Radiological case of the month. Pica.
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Child, Preschool↗
Biomedical subjects
R Zaizov
Publications and source records attributed to R Zaizov.
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↗
Recurrent peripheral arterial occlusion by leukemic cells sedimentation in acute promyelocytic leukemia.
A 10-year-old boy presenting with recurrent successive events of peripheral arterial occlusion in both legs, more prominent on the left side, was diagnosed as having acute promyelocytic leukemia. Histological examination of the occluding material showed that it was composed of leukemic blast cells. The boy underwent five operations. Each time the arterial flow was reestablished by removing cylindrical hollow white material obstructing the lumen. However, after the last operation, irreversible ischemia developed, necessitating an above-knee amputation. There was no evidence of disseminated intravascular coagulation. Three months later a right lumbar sympathectomy was performed for relief of causalgic-ischemic pains, with successful results. Preoperative angiography demonstrated irregular narrowing of the right iliac arteries and tibioperoneal trunk stenosis.
Amputation, Surgical↗
Long-term follow-up of partial splenectomy in Gaucher's disease.
Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.
Adolescent↗
Thymic humoral factor-gamma 2, an immunoregulatory peptide, enhances human hematopoietic progenitor cell growth.
Thymus humoral factor-gamma 2 (THF gamma 2), an octapeptide important for T-lymphocyte regulation, was assessed for its effect on the in vitro growth of human hematopoietic progenitor cells. This was achieved using a recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF)-stimulated myeloid cell colony formation (granulocyte-macrophage colony-forming cells, GM-CFC) assay as well as a recombinant erythropoietin (rEpo)-stimulated erythroid burst formation (erythroid burst-forming units, BFU-E) assay. Cells were obtained from bone marrow (BM) and peripheral blood (PB) of normal healthy donors and from patients with suppressed bone marrows. The latter group included aplastic anemia, leukemia, and lymphoma patients and patients with solid tumors who responded to intensive chemotherapy with significant pancytopenia. THF gamma 2 significantly enhanced normal BM and PB GM-CFC and PB BFU-E by 2- to 2.5-fold. This effect was totally dependent on the presence of the respective growth factors, that is, rGM-CSF or rEpo, and was specifically reversed by an anti-THF gamma 2 antiserum. Furthermore, although THF gamma 2-induced enhancement of GM-CFC colony formation was not affected by lymphocyte or monocyte depletion, the augmenting effect of the peptide on BFU-E was completely abrogated in the absence of lymphocytes. THF gamma 2-induced augmented growth of progenitor cells derived from severely suppressed marrows was minimal. However, cells from moderately neutropenic patients with leukemia in remission or with lymphoma under chemotherapy responded to the peptide similarly to cells from normal donors. These results suggest a stimulatory role for THF gamma 2 on human myeloid and erythroid hematopoietic progenitor cells. They also suggest the lymphocyte dependence of BFU-E enhancement and lymphocyte independence of GM-CFC stimulation by THF gamma 2. In the former case the thymus-derived peptide may act through the induction of certain erythroid-enhancing lymphokines.
Adjuvants, Immunologic↗
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↗
The prognostic significance of glial fibrillary acidic protein staining in medulloblastoma.
Histologic and immunohistochemical properties of 53 medulloblastomas were analyzed with regard to clinical features and survival rate. No correlation was found between survival rate and histologic features of the tumor, such as desmoplastic reaction, number of mitoses, hemorrhages, necrosis, endothelial proliferation, glomerular arrangement, calcifications, rosettes, or oligodendroglial cells. However 82% of the patients with positive glial fibrillary acidic protein (GFAP) staining in numerous tumor cells survived more than 5 years; only 30% survived that long if their tumor cells were GFAP negative (P = 0.0093). This significant difference was not related to the mode of therapeutic protocol used. The authors suggest that GFAP staining may be a useful prognostic tool in medulloblastoma.
Adolescent↗
Hepatic iron overload: quantitative MR imaging.
Iron deposits demonstrate characteristically shortened T2 relaxation times. Several previously published studies reported poor correlation between the in vivo hepatic 1/T2 measurements made by means of midfield magnetic resonance (MR) units and the hepatic iron content of iron-overloaded patients. In this study, the authors assessed the use of in vivo 1/T2 measurements obtained by means of MR imaging at 0.5 T using short echo times (13.4 and 30 msec) and single-echo-sequences as well as computed tomographic (CT) attenuation as a measure of liver iron concentration in 10 severely iron-overloaded patients with beta-thalassemia major. The iron concentrations in surgical wedge biopsy samples of the liver, which varied between 3 and 9 mg/g of wet weight (normal, less than or equal to 0.5 mg/g), correlated well (r = .93, P less than or equal to .0001) with the preoperative in vivo hepatic 1/T2 measurements. The CT attenuation did not correlate with liver iron concentration. Quantitative MR imaging is a readily available noninvasive method for the assessment of hepatic iron concentration in iron-overloaded patients, reducing the need for needle biopsies of the liver.
Adolescent↗
Human growth hormone and insulin-like growth factor-1 enhance the proliferation of human leukemic blasts.
As the number of long-term survivors of childhood leukemia increases, growth retardation has emerged as a significant complication. Treatment of these children with growth hormone (GH) has been suggested and sporadically implemented. We, therefore, studied the effect of human GH (hGH) and its by-product insulin-like growth factor-1 (IGF-1) on the growth of leukemic cells in vitro. Under serum-free conditions hGH and IGF-1 induced a significant dose-dependent proliferative effect on promyelocytic leukemia (HL60) and Burkitt's lymphoma (Daudi) cell lines. Anti-hGH antibodies negated the stimulatory effect of hGH and anti-IGF-1 serum abrogated the growth-promoting effect enhanced by IGF-1. Similar statistically significant stimulatory properties were found when freshly obtained marrow cells from four of five acute lymphoblastic leukemia (ALL) of childhood and four acute myelogenous leukemia (AML) patients were studied in ALL and AML blast-cell clonogenic assays. ALL colonies increased numerically by 72% (P less than .025) and AML colonies by 92% (P less than .01) in the presence of hGH at concentrations of 2.5 x 10(2) and 3.0 x 10(2) ng/mL, respectively. IGF-1 stimulated ALL and AML blast-colony growth at concentrations ranging from 0.05 to 0.5 ng/mL by up to 105% (P less than .025) and 65% (P less than .03), respectively. Our in vitro data suggest that circulating hGH and IGF-1 may promote leukemic blast cell replication in vivo, and the supplemental administration of hGH to leukemia patients in remission must be carefully monitored for early relapse.
Bone Marrow↗
Bone scans in the diagnosis of bone crisis in patients who have Gaucher disease.
Of thirty-four occurrences of bone pain in seventeen children and young adults who had type-I Gaucher disease, twenty-five were finally diagnosed as bone crises. On the basis of a bone scan with technetium-99m methylene diphosphonate, a correct diagnosis of bone crisis was made for twenty-three occurrences, with a sensitivity of 0.92. At the onset of a crisis, the bone scan typically showed decreased uptake of radionuclide at the involved site. After six weeks, a repeat bone scan of the long bones showed a ring of increased uptake surrounding an area of decreased uptake. At six months, the appearance of the bones on the radionuclide scan was normal.
Adolescent↗
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↗
Neonatal ABO incompatibility. Complicated by hemoglobinuria and acute renal failure.
The authors present two infants with isoimmune hemolytic disease due to ABO incompatibility complicated by massive hemoglobinuria and secondary acute renal failure. This represents an incidence of 0.36% of all neonates with ABO hemolytic disease in the author's newborn population. Only two patients have been reported previously to have similar complications. Analysis of data of these four infants revealed the clinical characteristics of this complication of ABO incompatibility: 1) very low frequency; 2) early onset of hemoglobinuria (first voided urine) and of acute renal failure (first 2 days of life); 3) lack of correlation between the clinical presentation of hemolytic disease and appearance and severity of renal failure; 4) complete recovery of renal functions following intravenous fluid administration; and 5) normal renal radiologic investigations.
ABO Blood-Group System↗
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↗
[Immune neutropenias of infancy and childhood--update on diagnosis and treatment].
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Autoimmune Diseases↗
[Transfusion-associated graft-versus-host disease and its prevention].
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Graft vs Host Disease↗
Premature separation of placenta in a patient with idiopathic thrombocytopenic purpura.
The most common autoimmune hemolytic disorder encountered during pregnancy is idiopathic thrombocytopenic purpura. Premature separation of the placenta is one of the most serious complications in the second half of pregnancy. Since the association of the two is not known, the aim of the present paper is to present such a case and to draw the attention of the obstetrician to this possibility.
Abruptio Placentae↗
Long-term proliferation of human leukemia cells induced by mouse stroma.
Acute lymphocytic leukemias (ALL) of infants and children were found to preferentially survive in coculture with a cloned cell line of endothelial adipose cells (14F1.1) from mouse bone marrow. One of these ALLs expressed a phenotype compatible with an early stage of differentiation (HLA-DR+, CD19+, and CD34+) and exhibited extensive growth in the presence of the mouse stromal cells during a period greater than 25 weeks following seeding. These ALL cells were strictly dependent upon the mouse stromal clone 14F1.1 and failed to proliferate in the absence of the endothelial adipocytes or with a variety of "feeder cells." Throughout the culture period the cells died if removed from the stroma. No similarly proliferative cell population with strict dependence upon stromal cells was found among a variety of other leukemias including hairy cell, acute myeloid, and chronic lymphocytic leukemia. The 14F1.1 clone has been previously found to promote the renewal of mouse and human stem cells. It is therefore possible that leukemias with a stem cell-like phenotype depend upon stromal cell factors similar to those affecting the growth of normal stem cells. These factors appear to operate across genetic barriers.
Antibodies, Monoclonal↗
Inhibition by retinoic acid of myeloid progenitors in chronic myeloid leukemia and myeloproliferative disease: increased sensitivity in blastic phase of chronic myeloid leukemia.
The effects of all-trans retinoic acid (RA) were tested on the growth in vitro of myeloid progenitors from peripheral blood or bone marrow, in 25 patients with chronic myeloid leukemia (CML), ten of whom were either in accelerated or blastic phase, and in nine patients with myeloproliferative disease (MPD). The responses were compared with 12 normal bone marrow controls obtained from patients with lymphoma. Clonal growth in CML blastic and accelerated phase was inhibited to the greatest degree (mean 49 +/- 9% (SEM) of control at 0.3 microM RA). The responses in CML chronic phase and MPD were more heterogeneous, but significant inhibition was seen at higher concentrations of RA (50 +/- 12% CML chronic phase, 58 +/- 26% MPD at 3.0 microM RA). At 0.3 microM and 1.0 microM RA there were significant differences between the CML chronic phase and the CML blastic phase patients (p less than 0.02 and p less than 0.05 respectively). At these concentrations there was no significant inhibition on normal bone marrow myeloid progenitors. Inhibition was independent of the proportions of progenitors in S phase, as assessed by tritiated thymidine suicide. Preincubation of cells from selected patients with RA for 48 hours before culture in agar resulted in a significant degree of inhibition (48 +/- 8% of control). Inhibition was prevented by delaying the addition of RA from 24 to 48 hours from the beginning of the culture, indicating that RA exerts an early direct effect on myeloid progenitors.
Blast Crisis↗