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

Congenital leukaemia is a rare disease with approximately 100 cases reported in the literature. It is most often diagnosed as acute myelogenous leukaemia (AML). Leukaemic skin nodules and hepatosplenomegaly are the most frequent clinical findings noted. The laboratory manifestations include a markedly elevated white count with a large percentage of blasts, and a bone marrow aspirate that is cellular with a monotonous population of immature cells. Neonatal leukaemoid and leucoerythroblastic reactions may cause diagnostic confusion. In particular, the neonate with Down's syndrome can manifest either AML or a profound transient myeloproliferative syndrome that is clinically and haematologically indistinguishable from congenital AML. In contrast to congenital leukaemia, however, this myeloproliferative syndrome is transient and resolves spontaneously without anti-leukaemia therapy. On the other hand, untreated congenital leukaemia is a fatal disease. For this reason it is important to establish early diagnosis of congenital leukaemia and institute therapy. Treatment programmes should be modelled after established childhood programmes for acute lymphocytic leukaemia and acute myelogenous leukaemia.
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PMID:Congenital leukaemia and the neonatal myeloproliferative disorders associated with Down's syndrome. 14 90

A case of refractory anaemia with medullary myeloblastosis (RAMM) is described. RAMM is a very rare disease and its relationship to aplastic anaemia and smouldering leukaemia is not clearly established. The diagnosis is confirmed by evaluation of the bone-marrow aspirate and ferrokinetic studies which demonstrate ineffective erythropoiesis. The disease is combined with leucopenia, thrombocytopenia and a hyperplastic bone-marrow. Our patient was monitored for 18 months, during which time her haematological findings remained stable. Since transformation into acute leukaemia occurs in about 25% of the cases, a bone-marrow culture study was performed in order to determine such a leukaemic transformation which is not detectable on examination by the light microscope. Culture studies are discussed along with some prognostic, therapeutic and pathophysiological problems of RAMM.
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PMID:[Anaemia with medullary myeloblastosis (author's transl)]. 27 27

The mitotic indices of the eosinophilic precursors in the bone marrow of 3 untreated patients with eosinophilic leukaemia were found to be significantly lower than those of 11 patients with reactive eosinophilia and those of 15 healthy controls. The same differences were found in the neutrophilic precursors and in the myeloblasts indicating leukaemic involvement of both the eosinophilic and neutrophilic cell lines. Mitotic countings therefore seem to be of diagnostic significance in this rare disease.
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PMID:Low mitotic activity in eosinophilic leukaemia. 81 53

The juvenile type of "chronic myelogenous" leukemia (CMLJT) is a rare disease with only 40 cases reported to date. Clearly distinguishable from adult CML on both clinical and laboratory grounds, is is often confused with "congenital" leukemia, pseudoleukemia, leukemoid reactions or chronic granulomatous disease. According to studies of muramidasuria and colony-forming cells it is neither a chronic nor a granulocytic leukemia. It is a panmyelopathy with monocyte predominance and should thus be classified as a variant of myelo-monocytic leukemia. We review reported responses to chemotherapy and splenectomy and report our results with cytosine arabinoside in the treatment of 2 cases with this disease. Chemotherapy may prolong life and splenectomy may be useful in some cases; but the survival rate is 0%, justifying new approaches.
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PMID:"Chronic myelogenous" leukemia of juvenile type. Report of two cases and review of therapy. 106 53

Congenital leukemia is a rare disease accounting for about 1% of all leukemias in childhood. While cases associated with Down's syndrome not infrequently show a spontaneous regression, such an event is very rare in non-Down cases and exceptional in those (among the latter) which present clonal cytogenetic alterations in the neoplastic cells. We present the case of a patient with congenital leukemia and an abnormal karyotype (limited to the neoplastic clone), in which an apparently spontaneous and prolonged remission occurred after a relapse.
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PMID:Congenital leukemia: persistent spontaneous regression in a patient with an acquired abnormal karyotype. 249 35

Congenital leukemia is a rare disease that can become manifest soon after birth. Cutaneous involvement consists of red, brown, or purple papules and nodules, and confluent areas of purpura. The diagnosis is established by the presence of leukemic cells in biopsy specimens of bone marrow and involved skin, and by immunocytochemical characterization of these cells. We report a case of congenital monocytic leukemia with a normal karyotype, whose disease underwent temporary spontaneous regression.
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PMID:Congenital monocytic leukemia: report of a case with cutaneous involvement, and review of the literature. 269 29

Large granular lymphocyte (LGL) leukemia is a rare disease characterized by clonal expansion of LGL associated with chronic neutropenia, multiple auto-antibodies, and occasionally polyarthritis. We studied cell surface antigen expression and functional activity of leukemic LGL from ten such patients. Using two-color flow cytometric analysis, we found that leukemic LGL from all ten patients expressed the CD3 and HNK-1 markers, while cells from only four patients expressed IgG Fc receptors (FcR). The LGL leukemic cells had little or no NK activity (defined as MHC-nonrestricted cytotoxicity against K562 target cells); however, NK activity could be induced in leukemic LGL by in vitro treatment with as little as 0.05 microgram/mL of anti-CD3 monoclonal antibody. Cell sorting experiments demonstrated that NK activity was induced in CD3+ leukemic LGL (either CD3+, HNK-1+ or CD3+, FcR+) with anti-CD3 monoclonal antibody but not in normal CD3+, FcR- T cells. Treatment with purified interleukin 2 (IL 2) also caused direct activation of some CD3+ leukemic LGL. Despite induction with anti-CD3 MAb or IL 2, activated leukemic LGL did not proliferate or express high density IL 2 receptors detectable by cell sorter analysis. Treatment with alpha interferon had minimal effect on NK activity of LGL leukemic cells. These results suggest that leukemic LGL may provide a useful model for examining the signals required for LGL maturation and activation.
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PMID:Induction of NK activity in large granular lymphocyte leukemia: activation with anti-CD3 monoclonal antibody and interleukin 2. 309 27

Basophilic leukemia is a rare disease accounting for less than 2% of all hematopoietic malignancies and little is known about the best therapeutical approach. We report a case of basophilic leukemia occurring as an accelerated phase of chronic myelogenic leukemia in a 50 year old man with a past history of uranium and thorium exposure. He was successfully treated with intensive chemotherapy and total body irradiation followed by autologous peripheral stem cell transplantation. Such an approach has never been reported in this disease. However, the benefit on survival could not be evaluated because of a fatal opportunistic infection. The cases of basophilic leukemia reported in the literature are discussed.
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PMID:[Basophil leukemia, development of a chronic myeloid leukemia. Apropos of a case and review of the literature]. 332 38

A proportionate analysis of cause of death in 1,401 commercial pressmen was initiated following a report of a cancer cluster in this group. The study found a significant elevated risk of all cancers [proportionate mortality ratio (PMR) = 127] and cancers of the lymphatic and hematopoietic system (PMR = 122), with non-Hodgkin's lymphomas responsible for much of the excess. Three deaths in the cohort were attributed to myelofibrosis, a rare disease associated with benzene exposure. A significantly elevated PMR was also detected for colorectal cancer (PMR = 171) and, among those employed 20 years or more, for cancers of the liver (PMR = 216) and pancreas (PMR = 162). No excess risk of bladder or lung cancer or leukemia was seen. Proportionate mortality analyses rarely show excess risk of both cancer and heart disease in a working population. Surprisingly, a significantly elevated risk of arteriosclerotic heart disease (PMR = 113) was found in this group. These findings indicate that solvent exposure may be associated with excess mortality risk in commercial pressmen.
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PMID:Patterns of mortality among commercial pressmen. 345 42

Congenital leukemia, a rare disease with distinct features, was diagnosed in a 5-week-old girl. The infant was tachypneic and tachycardic, with intercostal retractions, distended abdomen, and multiple hematomas. Despite aggressive treatment, the child died. Histologic examination of tissue obtained at autopsy disclosed leukemic infiltrates in the ocular muscles, conjunctiva, uveal tract, and optic nerve.
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PMID:Ocular involvement in congenital leukemia. 371 59


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