therapy-related myeloid leukemia (t-ML) in children. Case reports and review of the literature.

The cytogenetic findings of therapy-related myeloid leukemia (t-ML) in three children are presented. These included one male patient with acute lymphoblastic leukemia (ALL) who underwent bone marrow transplantation and developed therapy-related myeloproliferative disease (t-MPD) in the female-donor hematopoietic cells 2.5 years after receiving radiation and epipodophyllotoxin therapy for ALL testicular relapse. Bone marrow leukemic cell karyotype revealed 46,XX,add (11)(p15) and a normal female karyotype in the peripheral blood lymphocytes. The other two children, one with ALL and one with ganglioneuroblastoma, developed fatal t-MPD and therapy-related acute myeloblastic leukemia (t-AML) preceded by myelodysplastic syndrome (t-MDS), respectively, 5 years after diagnosis, following administration of alkylating agents and irradiation. Monosomy 7 was present in both, and was combined with inv(3)(q21q26) in the second patient. Our review of the cytogenetic findings in 91 previously reported pediatric patients with t-ML suggested that the involvement of 11p15 and 3q21-->23, 3q24-q26 with or without a combination of translocation 11q23 and -7/7q-, respectively, are nonrandom aberrations of t-ML in children. Comparison of the chromosomal changes in t-ML between the pediatric and an adult series revealed some differences which may result from differences in treatment modalities and which, in addition, may indicate a possible role of genetic and/or age-dependent factors in the pathogenesis of therapy-related leukemogenesis in children.

Antineoplastic Agents↗
PubMed1994

Chronic childhood idiopathic thrombocytopenia purpura: long-term follow-up.

An understanding of the natural history of childhood chronic idiopathic thrombocytopenia purpura (ITP) could contribute to a rational therapeutic approach to its treatment, which remains controversial. In our retrospective study of 92 children with ITP, 22 had a chronic course and were followed for 3-14 years (median 8.6 years). Treatment, when indicated, was individualized: 4 patients (18.2%) did not receive any treatment, 14 (63.6%) received steroids only, while 4 (18.2%) were treated with steroids and one of the following: high-dose gamma globulin (4 patients), splenectomy (2 patients) or immunosuppressive therapy (2 patients). During follow-up, 14 patients (63.6%) achieved complete remission, 5 (22.7%) partial remission and only 3 (13.5%) remained severely thrombocytopenic, with minimal bleeding tendency. Eleven patients (50%) responded to the initial prednisone course (1-5 mg/kg/day), but showed a marked decrease in platelet count when steroids were tapered off. In view of the high rates of complete and partial remission and the mild course of the few non-responding patients, it is suggested that with adequate supportive therapy, follow-up problems and fatalities can be kept to a minimum. We believe that aggressive therapy, such as splenectomy, should be reserved for the rare symptomatic and severely thrombocytopenic patient.

Child↗
PubMed1994

Childhood B-cell non-Hodgkin's lymphoma in Israel.

The non-Hodgkin's lymphomas of childhood and adolescence are a heterogeneous group of diseases that constitute 7-10 percent of all pediatric malignancies. Major strides have been made in recent years in understanding the pathogenesis of lymphomas at a molecular level but have not yet led to a modification of clinical management. Between 1973 and 1992, 112 children with B-cell non-Hodgkin's lymphoma were diagnosed. The median age was 5.7 years, with male to female ratio of 3 to 1. Fifty percent presented with abdominal masses, mostly confined to the ileo-ceccal region and to the retroperitoneum. In 39 percent there was head and neck involvement, with 15 percent jaw lesions. Until 1978 patients were treated with the Beilinson Medical Center (BMC) protocol, which yielded only 26 percent disease-free survival for stage IV patients. The introduction of the LMB-86 regimen significantly improved the disease-free survival of patients with advanced stage III and IV disease (84 and 68 percent, respectively). Bone marrow (over 70 percent blasts) and central nervous system involvement remain the major prognostic criteria.

Adolescent↗
PubMed1994

Genetic alterations involving chromosome 1p in children with neuroblastoma.

Neuroblastoma is a common childhood tumor of the sympathetic nervous system, characterized by N-myc amplification and loss of heterozygosity (LOH) for sequences on chromosomes 1p, 11q and 14q. Using restriction fragment length polymorphism analysis, the constitutional and tumor genotypes were compared for LOH on 1p in 25 children with neuroblastoma at diagnosis: 19 in stages III-IV, 5 in stages I-II and one in IVs. The genetic alterations observed on chromosome 1p were frequent in the early and advanced stages. Thirty-three percent of the alterations were found in patients under the age of 1 year, and in one patient with IVs. It is concluded that 1p alterations were identified in children with neuroblastoma in early stages and infants and thus of no prognostic relevance. 1p genetic alterations may be early events in neuroblastoma.

Blotting, Southern↗
PubMed1994

Ambulatory treatment with ceftriaxone in febrile neutropenic children.

We conducted a prospective nonrandomized study of outpatient therapy with ceftriaxone as a single agent in 50 episodes of fever and neutropenia in children treated with various myelosuppressive regimens for different malignancies. All patients underwent clinical and radiological evaluation and blood/urine cultures taken before starting therapy. Patients with dehydration, hypotension, rigor and clinical exit-site infection of indwelling right-sided catheters were excluded. Forty-one patients completed an antibiotic course of 7 days: in 12 patients fever returned to normal on day 2, in 10 patients on day 3, and in 8 patients on day 4. The duration of neutropenia following the initial febrile episode was 3-10 days. In some patients fever returned to normal after 2 days, but neutropenia persisted up to 10 days. Two patients were bacteremic--Escherichia coli in one, and Acinetobacter/Staphylococcus coagulase negative in another; all isolates were sensitive to ceftriaxone. In nine episodes, antimicrobial therapy was modified because of persistent fever > 39 degrees C in five patients, bacteremia in two, enterocolitis in one, breakthrough fever in two, and bronchopneumonia in one. The low incidence of bacterial isolation is probably attributed to the selection of patients with low risk features. Patients and parents complied with and favored outpatient therapy to hospitalization.

Ambulatory Care↗
PubMed1994

Long-term intravenous deferoxamine treatment for noncompliant transfusion-dependent beta-thalassemia patients.

With the introduction of long-term subcutaneous administration of deferoxamine (DFO), there has been a decline in the morbidity and mortality of transfusion-dependent beta-thalassemia patients. However, since the use of subcutaneous DFO is hindered by poor compliance, long-term i.v. DFO therapy has been attempted in order to improve compliance, prevent excessive iron accumulation and extend survival. Thirteen patients (aged 5.4-18.4 years) were started on i.v. home administration of DFO (100 mg/kg per day) via an exteriorized, tunneled right atrial catheter (Hickman type). After a median follow-up of 36 months, the mean ferritin levels had dropped significantly (5,117 +/- 1,737 to 1,816 +/- 1,062 micrograms/l. P = 0.0001). None of the patients developed new endocrine or cardiac diseases due to iron overload. Patients beginning therapy at an early age (< or = 11 years) showed a tendency for improved growth parameters at the end of the treatment period. Two patients developed moderately high frequency sensorineural hearing loss. One patient developed a right atrial thrombus. The line infection rate was low (1.7 episodes per 1,000 patients days). In view of the grave prognosis for iron overloaded patients and the fact that oral chelators are not yet readily available, we recommend this form of therapy for the young, noncompliant beta-thalassemia patient, despite the occasional complications observed.

Adolescent↗
PubMed1993

Transient erythroblastopenia of childhood. Evidence for cell-mediated suppression of erythropoiesis.

PURPOSE: T cell-mediated red cell aplasia in a 4 1/2-year-old child with transient erythroblastopenia of childhood (TEC) is described. PATIENTS AND METHODS: Erythropoiesis was studied by assessing the colony growth of marrow erythroid progenitors at the time of diagnosis and during recovery. RESULTS: The colony-forming unit-erythroid (CFU-E) growth of whole marrow at diagnosis was only 28% that of the control. T-cell depletion of the patient's marrow was followed by a more than fivefold increase in CFU-E growth, as compared with 20% inhibition of CFU-E and 40% inhibition of burst-forming unit-erythroid (BFU-E) growth in control marrow. The number of colony-forming unit-granulocyte-macrophage (CFU-GM) in both control and patient's marrow was not significantly altered by all of these manipulations. During early and late recovery, CFU-E and BFU-E growth improved substantially, and the effect of T-cell depletion diminished. Increased numbers of peripheral T-suppressor lymphocytes, as well as activation of natural killer (NK) cells and high levels of interferon, all consistent with viral infection, were found at presentation. Clinical recovery was associated with normalization of T-suppressor lymphocyte number. CONCLUSIONS: The results suggest that in this child with TEC, a preceding viral infection may have caused activation of suppressor T-cells and interferon secretion leading to cell-mediated suppression of erythropoiesis.

Anemia↗
PubMed1993

Improved results in osteogenic sarcoma 1973-79 vs. 1980-86: analysis of results from a single center.

Analysis of treatment results in osteogenic sarcoma patients with classical limb primary tumors and without metastasis at diagnosis or major protocol violations showed improved prognosis with a minimum follow-up of over 5.5 years when divided by years of treatment for all event-free survivors. Twelve patients treated in 1980-86 had a 5-year disease-free survival of 67% and 9 treated in 1973-79 had a 5-year disease-free survival of 33% (P = 0.0368). The improvement appeared to reflect the increase in the intensity of the chemotherapy utilized. Definitive surgery, amputation or limb salvage did not affect the outcome. With these surgical approaches the disease-free survival was 67% and 60% respectively.

Adolescent↗
PubMed1992

T-cell acute lymphoblastic leukemia following therapy of rhabdomyosarcoma.

Multiple studies have documented an increased risk of secondary malignancies in patients receiving alkylating agents. Secondary leukemia following chemotherapy accounts for about 20% of all secondary neoplasms; most are acute nonlymphocytic. Secondary acute lymphoblastic leukemia has rarely been reported in either adult or childhood cancer. We report the development of acute T-cell lymphoblastic leukemia in a child following successful treatment of a paravertebral embryonal rhabdomyosarcoma (ERS). Southern blot analysis of DNA extracted from the T-cell lymphoblasts, using probes homologous to loci on the short arm of chromosome 11; P-calcitonin, P40.1 and H-ras, did not demonstrate the chromosomal loss of heterozygosity (LOH), a common feature of embryonal rhabdomyosarcoma. The data presented support the assumption that de novo leukemia emerged following treatment of the primary malignancy.

DNA, Neoplasm↗
PubMed1991

Esophageal obstruction 14 years after treatment for Hodgkin's disease.

The incidence of late radiation injury of the esophagus is not precisely determined but, overall, the occurrence of clinically apparent damage is infrequent. The authors report a complete esophageal obstruction in a 21-year-old man, 14 years after chemo-radiation therapy for Hodgkin's lymphoma. Although endoscopy failed to demonstrate a gross morphologic abnormality, an esophagogram detected abnormal peristalsis and stricture, and esophageal manometry coupled with dynamic isotopic study clearly demonstrated a multilevel secondary neuronal damage. Data in the literature suggest that alteration in motility is by far the most frequent radiologic manifestation. Further prospective studies will probably clarify the actual incidence of late esophageal damage after chemo-radiation therapy.

Adult↗
PubMed1991

Life-threatening ventricular tachycardia as the presenting symptom of metastatic cardiac disease.

We present 2 cases in whom repetitive rapid ventricular tachycardia (VT) was the initial manifestation of metastatic cardiac disease. In one patient, repetitive VT appeared during chemotherapy for stage IV paratesticular rhabdomyosarcoma which led to the diagnosis of cardiac metastases. In the other, it led to the diagnosis of malignant pericardial effusion 17 years after successful therapy for a breast carcinoma. In conclusion, in patients with present or past history of malignancy, the appearance of life-threatening VT should raise the suspicion of cardiac metastases.

Adult↗
PubMed1991

Liver candidiasis. The various sonographic patterns in the immunocompromised child.

Liver candidiasis is characterised by small abscesses spread throughout the organ at random. These lesions may be identified sonographically. In the active phase of the disease, hepatomegaly is seen together with the "wheel in wheel" phenomenon, the "wagon wheel" appearance and/or the "bull's eye" lesion. Later on, hypoechoic defects develop. When the echogenic foci are revealed, the healing phase is initiated. These manifestations are demonstrated in 4 pediatric oncology patients. The sonographic patterns as well as its differential diagnosis from primary liver tumors, metastatic infiltrations or other abscess formations are discussed.

Candidiasis↗
PubMed1991

Neuroblastoma: evaluation with contrast enhanced MR imaging.

The use of GD-DTPA was introduced recently for MR imaging of the body. This paper presents our experience with GD-DTPA enhanced MRI in the evaluation of neuroblastoma in children. The characteristics of the tumor-enhancement are described, its contribution to the diagnosis and the follow-up of this disease are discussed.

Child↗
PubMed1990

Importance of phenotypic and molecular characterization for identification of a neuroepithelioma tumor cell line, NUB-20.

A neuroblastic-like cell line (NUB-20) was derived from a case of histopathologically diagnosed metastatic neuroblastoma. The metastatic tumor and nude mouse heterotransplant resembled neuroblastoma by histological criteria, in contrast to the primary tumor, which was differentially classified as Ewing's sarcoma. However, the cell line demonstrated a unique phenotype in culture with respect to morphology, immunohistochemical markers, and sensitivity to a battery of differentiation modulators. These characteristics, together with the presence of a chromosomal translocation (11;22),(q24;q12) and amplification with enhanced expression of the c-myc protooncogene rather than N-myc, established this tumor as neuroepithelioma. Neuroepithelioma is a tumor type distinct from, but related to, neuroblastoma in its development from the neural crest lineage. These results emphasize the growing importance of cytogenetic and molecular markers in the classification and characterization of human tumors.

Bucladesine↗
PubMed1990

Head, neck, and maxillofacial childhood Burkitt's lymphoma: a retrospective analysis of 31 patients.

Thirty-one children with Burkitt's lymphoma of the head, neck, and maxillofacial region diagnosed between 1976 and 1988 were reviewed. The age range was 2 to 17 years (median, 7.2 years), and 77.4% were males. The most common presenting symptoms were detectable masses, floating and/or painful teeth, enlarged cervical lymph nodes, sore throat, and neurologic signs. The predominant primary tumor sites were the jaws and tonsils. All patients were staged by a clinical staging system, 17 of them having stage I-II, and 14 stage III-IV. Levels of lactate dehydrogenase and ferritin were the only significant laboratory parameters correlating with initial staging and disease-free survival. Radiologic features in the jaws were poorly circumscribed destructive lytic lesions with migration and crypt destruction of unerupted teeth buds. Complete disappearance of these findings was noted after successful chemotherapy and clinical regression of the tumor. Eighteen (58.1%) patients attained complete remission with a follow-up of 5 to 100 months. Stage was the most significant variable affecting outcome, with 90.2% disease-free survival of stage I patients, 72.4% of stage II, and 18.2% of stage III-IV. Based on these results, it is concluded that localized (stage I and II) Burkitt's lymphoma is responsive to chemotherapy and thus has a favorable prognosis.

Adolescent↗
PubMed1990

Methotrexate-induced leukoencephalopathy is treatable with high-dose folinic acid: a case report and analysis of the literature.

An episode of leukoencephalopathy is reported in a 13-year-old girl who, after standard radiotherapy for a posterior fossa medulloblastoma, received 8 treatments with a protocol containing a 4-hour infusion of 500 mg/m2 methotrexate and 12 mg intrathecal methotrexate. The leukoencephalopathy, documented clinically and by CT and EEG, cleared after 2350 mg of leucovorin (citrovorum factor, folinic acid) was given in addition to the 135 mg given as part of the therapy. A review of the literature suggests that leukoencephalopathy may be prevented by high doses of leucovorin and can be treated by high doses, if lower doses were used initially. When high dose leucovorin was not used, residual neurological damage is not unusual.

Adolescent↗