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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1972 to 1981 10 patients with hairy cell leukaemia were observed in the Medical University Clinic Cologne, this represents 1.8% of all leukaemias. Typical clinical signs are splenomegaly, no or only slightly enlarged lymph nodes and a moderate hepatomegaly. Almost in all cases an anaemia, thrombocytopenia and neutropenia with lymphocytosis was found, mostly combined as pancytopenia. The pathognomonic tartrate resistant acid phosphatase was found in the hairy cells to a differing amount besides a fibrosis and a lymphatic infiltration of the bone marrow. A normalization of the anaemia, the thrombocytopenia and the neutropenia was reached by splenectomy, but the increased susceptibility to infections could not be affected significantly.
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PMID:[Clinical aspects of hairy cell leukemia and its modification by splenectomy]. 676 22

In order to assess the prevalence of venocclusive disease in autopsied recipients of bone marrow transplantation, we reviewed coded liver histology from 204 consecutive autopsied recipients transplanted for leukemia (142), other malignancies (5), or aplastic anemia (57). Twenty-seven patients with leukemia, 2 with carcinoma, and 3 with aplasia had venocclusive disease and survived 2-86 days post-transplant. Early lesions showed subintimal edema and hemorrhage within small central venules and centrilobular congestion with hepatocyte degeneration. Later lesions showed subtotal to complete fibrous obliteration of the central venule lumina and centrilobular sinusoidal fibrosis. Thirteen patients had a subclinical course, and 19 were symptomatic. Venocclusive disease was life-threatening or lethal in 13. Typical symptoms developed 1-3 wk post-transplant and consisted of sudden weight gain, hepatic enlargement, ascites, high bilirubin, and encephalopathy. Statistical analyses showed a significantly higher prevalence of venocclusive disease associated with transplantation for leukemia (P = 0.014), pretransplant conditioning with more rigorous chemoradiotherapy regimens (P < 0.001) and three- to fourfold increase of venocclusive disease in patients whose conditioning included dimethyl busulfan (P < 0.005). Abnormal liver tests before transplant were also more prevalent among patients with venocclusive disease. No factors predicted the clinical outcome of established venocclusive disease. Venocclusive disease showed no association with hepatic graft-versus-host disease even among prolonged cases with severe periportal hepatitis and cholestasis. Other centrilobular lesions (hepatocyte degeneration, sinusoidal fibrosis, and phlebosclerosis) were identified in 23 patients. These non-specific changes may occur with viral hepatitis, graft-versus-host disease or chemoradiotherapy effects.
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PMID:An analysis of hepatic venocclusive disease and centrilobular hepatic degeneration following bone marrow transplantation. 700 4

Thirty-six patients with hairy-cell leukemia (HCL) were evaluated, and were divided in two major subtypes: leukopenic (WBC less than 3000/microliters) and non-leukopenic (WBC greater than or equal to 3000/microliters). There were 22 leukopenic and 14 non-leukopenic patients. The leukopenic group were older than the non-leukopenic group, with an average age of 58.4 years compared with 47.6 years. The male/female ratio was higher in the leukopenic (6.3) than the non-leukopenic (2.0) patients. Splenomegaly, hepatomegaly and lymphadenopathy were found in 66%, 32%, and 18% of the leukopenic patients, compared with 92%, 57%, and 35% in the non-leukopenic patients. The leukopenic HCL was associated with more severe anemia, granulocytopenia, monocytopenia, and thrombocytopenia, and higher incidence of serious infections than the non-leukopenic HCL. Increased bone marrow reticulin fibers and unsuccessful marrow aspirations (dry taps) were more frequently associated with the leukopenic than the non-leukopenic HCL.
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PMID:Clinicopathological subtypes in hairy-cell leukemia. 710 11

The occurrence of a transitory aplasia followed shortly thereafter by an acute leukemia in a known sequence in children is rare. We report 13 observations involving children between 6 months and 10 years of age. Splenomegaly was observed in 5 patients and hepatomegaly in two. There was a tricytopenia in 5 cases, bicytopenia in 4 and an isolated cytopenia in the remaining 4 cases. The phase of aplasia was short, lasting from 6 to 30 days. Complete bone marrow recovery occurred with integral restitution. Remission was spontaneous or followed transfusion or corticosteroid therapy and lasted for 2 to 6 months. The leukemia had no particular character when it appeared: there was one acute myeloblastic leukemia, 11 acute lymphoblastic cases and one sarcoma. Median survival time was 5 to 32 months and relapses were not aplasic. It is noteworthy that among these cases there are two long remissions lasting more than ten years. The interpretation of these observations is difficult because of the following choice: an initially non-leukemic aplasia or a leukemia present at the onset but undetected, camouflaged or confined to several infrequent blast cell islets.
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PMID:[Acute curable preleukemic bone marrow aplasia in children]. 712 79

The natural history of 45 cases of smouldering leukaemia has been studied. Males and females were equally represented, with a median age of 60.5. The median survival of the whole group was only 20 months, but rare cases lived 10 years or longer. 38% developed acute leukaemia; the remainder usually died of the results of marrow failure. Although it was possible to divide these marrow dysplasias morphologically into 3 major subgroups (refractory anaemia with excess of myeloblasts, chronic myelomonocytic leukaemia and chronic erythraemic myelosis), several displayed transitional features. Many showed refractory macrocytosis at diagnosis. The survival of the 3 groups was similar, though patients with high monocyte counts tended to present with less anaemia and fared rather better than the others. Statistical analysis suggests that increasing age, severe anaemia, thrombocytopenia and hepatomegaly are associated with a poor prognosis. Chemotherapy, when attempted, was usually unsuccessful.
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PMID:Natural history of smouldering leukaemia. 715 Apr 70

We evaluated the presence of abdominal organomegaly and lymphadenopathy with ultrasound in 92 children with acute lymphoblastic leukemia (ALL) prior to chemotherapy, and compared these findings with the different immunophenotypes, age groups, and white blood cell (WBC) counts as well as the survival of the patients and the clinical findings of organomegaly. All the patients (n = 13) with a WBC higher than 50/microL showed intra-abdominal pathology compared with the patients with a low WBC, of whom 37% (n = 18) had normal scans. The children with a high WBC count also had hepatomegaly (P = 0.003) and splenomegaly (P = 0.06) significantly more often, and showed high echogenicity of the kidneys (P = 0.001). Lymphadenopathy was found significantly more often in children with T-cell leukemia (P = 0.005). The younger age groups (0 to 2 and 2 to 5 years of age) had hepatomegaly significantly more often (P = 0.02), and the youngest age group (0 to 2 years) showed increased echogenicity of the kidneys more often (P = 0.04). Ultrasound showed hepatomegaly in 14 patients and splenomegaly in 23 patients who were assessed clinically as normal. According to our results, abdominal ultrasound is a useful tool for evaluating abdominal organomegaly and the extramedullary leukemic burden and can give information that is not available in clinical examination. There was no statistical association between the primary ultrasonographic findings and the patients' later survival.
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PMID:Abdominal sonographic findings at primary diagnosis of acute lymphoblastic leukemia in children: a comparison with different clinical risk factors. 757 87

Under myelodysplastic syndromes we presume a heterogeneous group of malignant hemopathies with clearly described characteristics of the disease given by a cooperative group of French, American and British authors. Myelodysplastic syndromes most frequently occur at older age. Survival of these patients, after the diagnosis is made, is mostly short because the disease evolves into acute leukemia. Myelodysplastic syndrome is characterized by appearance of refractive anemia, leukemia, thrombocytopenia with signs of expressed dishematopoiesis of the bone marrow. Clear criteria which could define forms with fast or slow course leading to acute leukemia don't exist, so there is a need to group patients into those with good or with bad outcome. The investigation included following parameters important for the outlook of the disease: 1. enlargement of lymph nodes, liver and spleen, 2. biochemical examination of peripheral blood, 3. cytomorphologic changes in the peripheral blood cells and bone marrow. By a follow-up of described parameters a statistically significant influence on survival of the sick concerning the degree of present anemia, absolute number of granulocytes, number of thrombocytes, dishematopoiesis of the peripheral blood and bone marrow, lymphadenomegaly, hepatomegaly and splenomegaly was not found. The percentage of blast in the peripheral blood and bone marrow has a statistically significant influence on patients' short survival.
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PMID:[Prognosis in patients with myelodysplastic syndromes based on cytomorphologic and clinico-biologic parameters]. 779 74

Previously diagnosed cases of hepatosinusoidal T-cell lymphoma and malignant histiocytosis (MH) may include lymphoid neoplasms of natural killer (NK) cell lineage associated with Epstein-Barr virus (EBV). Such hepatosinusoidal neoplasms were found to demonstrate hepatomegaly but not lymphadenopathy, and all were diagnosed by a liver biopsy. Sixteen adult patients diagnosed with hepatosinusoidal leukaemia/lymphoma (six NK-cell leukaemia/lymphomas [NKLLs], five instances of MH, three T-cell malignant lymphomas [T-MLs], and two adult T-cell leukaemia/lymphomas [ATLLs] were examined for EBV by in situ hybridization, then were studied immunohistochemically and subjected to a DNA analysis. Among our five patients with MH, neoplastic cells showed T-cells, but no histiocytic markers, and they were considered to have either a T-cell or NK-cell lineage. All NKLLs, MHs and T-MLs, except for ATLLs accompanied by reactive hemophagocytic histiocytes, varied in number in each case. In situ hybridization revealed the presence of EBV in the nuclei of atypical cells in all of the six lymphoid neoplasms of NK-cell lineage. Each case of MH and each T-ML which represented EBV demonstrated no definite T-cell or histiocytic markers. Patients with ATLL did not reveal EBV. In all patients with hemophagocytosis, EBV was present in the nuclei of the neoplastic lymphocytes, but not in the hemophagocytic cells. Finally, the 16 cases were reclassified into eight cases with EBV-containing NKLLs, six T-MLs, and two ATLLs. In addition, no true histiocytic neoplasms were observed. The mechanism of hemophagocytosis may be therefore the production of lymphokines (macrophage-activating factors) by neoplastic lymphocytes. EBV-associated hepatosinusoidal leukaemia/lymphoma may thus contain a lymphoid neoplasm of NK-cell lineage, which made it difficult to be distinguished from the previously designated malignant histiocytosis.
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PMID:Hepatosinusoidal leukaemia/lymphoma consisting of Epstein-Barr virus-containing natural killer cell leukaemia/lymphoma and T-cell lymphoma; mimicking malignant histiocytosis. 779 96

We present the clinical and immunological features of a rare case of chronic lymphoid leukaemia with lymphoplasmacytoid morphology. The patient was first admitted suffering from weakness, pallor, dyspnoea, marked splenomegaly, hepatomegaly and systemic lymphadenopathy and panhypogammaglobulinaemia. White blood cell count revealed important leukocytosis (220 x 10(9) WBC/l) with 2% neutrophils and 98% lymphoid cells showing lymphoplasmacytoid features, while lymphoid cells of identical morphology severely infiltrated the bone marrow and lymph nodes. The disease, initially controlled by non aggressive chemotherapy over a period of 30 months, later evolved to a clinical and haematological picture suggestive of Richter's syndrome. Immunophenotyping of the leukaemic cells demonstrated a monoclonal expansion of B-cells bearing surface markers of typical CLL (CD5, CD19, CD20, CD21, CD22, CD23, CD24, CD40 and low density IgM+IgD/kappa) and also the CD11c and CD38 antigens. A proportion of these cells expressed activation markers (CD25, CD69 and CD71). Following in vitro activation with TPA or PWM, the cells responded by weak incorporation of 3H-TdR but failed to secrete immunoglobulins. These findings confirm the broad morphological, phenotypical and clinical spectrum of chronic lymphoid leukaemias.
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PMID:Monoclonal expansion of immunoglobulin not-secreting CD5+ CD11c+ CD38+ B-cells in a rare case of chronic lymphoplasmacytoid leukaemia. 797 Dec 44

The clinical characteristics and treatment outcome in 40 children with acute promyelocytic leukemia (APL) treated at institutions participating in the Children's Cancer and Leukemia Study Group (CCLSG) were studied retrospectively. The median age at diagnosis was 8 years old. Bleeding diathesis was the predominant presenting symptom (90%), associated with laboratory findings of disseminated intravascular coagulation. Hepatomegaly, splenomegaly and lymphadenopathy were observed in 35%, 10%, and 15% of the cases, respectively. The median WBC count was 4.25 x 10(9)/l. Anemia (hemoglobin < 8 g/dl) and thrombocytopenia (< 30 x 10(9)/l) were present in more than half of the patients. Cytogenetic studies demonstrated the characteristic 15; 17 translocation in about 90% of the patients analyzed. Induction therapy consisted of cytosine arabinoside and an anthracycline, with or without other agents. Twenty-nine patients (73%) achieved complete remission (CR) while early fatal hemorrhage was the predominant cause of induction failure. The survival rates continued to decrease (28% at 3 years, 24% at 5 years, and 7.9% at 10 years) due to late marrow relapses. Anthracycline cardiotoxicity was fatal in three patients in remission. These clinical features of childhood APL should be taken into account in the development of new protocols.
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PMID:[Clinical characteristics and treatment results of acute promyelocytic leukemia in children (Children's Cancer and Leukemia Study Group)]. 823 Jul 51


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