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Contrast enhancing lesions in progressive multifocal leukoencephalopathy: a clinicopathological correlation.

A 60 year old man with chronic lymphocytic leukemia (CLL) developed a subacute neurological illness associated with multiple contrast enhancing lesions on CT scan. At autopsy large demyelinating lesions characteristic of progressive multifocal leukoencephalopathy (PML) were found in the right cerebral hemisphere surrounded by a dense leukemic infiltrate. The areas of contrast enhancement, highly unusual for PML, coincided with the CLL infiltrate.

Brain Neoplasms↗

Leukemic ascites complicating acute myelomonoblastic leukemia.

We describe a patient with acute myelomonoblastic leukemia, jaundice, and ascites. The ascitic fluid contained leukemic cells comparable with those of the blood and bone marrow. Treatment with cytarabine (cytosine arabinoside) caused a decrease in the peripheral blood blast cell count and the serum bilirubin level, but the leukemic ascites did not change. Ascites in patients with acute leukemia must be examined to differentiate leukemic infiltration from other causes such as infection.

Ascites↗

[Clinico-hematic study of lymphoblastic leukemia of childhood].

The authors write their personal experience in 205 children with lymphoblastic leukemia. The disease has its peak incidence between 5 and 14 years, showing a preference for females and white individuals. The clinical form of presentation was "complete" (66.8%) or "incomplete" (33.2%). Most of the symptoms were due to leukemic infiltration of bone marrow and some other tissues. The amount of leukocytes was variable; most of the cases showed anemia and thrombocytopenia. However, a few patients had normal values of hemoglobin and platelets, whose prognostic significance is discussed. After the FAB classification, the L1 variety predominated. About 3.9% of the patients showed radiological bone alterations suggesting acute leukemia.

Adolescent↗

Sampling variability on percutaneous liver biopsy.

Sampling variability of liver biopsy was determined in three consecutive biopsy specimens obtained from each of 118 patients immediately prior to autopsy. No sampling variability was found for fatty liver, alcoholic hepatitis, nonspecific hepatitis, fulminant hepatitis, leukemic infiltrate, and venous congestion. Cirrhosis was diagnosed in 80% of cases at the first biopsy but in all cases after three biopsies. Chronic aggressive and chronic persistent hepatitis were diagnosed correctly in two of three cases each at the first biopsy, and in all cases after three biopsies. Metastatic carcinoma was detected in 46% of cases at the first biopsy and in 69% after three biopsies. Granulomas were missed once on the first biopsy, but found on a subsequent biopsy. The amounts of fat and fibrosis in the biopsy specimens often were not representative of the amounts present at autopsy.

Biopsy, Needle↗

Leukopheresis therapy of leukemic reticuloendotheliosis (hairy cell leukemia).

Intensive leukopheresis has been valuable in the short-term palliation of chronic lymphocytic and granulocytic leukemias. A 47-yr-old man with refractory leukemic reticuloendotheliosis (hairy cell leukemia) manifested by anemia, thrombocytopenia, elevated peripheral leukemia cell counts, generalized lymph node enlargement, and leukemic infiltrative skin disease was treated with serial leukopheresis. Removal of approximately 7 X 10(11) peripheral leukemia cells resulted in marked clinical and hematologic improvement with resolution of enlarged lymph nodes and clearing of skin infiltrates. At the time of this reporting, more than 400 wk since the last leukopheresis, the patient continues to do well. The improvement in all blood counts, reduction in lymph node size, and clearing of skin lesions paralleled the reduction of peripheral leukemia cell load by leukopheresis, suggesting mobilization of leukemia cells from marrow, lymph nodes, and skin. Removal of large numbers of leukemia cells in hairy cell leukemia has the potential of achieving sustained clinical improvement and may be a useful alternative therapy for these patients.

Bacterial Infections↗

Radiotherapy in the management of leukemia.

Localized radiation therapy directed to the spleen and other sites, supplemented by a whole-body radiation technique, appeared to increase survival time in the chronic leukemias. In recent years the alkylating drugs have provided a more convenient form of "whole-body effect", because treatment can be taken at home and yet observation maintained.Small or medium field radiation therapy remains the most efficient means of controlling localized leukemic infiltrations. Splenic irradiation has been used successfully in the control of hemolytic anemia occurring during the course of chronic leukemia where steroids have failed.Proper management of the leukemias requires close co-operation between the radiotherapist, internist and hematologist.

Humans↗

[Long-term second remission in acute leukemia of childhood (author's transl)].

Report on a case of acute childhood leukemia, who presents with the following exceptional features: During complete remission early bilateral leukemic infiltrations of the testes, followed--after an intervall of several months--by a serve, general relapse with ascites. New induction therapy resulted in a second complete remission, persisting for the next 8 years with 6MP as well as after cessation of therapy until up to more than 17 years. Comparable courses are not as yet on record.

Acute Disease↗

[Proctologic examination in pediatrics].

The study included 873 rectal examinations carried out in children where inspection, rectal examination and endoscopy were performed in each case. Most examinations were done in children complaining of gastroenterocolitis. Inspection of gluteal, perianal and sacrococcygeal areas was normal in most cases: however, ammoniacal erythema was frequently found (44.2%). Rectal examination showed hypotonicity of the external sphincter in 146 cases which was linked to prolonged diarrhea and malnutrition. At the endoscopy, the rectal mucosa showed ulcerations, congestion, friability, edema and bleeding; however, it was considered normal in 274 occasions. In 540 patients, the endoscopic diagnosis was colitis of different types. In 54 cases, the endoscopic examination, plus the examination of fecal mucus taken directly from the rectal mucosa led to the diagnosis of amebic colitis. In opposition with adults, neoplastic lesions were uncommon and usually corresponded to leukemic infiltration or tumoral metastases. There were no complications attributable to the process.

Adolescent↗

[Acute leukemia].

Within a few years of the introduction of effective systemic chemotherapy for the treatment of acute leukemia, central nervous system (CNS) leukemia emerged as a significant problem. The strategy for treatment of leukemia is that one achieves cure by destruction of all leukemic cells, through this cannot be verified with present technology. The incidence of CNS-leukemia and the factors influencing this complication have been studied in a group of 131 cases with acute childhood leukemia. The overall incidence of CNS-leukemia was 30% in acute lymphocytic leukemia (ALL), 8% in acute myeloblastic leukemia (AML). However, cumulative incidence of CNS-leukemia calculated by modified Life-Table Method showed 56.8% in children who survived over 48 months after diagnosis of ALL. It was considered that the chief cause of the increased incidence of this complication was prolongation of survival. The CNS-leukemia was significantly greater in patients with under 10 year of age (p greater than 0.05), high initial leukemic cell counts (p less than 0.025), high appearance rate of leukemic cells in peripheral blood (over 50%) (p less than 0.005), and hepatosplenomegaly, especially lymph node enlargement (p less than 0.05). Blood-borne metastasis was considered to be probably the chief route of leukemic infiltration to the CNS. Between 1972 and 1978, 153 children with ALL were treated with multiple methods of CNS-prophylaxis, and were analyzed in relation to treatment regimens, age, sex and initial hematologic status. Patients received CNS-prophylaxis; Group I: three doses of intrathecal methotrexate (MTX 12 mg/m2) and hydrocortisone (HDC 12 mg/m2), Group II: same as in Group I followed by cyclic MTX and HDC, Group III: same as in Group I plus 2,400 rads of cranial irradiation. CNS-leukemia terminated complete remission 25 of 153 patients (16.3%). The cumulative incidence of CNS-leukemia at 4 year calculated by the Kaplan-Meier Method was 40.5% in Group I, 26.9% in Group II, and 14.5% in Group III. Development of CNS-leukemia was more frequent in male than in female (p less than 0.05), and in patients with initial high leukocyte counts (WBC greater than 25,000/mm3). We conclude that the combination of cranial irradiation and intrathecal MTX and HDC was highly efficacious. However, more intensive CNS-prophylaxis should be needed for such a high risk patients in male with high initial WBC.(ABSTRACT TRUNCATED AT 400 WORDS)

Acute Disease↗

Hepatic injury during doxorubicin therapy.

Six patients with acute lymphoblastic leukemia manifested liver dysfunction related to doxorubicin hydrochloride therapy. Other causes, eg, infection, hepatitis, posttransfusion reaction, and leukemic infiltration were ruled out. There was close correlation between the administration of doxorubicin and the appearance of hepatic dysfunction. Doxorubicin may produce an idiosyncratic reaction and must be considered a drug with potential liver toxicity.

Adolescent↗

Association of circulating immune complexes with cytogenetic abnormalities but not with prognosis in acute nonlymphocytic leukemia.

Sera from 91 adult acute nonlymphocytic leukemia patients were tested for circulating immune complexes by the C1q binding test. In 42 patients tested prior to the initiation of therapy, C1q binding activity was inversely correlated with leukemic infiltrate of the bone marrow (rs = -0.34, p less than 0.02), but it was not related to peripheral white blood cell count or presence of infection. Patients with abnormal C1q binding activities (greater than 6%) were more likely to have cytogenetic abnormalities in their bone marrow (p = 0.02); the most frequent abnormality was an extra No. 8 chromosome. The median survival of 8 patients with abnormal values was 1.5 months as compared to 7.8 months in 34 patients with normal values. However, the C1q binding test did not identify patients likely to achieve remission; and after remission was achieved, it did not identify those likely to relapse. Differences between these findings and those recently published by other investigators are discussed.

Acute Disease↗

[Tomodensitometric aspects of a case of intracranial toxoplasmosis in an immunosuppressed patient].

The case of a 42 years old man who had a monoblastic acute leukemia treated by a bone-marrow graft is described. The patient died of a cerebral toxoplasmosis and a cerebral computed tomography done two days before is correlated with anatomopathologic slices done in the same planes than the computed tomagraphy examination. There is a good correlation between necrotic abceded areas and hypodense areas accompanied by serpiginous fixations of contrast media. The immunocompromised patient is very favorable for the development of a cerebral toxoplasmosis. CT, while not specific, has the advantage to suspect the diagnosis which must be discussed with other oportunistic infections, leukemic infiltrates, methotrexate encephalopathy, progressive multifocal encephalopathy, and small infarcts.

Adult↗

[Inhibition of kininogenase activity of cathepsins D by acid-resistant proteinase inhibitor from rabbit serum].

The inhibitory effect of thermo- and acid-resistant inhibitor of trypsin, chymotrypsin and leukocyte proteinases (TASPI) from rabbit serum on the kininogenase activity of cathepsins D from different organs and tissues (human spleen and liver, chicken liver, spleen leukemic infiltrate from patients with myeloid leukemia) was revealed. The progressive mechanism of TASPI and cathepsins D complexation dependent on time and temperature was revealed. The rate constant of inhibition (ki) of chicken liver cathepsin D by TASPI at 37 degrees was 4,25.10(3)M-1 min-1. It was shown that the kininogenase activity of chicken liver cathepsin D was slightly inhibited by the basic pancreatic trypsin and kallikrein inhibitor from bovine organs (Kunitz type) and by soya bean trypsin inhibitor. The role of TASPI as regulator of cathepsins D activity under pathological conditions accompanied by lysosomal disintegration is discussed.

Animals↗

[Diagnosis of chronic monocytic leukemia].

Clinico-morphological, cyto- and biochemical, cytogenetic, immunological studies were carried out in 68 patients with chronic monocytic leukemia. It was found that the main diagnostic signs of this lesion are an associated or isolated enlargement of lymph nodes, the speen and liver, persistent absolute and relative monocytosis in the peripheral blood and sternal needle biopsy specimens, multifocal or diffuse outgrowth of monocytic elements in the bone marrow trepanobiopsy specimens from the ileac bone. Also, dysproteinemia mainly on account on hypergammaglobulinemia, a rather in monocytes and the reduced activity of alkaline phosphatase in neutrophils are characteristic of chronic monocytic leukemia. The basic histomorphological manifestations of the disease are leukemic infiltration by monocytes and reticular cells of the bone marrow, of flat and tubular bones, lymphnodes, the spleen, liver and other organs.

Anemia↗

[Kininogenase activity of cathepsins D].

The kininogenase activity of highly purified preparations of cathepsins D from human liver and spleen, leukemic infiltrate obtained from patients with myeloic leukemia, and from chicken liver was studied. It was found that pepstatin, a specific inhibitor of carboxylic proteinases, inhibits this activity of cathepsins D. Interaction of chicken liver cathepsin D with human plasma substrate, which is possibly a low molecular weight kininogen (Ks = 1.3.10(-7) M) results in a production of the bradikinin analog methionyl-lysyl-bradikinin. The role of cathepsins D as potent inflammatory agents responsible for the generation of biologically active peptides--mediators of inflammation from the protein substrates including kininogens under desintegration of lysosomes is discussed.

Animals↗

[Lesions of the lungs in children with acute leukemia].

Investigation of the lungs of 65 children who died of acute leukemia, revealed pathological alterations in 39 of them. Specific leukemic infiltration of the lung parenchyma was found in 29.3%, but it did not play an important role in tanathogenesis. Pneumonias were diagnosed in 46.1% of the observations being the main cause of death in 23%. The inflammatory processes showed a marked tendency for spread because of low immunologic responsiveness of the patients and poor neutrophil reaction. Because of this, segmental pneumonias predominated in our observations, and in 5 cases total bilateral interstitial pneumonias had developed. Three of them were due to Pneumocystis carinii and two were drug pulmonitis.

Adolescent↗

Clinical utility of initial terminal deoxynucleotidyl transferase determinations in childhood acute leukemias.

Terminal deoxynucleotidyl transferase (TDT) activity was measured in bone marrow lymphoblasts obtained at diagnosis from 168 consecutive patients with childhood acute leukemia. Absolute concentrations of TDT were increased (greater than or equal to 20 units/10(8) blasts) in samples from 98 of 112 assessable patients with acute lymphocyte leukemia (ALL). The values ranged from less than 1 to 1502 units/10(8) blasts with a median of 90 units contrasted with less than 1 to 219 units (median, 2.6 units) in studies of children without leukemia. Results of an immunofluorescence assay were in good agreement with enzymatic detection of the polymerase. Among 115 patients with adequate marrow smears, 105 had TDT-positive blasts. By contrast, in most children with acute myelogenous leukemia, TDT activity was either undetectable or less than 10 units/10(8) blasts. Although the highest levels of TDT were found in blasts with the common ALL phenotype, quantitative determinations were not significantly related to the major immunological subtypes of ALL or to morphological features or periodic acid-Schiff reactivity of the lymphoblasts. The probability that a newly diagnosed case of leukemia would be ALL was 90% if TDT levels were greater than 20 units/10(8) blasts. We conclude that absolute concentrations of TDT, as determined in this study, are of little value in identifying subclasses of ALL. The immunofluorescence assay, which is much less expensive and easier to perform than the enzyme assay, should prove useful for confirming the diagnosis of ALL and for detecting extramedullary sites of leukemic infiltration.

Acute Disease↗

[Symptomatology and clinical diagnosis in acute leukemia].

Anemia, bleeding tendency, infection, hepato-splenomegaly and enlarged lymphenodes are the main symptoms of acute leukemia. Moreover, leukemic infiltrations may be found in many other organs. Diagnostic procedures include the morphological, cytochemical and immunological classification of the leukaemic cells as well as the determination of the extent of the disease and functioning of the different organs. Psycho-social evaluation should also take place at the beginning of treatment.

Acute Disease↗