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At least 685 records · Page 38Linked to original sources
PubMed1997

The management of spinal metastasis in children.

OBJECTIVE: To seek an optimal treatment plan from the results of treatment for metastatic disease of the spine in children. DESIGN: An 8-year retrospective study of children with metastatic disease of the spine. Imaging studies were reviewed and treatment modalities analysed. SETTING: The divisions of pediatric orthopedics and pediatric neurosurgery at the Children's Hospital of Eastern Ontario, Ottawa. PATIENTS: All children seen between April 1980 and December 1987 who had lesions metastatic to the spine by hematogenous or direct extension. There were 20 children (15 boys, 5 girls) with a mean age at the time of diagnosis of 9.5 years. Follow-up ranged from 2 weeks to 108 months. One child was lost to follow-up. INTERVENTIONS: Eleven children underwent laminectomy and decompression. Of the 14 neurologically compromised children, 5 received chemotherapy and radiotherapy and 9 received chemotherapy, radiotherapy and surgery. MAIN OUTCOME MEASURES: Type of metastatic lesion, vertebrae involved and response to therapy. RESULTS: Vertebrae involved with metastases were as follows: cervical (3), thoracic (5), lumbar (8) and multilevel (2). Meninges were involved in 2 cases. The most common causes of metastatic spinal involvement were neuroblastoma (4 cases) and astrocytoma (6 cases). Pathologic fractures occurred in 4 children and kyphoscoliosis in 4. Spinal cord paresis developed in 14 of the 20 children. Of the 6 children who survived from 48 to 108 months, 5 had tumours of neural origin, 4 being astrocytomas. Children with neuroblastoma or leukemic infiltration had a good initial response to chemotherapy. Five of the 6 surviving children had astrocytomas, and 5 were treated by surgical decompression. CONCLUSIONS: Metastatic disease of the spine in children secondary to astrocytoma should be treated aggressively, but from the experience gained from this study it is impossible to devise a rigid treatment plan for each type of metastatic tumour. The choice of chemotherapy, radiotherapy or surgery depends on the type of tumour, the age of the child and whether or not the spinal cord is compromised.

Adolescent↗
PubMed1997

Long-term results in hairy cell leukemia treated with 2-chlorodeoxyadenosine.

We treated 19 patients with hairy cell leukemia (HCL) with 2-chlorodeoxyadenosine. 15 patients followed up at least 6 months were evaluated. The follow up period varied between 6 months and 37 months (median, 19 months). 8 patients were previously treated. The overall response in 15 evaluable HCL patients was 100%, with 87% complete hematological remissions including three patients with retroperitoneal and mediastinal lymphadenopathy and one patient with leukemic infiltrates of the cornea: 13% of patients achieved partial hematological remission. Soluble interleukin-2 receptor (sIL-2R) considered as a reliable non-invasive marker of HCL tumor burden dropped from the median of 1350 pM/ml (range 188 to 9000 pM/ml) to the median of 84.3 pM/ml (range 37 to 382 pM/ml) RdW which reflects the anisocytosis of red cells decreased after therapy from the median of 20.6% (range 13.1-25.0%) to the median of 13.7% (range 12.4-16.3%).

Adult↗
PubMed1997

Human T-cell lymphotropic virus-1-positive T-cell leukemia/lymphoma in a child. Report of a case and review of the literature.

Adult T-cell leukemia/lymphoma is a monoclonal T-cell neoplasm associated with human T-cell lymphotropic virus-1 (HTLV-1) that occurs almost exclusively in adults. This report concerns a Romanian girl who had recurrent skin eruptions since infancy, subcutaneous tumors in childhood, and peripheral blood lymphocytosis, which initially developed at the age of 12 years. The circulating lymphocytes were of helper T-cell immunophenotype. Serologic studies demonstrated a number of HTLV-1 antigens in the child and her mother, and molecular analyses revealed monoclonal T-cell-receptor gamma gene rearrangement and detectable HTLV-1 proviral DNA. Conventional cytogenetic studies revealed a t(3;6)(q23;q27) chromosome translocation in most of the neoplastic cells. The patient initially responded well to interferon alfa therapy and showed regression of skin lesions and diminished lymphocytosis, but 4 years later, she developed massive lymphadenopathy and leukemic infiltration of the breast. At last clinical follow-up, at the age of 17 years, the patient had stable low-level peripheral lymphocytosis and subcutaneous tumors while being continuously treated with interferon alfa. Our review of the literature revealed six additional children with HTLV-1-associated T-cell leukemia/lymphoma, including one case with a similar clinical presentation and ethnic background. To our knowledge, the t(3;6)(q23;q27) translocation identified in this patient's neoplasm has not been previously reported in adult T-cell leukemia/lymphoma cases and may explain the early onset of disease. Although adult T-cell leukemia/lymphoma is rare in Romania, the identification of healthy carriers and vertical transmission raise the possibility that Romania might be an endemic region for HTLV-1 infection.

Base Sequence↗
PubMedDate not supplied

[Incidentaloma of the adrenal glands: analysis of 9 surgical cases].

In the assessment of incidentally discovered adrenal masses the detection of hormonal activity and the evaluation of benignity or malignity, either primary or metastatic, constitute the most important issues. This article reports 9 asymptomatic adrenal masses: The histopathological diagnosis consisted of cortical adenoma in 5 cases, adrenal metastatic mass in 2, respectively from a pulmonary microcytoma and from a renal carcinoma, a myelolipoma with leukemic infiltration and an hemorrhagic pseudocyst in the remnants. Evaluation of biochemical activity showed no endocrinological abnormality in all patients except in two cases of adenoma: the positivity of the 1 mg dexamethasone test, the low serum DHEAS levels and a concordant scintigraphic uptake were consistent with the pre-Cushing syndrome in the first case, whereas the ACTH inhibition revealed by low serum DHEAS levels without other hormonal alterations were the biochemical pattern in the second. Ultrasonography has been helpful in the diagnosis of adrenal mass in 6 cases, whereas CT scan allowed an etiopathogenetic diagnosis in 8 cases. All patients were submitted to adrenalectomy through the conventional surgical accesses; in 4 cases the adrenalectomy was performed as a associated intervention during vascular or gastrointestinal surgery. No postoperative death occurred. At follow-up ranging from 3 to 6 years, we recorded 4 deaths: the causes were represented by the progression of the primary malignancy in 2 patients that have been operated on for adrenal metastatic tumors, by hemorrhagic shock from an aorto-duodenal fistula and by systemic infectious complications respectively in the remnant two cases. The other patients were well and the endocrinological assessment showed normal findings. The Authors, according with data from literature, suggest an essential biochemical screening to evaluate the adrenal function in case of incidentally discovered mass: it is characterized by determination of plasma and urinary electrolytes, catecholamines, serum DHEAS and 17-OH progesterone levels, dexamethasone suppression test. In case of asymptomatic mass suspect for pheochromocytoma we advocate the MIBG scintigraphy.. The adrenocortical scintigraphy (NP 59) provides both anatomical and functional characterization of the adrenal glands: the concordant or discordant imaging patterns are useful in the diagnosis of benignity or malignancy. Although the management of patients with incidentally discovered masses remain controversial, we advocate adrenalectomy when they are hormonally hypersecreting, increasing in the diameter or malignant and in association with other abdominal operation.

Adenocarcinoma, Clear Cell↗
PubMed1992

Value of immunohistochemistry in the diagnosis of leukemia cutis: study of 54 cases using paraffin-section markers.

A grave prognosis is usually associated with leukemic skin infiltrates (leukemia cutis). However, some leukemic skin infiltrates are clinically similar to reactive non-leukemic infiltrates in patients with leukemia; thus it is of great importance to distinguish them. Fifty-four cases which were thought clinically to be leukemia cutis underwent immunophenotyping with a panel of nine T, B, monocytic, and macrophage markers using paraffin sections. Immunohistochemistry helped identify 44 cases with leukemia cutis and 10 with reactive infiltrates. In all cases of leukemia cutis, the staining patterns of skin infiltrates were concordant with cell type in the bone marrow. Furthermore, the panel of markers was usually helpful in distinguishing reactive from leukemia infiltrates, especially in cases with chronic lymphatic leukemia. Immunohistochemistry is a valuable adjunct in histopathologic differentiation of skin infiltrates in most cases of leukemia. With formalin-fixed, paraffin-embedded biopsies, we recommend that CD45 (LCA), CD45RO (UCHL-1), CD3, CD20 (L-26), CD43 (Leu-22), CD68 (KP-1), lysozyme, and chloroacetate esterase be considered in cases of systemic leukemia with cutaneous papules and nodules that prove difficult to interpret with routine section.

Acute Disease↗
PubMed1989

Phenotyping of acute myelomonocytic (AMMOL) and monocytic leukemia (AMOL): association of T-cell-related antigens and skin-infiltration in AMOL.

Leukemic blast cells in 25 cases of AMMOL (13 cases) and AMOL (12 cases) were positive for My7 (CD13) and My4 (CD14), while only 44% reacted with LeuM3 (another CD14 MoAb). T-cell-related antigens were detected in 44% of the cases (CD2, 24%; CD4, 12%; CD7 36%). The expression of LeuM3 and TcrAg on blast and monocytic cells was mutually exclusive, with three cases expressing neither LeuM3 nor TcrAg. All six patients with myeloperoxidase negative AMOL and the TcrAG+/LeuM3- phenotype had leukemic skin infiltrations.

Adult↗
PubMed2006

Extensive myocardial infiltration by hemopoietic precursors in a patient with myelodysplastic syndrome.

BACKGROUND: Although myocardial infiltration with leukemic blasts is a known finding in patients with acute leukemia, this phenomenon in myelodysplasia is not reported in the literature. Cardiac symptoms in patients with myelodysplasia are often due to anemia and may be due to iron overload and side effects of therapy. CASE PRESENTATION: Herein we report the first case of neoplastic infiltration of the heart with associated myocardial necrosis in a patient with myelodysplasia. It was associated with unicellular and multifocal geographic areas of necrosis in the left ventricle and the interventricular septum. It is likely that cardiac compromise in our patient was due to a combination of restrictive cardiomyopathy due to leukemic infiltration, concomitant anemia, cardiac dilatation, conduction blocks and myocardial necrosis. Myocardial necrosis was most likely due to a combination of ischemic damage secondary to anemia and prolonged hypotension and extensive leukemic infiltration. Markedly rapid decrease in ejection fraction from 66% to 33% also suggests the role of ischemia, since leukemic infiltration is not expected to cause this degree of systolic dysfunction over a 24-hour period. The diagnosis was not suspected during life due to concomitant signs and symptoms of anemia, pulmonary infections, and pericardial and pleural effusions. The patient succumbed to cardiac failure. CONCLUSION: Hemopoietic cell infiltration was not considered in the differential diagnosis and contributed to this patient's morbidity and mortality. This case highlights the clinical importance of considering myocardial infiltration in patients with myelodysplasia and cardiac symptoms.

Journal Article↗
PubMed2001

Endothelial cell activation by myeloblasts: molecular mechanisms of leukostasis and leukemic cell dissemination.

Leukostasis and tissue infiltration by leukemic cells are poorly understood life-threatening complications of acute leukemia. This study has tested the hypothesis that adhesion receptors and cytokines secreted by blast cells play central roles in these reactions. Immunophenotypic studies showed that acute myeloid leukemia (AML) cells (n = 78) of the M0 to M5 subtypes of the French-American-British Cooperative Group expressed various amounts of adhesion receptors, including CD11a, b, c/CD18, CD49d, e, f/CD29, CD54, sCD15, and L-selectin. The presence of functional adhesion receptors was evaluated using a nonstatic adhesion assay. The number of blast cells attached to unactivated endothelium increased by 7 to 31 times after a 6-hour exposure of endothelium to tumor necrosis factor (TNF)-alpha. Inhibition studies showed that multiple adhesion receptors--including L-selectin, E-selectin, VCAM-1, and CD11/CD18--were involved in blast cell adhesion to TNF-alpha-activated endothelium. Leukemic cells were then cocultured at 37 degrees C on unactivated endothelial cell monolayers for time periods up to 24 hours. A time-dependent increase in the number of blasts attached to the endothelium and a concomitant induction of ICAM-1, VCAM-1, and E-selectin were observed. Additional experiments revealed that endothelial cell activation by leukemic myeloblasts was caused by cytokine secretion by blast cells, in particular TNF-alpha and IL-1 beta, and direct contacts between adhesion receptors expressed by blast cells and endothelial cells. Thus, leukemic cells have the ability to generate conditions that promote their own adhesion to vascular endothelium, a property that may have important implications for the pathophysiology of leukostasis and tissue infiltration by leukemic blast cells. (Blood. 2001;97:2121-2129)

Antigens, CD↗
PubMedDate not supplied

Changes in drug delivery (by blood-brain barrier dysfunction) on arachnoid leukemia: implication for CNS leukemic dissemination.

The infiltrates of intracerebrally inoculated DBLA-6 leukemia cells of the rat were used as an experimental model of meningeal leukemia in acute leukemia. Systemic administration of an antileukemic agent (daunomycin) started at the early stage of the leukemic involvement in the arachnoid, when the blood-brain barrier (blood-cerebrospinal fluid barrier) was not yet broken in the pial microcirculation. In those animals in which the antileukemic agent alone was administered, leukemic infiltration was decreased in the dura while it was clearly observed in the subarachnoid space. In those animals in which the blood-brain barrier was damaged by acute angiotensin-induced hypertension, the chemotherapeutic effect on leukemic infiltration was markedly enhanced in the arachnoid and other parts of the brain. The blood-brain barrier dysfunction was analysed using the technique of fluorescein cine-angiography. The results indicated that the chemotherapeutic effect of the agent given systemically was impeded by the barrier, particularly in early arachnoidal infiltration of leukemic cells. Also, this in vivo experiment showed the importance of drug delivery to tumor cells growing outside blood vessels in cancer chemotherapy.

Angiotensin II↗
PubMed1994

Simultaneous progressive multifocal leukoencephalopathy, Epstein-Barr virus (EBV) latent infection and cerebral parenchymal infiltration during chronic lymphocytic leukemia.

We report a non-HIV patient who had B chronic lymphocytic leukemia (CLL) with progressive multifocal leukoencephalopathy (PML) and diffuse cerebral leukemic parenchymal infiltration in the presence of JC virus and Epstein-Barr virus (EBV) cerebral co-infection. Multiple subcortical hypodensities lining the cortico-subcortical junction were present within the white matter on computerized tomography (CT) scan, with large areas of high signal intensity on T2-weighted sequences on magnetic resonance imaging (MRI). JCV DNA was identified in peripheral blood nuclear cells and cerebrospinal fluid polymerase chain reaction (PCR) DNA/DNA hybridization plus Southern blot analysis. Frontal stereotactic biopsy confirmed the diagnosis of PML by immunocytochemistry, in situ hybridization (ISH) with JC Enzo probe and electron microscopy. Leukemic B cells with the same phenotype as leukemic blood cells were disseminated in the demyelinated areas. They were labeled by anti-latent membrane protein and by BamHl W EBV probe after ISH. Adhesion and activation molecules were positive for CD23. Autopsy showed diffuse visceral leukemic infiltration without acutization. EBV-transformed B lymphocytes would favour JCV penetration and/or intracerebral reactivation of previously latent JCV infection with further development of simultaneous PML and cerebral CLL infiltration in an immunosuppressed patient.

B-Lymphocytes↗
PubMed1995

Constitutive overexpression of the L-selectin gene in fresh leukemic cells of adult T-cell leukemia that can be transactivated by human T-cell lymphotropic virus type 1 Tax.

L-selectin is an adhesion molecule of the selectin family that mediates the initial step of leukocyte adhesion to vascular endothelium. Upon cellular activation, expression of the L-selectin gene is downregulated at both the protein and mRNA levels. To understand the mechanism of leukemic cell infiltration into organs, we studied the expression and regulation of L-selectin mRNA in fresh leukemic cells of adult T-cell leukemia (ATL) patients and investigated the response of the L-selectin promoter to human T-cell lymphotropic virus type 1 (HTLV-1) Tax, which is a viral transcriptional transactivator. Flow cytometry showed that L-selectin was expressed on fresh ATL cells along with other activation antigens. Northern blot analysis showed that ATL cells overexpressed that L-selectin mRNA and that the level was aberrantly upregulated after PMA stimulation. Studies using in situ hybridization showed expression of the L-selectin mRNA in the infiltrating leukemic cells in the liver of two ATL patients. Intravenous injection of a rat T-cell line that overexpresses L-selectin showed increased organ infiltration. The induction of Tax expression in JPX9 cells resulted in about a twofold increase in the mRNA expression levels compared with the basal level. Chloramphenicol acetyltransferase (CAT) assay after transient cotransfection showed about a fivefold transactivation of the L-selectin promoter by Tax. The serum level of the shed form of L-selectin was significantly increased in ATL patients (mean +/- SD, 4,215.4 +/- 4,111 ng/mL) compared with those of asymptomatic carriers and healthy blood donors (mean +/- SD, 1,148.0 +/- 269.0 ng/mL and 991.9 +/- 224 ng/mL, respectively). These results indicated that ATL cells constitutively overexpress the L-selectin gene that can be transactivated by HTLV-1 Tax. The overexpression of L-selectin, as well as of inflammatory cytokines, by ATL cells may provide a basis for ATL cells to attach the vascular endothelium, leading to transmigration and organ infitration.

Adult↗
PubMed1979

Hairy cell leukemia: an autopsy study.

Autopsy material from 5 patients with hairy cell leukemia was examined. In addition to the expected widespread involvement of the hematopoietic system and of the liver, all of the patients had various amounts of pulmonary infiltration by leukemic cells. This infiltration was so severe in one instance that the resulting pulmonary insufficiency was the cause of death. Other areas of hairy cell infiltration included the peripancreatic connective tissue in all cases, kidneys in 3 cases, pericardium in 2 cases, and skin in 1 case. Association of plasma cells with the infiltrating neoplastic cells was prominent. In one patient, foci of large, bizarre cells were found in several lymph nodes and in the pericardium. Whether these cells represent transformation of the hairy cells into a larger, less differentiated cell type, or the emergence of a second hematopoietic neoplasm, is unknown.

Adult↗
PubMed1987

[MR tomography in lymphatic and leukemic bone marrow infiltrations. A comparison with scintigraphy and conventional x-ray diagnosis].

MR is the only noninvasive procedure that can demonstrate changes in the medullary space. If it is combined with bone marrow scintigraphy and conventional x-ray film diagnostics, it can yield a comprehensive diagnosis of bone marrow infiltrations in the regions of the pelvis, thighs and lumbar vertebral column in leukaemia or malignant lymphomas. The T1 relaxation times of the malignant infiltrations are enhanced compared with normal fatty marrow. MR tomography of the bone marrow is particularly suitable for determining the therapy or follow-up control of a malignant systemic disease.

Bone Marrow↗
PubMed1976

Aleukemic leukemia cutis.

Specific leukemic cutaneous infiltrates preceded observable leukemic cells in the blood in a case of acute lymphoblastic leukemia. Examination of a cutaneous biopsy specimen led to early diagnosis of the disease.

Adolescent↗
PubMed2005

[Chronic myelomonocytic leukemia with pleural effusion as the first clinical sign].

We report a chronic myelomonocytic leukemia (CMML) patient whose initial symptom was pleural effusion, which is extremely rare. A 61-year-old male was referred to our hospital because of leukocytosis with blasts and pleural effusion with chest pain. Bone marrow examination showed trilineage dysplasia with 14% blasts and a normal karyotype. He was diagnosed as having MDS (RAEB) and infectious pleuritis on admission. Despite administration of antibiotics, leukocytosis with monocytosis and pleural effusion progressed rapidly. His diagnosis was then changed to CMML-2 and pleural infiltration due to leukemic cells expressing CD13, CD14 and CD33. After the leukocytosis was brought under control with hydroxycarbamide, the patient's pleural effusion disappeared.

Antineoplastic Agents↗
PubMed1994

[Clinicopathologic changes in leukemic lung lesions].

Leukemic lung lesions are complex and various. The authors studied the clinicopathologic changes in 104 autopsied leukemic cases collected from 1953 to 1990. 22.1% of the cases had the symptoms of respiratory tract admission and 40.4% developed the symptoms before death. 12.8% of the patients were diagnosed with X-ray to have tuberculosis and infections on admission and 42% cases have these changes in the terminal phase. The main pathological changes are pleural effusion, pleural adhesion, intrapulmonary infections, hemorrhage and Myleran lung. The rate of leukemic cell infiltration in lungs was 96.2% and the infiltration was mainly interstitial. Intravascular leukemic cell stasis was found in 91.3% of the cases. The pathological changes in each case were usually multiple and 84.6% of the cases had more than two kinds of changes. We compared the X-ray diagnoses with pathological ones and it was shown that complete coincidence was present only in 2.9%, partial coincidence in 58.8% and no coincidence in 38.3% of the cases.

Adolescent↗