Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case is reported of a 45 year old woman with the rare leukaemia of natural killer cell large granular lymphocyte (NK/ LGL) type. Cytometric analysis of leukaemic blasts showed that they were positive for CD2, CD38, and CD56 antigens but negative for a series of antigens including CD3, CD7, CD16, and HLA-DR. Rearrangements of the beta T cell receptor, and heavy and kappa immunoglobulin genes were not detected and neither were chromosomal abnormalities. Leukaemic blasts developed NK cytotoxicity. The patient failed to respond to aggressive chemotherapy and died three months after diagnosis. The lack of expression of HLA-DR is an extraordinary characteristic of this case, as all cases of acute NK cell leukaemias described to date expressed HLA-DR. The immunophenotype observed in the NK cell leukaemic blasts may represent the counterpart of a hypothetical normal cell precursor in an early stage of ontogenic NK cell development.
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PMID:Leukaemia of natural killer cell large granular lymphocyte type with HLA-DR-CD16-CD56bright+ phenotype. 903 41

We performed immunocytochemistry to detect mdr1 and mdr3 P-glycoproteins (P-gps) in 81 patients with acute and chronic leukemia, using the mdr1 P-gp-specific monoclonal antibody (MoAb) MRK16, and the mdr3 P-gp-specific MDR3M. Immunoreactivity for the mdr1 gene product was positive in 27 out of 81 (33%) patients. Immunoreactivity with the anti-mdr3 P-gp MoAb was positive in 20 out of 81 (25%) patients. Of 54 patients with acute leukemia, 17 (31%) were positive for mdr1 P-gp and 8 (15%) for mdr3 P-gp. A high proportion (60%) of patients with chronic lymphocytic leukemia (CLL) were mdr3 P-gp positive. Of the patients with granular-lymphocyte proliferative disorder (GLPD), a chronic T-cell or natural killer cell leukemia, 8/17 (47%) were positive for mdr1 P-gp and 6/17 (35%) for mdr3 P-gp. Of 23 patients with chronic leukemia (CLL and GLPD), 10 (37%) were positive for mdr1 P-gp and 12 (44%) for mdr3 P-gp. To clarify the function of the mdr3 P-gp, we examined the intracellular rhodamine123 (Rh123) levels of mdr1 P-gp-negative and mdr3 P-gp-positive leukemic cells from patients with acute lymphocytic leukaemia, on the addition of 10 microM cyclosporin A (CyA). The addition of CyA led to significant increases in intracellular Rh123 levels in mdr1 P-gp-negative and mdr3 P-gp-positive leukemic cells. Results of the assay for dye efflux suggested that the mdr3 P-gp has a role in drug resistance, and functional drug-efflux capacity. In 31 acute leukemia patients at initial diagnosis, mdr1 or mdr3 P-gp expression correlated significantly to an outcome of complete remission (CR). In 54 acute leukemia patients, exposure to precytotoxic agents correlated significantly to expression, with a significant higher number of patients mdr1 or mdr3 P-gp positive than negative. In the 54 patients with acute leukemia, mdr1 P-gp expression correlated to mdr3 P-gp expression significantly (p=0.0007). In the 27 patients with chronic leukemia (CLL and GLPD), mdr1 and mdr3 P-gp expression did not correlate to exposure to precytotoxic agents, nor did mdr1 P-gp expression correlate to mdr3 P-gp expression. It may be speculated that precytotoxic agents induced mdr1 and mdr3 P-gp expression in acute leukemia; however, in chronic leukemia, both P-gps were expressed independently of exposure to precytotoxic agents.
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PMID:Expression of the MDR1 and MDR3 gene products in acute and chronic leukemias. 915 Mar 48

Although acute myeloid leukemia (AML) with t(8;21) (q22;q22) is associated with a high complete remission (CR) rate and prolonged disease-free survival, treatment outcome is not universally favorable. Identifying factors that predict for treatment outcome might allow therapy to be optimized based on risk. AML with t(8;21) has a distinctive immunophenotype, characterized by expression of the myeloid and stem cell antigens CD13, CD15, CD34, and HLADr, and frequent expression of the B-cell antigen CD19 and the neural cell adhesion molecule CD56, a natural killer cell/stem cell antigen. Because CD56 expression has been associated with both extramedullary leukemia and multidrug resistance, we sought to correlate CD56 expression with treatment outcome in AML with t(8;21). Pretreatment leukemia cells from 29 adult de novo AML patients with t(8;21) treated on Cancer and Leukemia Group B (CALGB) protocols were immunophenotyped by multiparameter flow cytometry as part of a prospective immunophenotyping study of adult AML (CALGB 8361). CD56 was expressed in 16 cases (55%). There was no correlation between CD56 expression and age, sex, white blood cell count, granulocyte count, the presence of additional cytogenetic abnormalities, or the presence of extramedullary disease at diagnosis. The CR rate to standard-dose cytarabine and daunorubicin was similar for cases with and without CD56 expression (88% v 92%; P = 1.0). Post-CR therapy included at least one course of high-dose cytarabine in 24 of 26 patients who achieved CR; numbers of courses administered were similar in cases with and without CD56 expression. Although post-CR therapy did not differ, CR duration was significantly shorter in cases with CD56 expression compared with those without (median, 8.7 months v not reached; P = .01), as was survival (median, 16.5 months v not reached; P = .008). We conclude that CD56 expression in AML with t(8;21) is associated with significantly shorter CR duration and survival. Our results suggest that CD56 expression may be useful in stratifying therapy for this subtype of AML.
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PMID:Expression of the neural cell adhesion molecule CD56 is associated with short remission duration and survival in acute myeloid leukemia with t(8;21)(q22;q22). 926 84

Presently, fever is thought to play a beneficial role in the control and containment of infection by curtailing virus replication, enhancing natural killer cell activity, and killing of natural killer-resistant leukemia cells. The first article in this set of articles on fever physiology, which was printed in a previous issue, covered the role of cytokines in fever. This article expands the physiology to describe the body's positive response to fever, so critical care nurses can decide which patients to cool, which ones not to cool, and how to revise cooling protocols.
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PMID:Pathophysiology of fever. Part 2: Relooking at cooling interventions. 933 64

A 12-year-old Korean girl gave a 9-year history of recurrent necrotizing papules and vesicles on the face, scalp and extremities. Skin biopsy specimens showed an atypical lymphoreticular infiltrate with vasculitis in the dermis and subcutis. In situ hybridization demonstrated latent infection by Epstein-Barr virus (EBV) of the lymphoid cells in the dermis. The disease was diagnosed as an EBV-associated lymphoproliferative skin eruption presenting as recurrent necrotic papulovesicles. The patient subsequently developed large granular lymphocytic leukaemia of natural killer cell origin. Our observations suggest that a patient with an EBV-associated lymphoproliferative skin eruption presenting with recurrent necrotic papulovesicles might progress to develop leukaemia as well as lymphoma.
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PMID:Epstein-Barr virus-associated lymphoproliferative eruption with progression to large granular lymphocytic leukaemia. 934 43

We report a case of aggressive natural killer cell leukemia (ANKL) in a Caucasian boy diagnosed on clinical, cytologic, immunophenotypic and genotypic grounds. An anomalous karyotype and integration of Epstein-Barr virus (EBV) within the leukemic population were identified.
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PMID:Aggressive natural killer cell leukemia: report of a case in a Caucasian boy. 954 32

A 32 year old Caucasian male presented with fever, massive hepatosplenomegaly and lymphadenopathy. Peripheral blood and bone marrow findings were consistent, both morphologically and immunophenotypically, with a natural killer cell leukemia/lymphoma. The disease was rapidly progressive and was fatal within five days after presentation.
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PMID:Aggressive natural killer cell leukemia/lymphoma. 964 96

We report a case of natural killer cell large granular lymphocytic (NK-LGL) leukaemia successfully treated with allogeneic peripheral blood stem cell transplantation (allo-PBSCT). The peripheral blood (PB) revealed an abnormal expansion of LGL that were CD3-, CD16- and CD56+, and had natural killer activity. In situ EBER-1 hybridization of the PB mononuclear cells showed the presence of Epstein-Barr virus (EBV) in the LGL as well as in lymphocytes infiltrating the tonsils and colon. Southern blotting with an EBV-terminal repetitive sequence probe demonstrated clonal proliferation of EBV+ cells. The patient received allo-PBSCT from his HLA-matched sister with a conditioning regimen involving the use of cyclophosphamide and fractionated total body irradiation. The patient promptly recovered trilineage haematopoiesis without graft-versus-host disease, and has been in complete remission without therapy for 10 months since allo-PBSCT, suggesting that allo-PBSCT could eradicate the NK-LGL leukaemic cells.
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PMID:Successful treatment of Epstein-Barr virus-associated natural killer cell large granular lymphocytic leukaemia using allogeneic peripheral blood stem cell transplantation. 967 65

This report describes six cases of feline large granular lymphocyte lymphoma identified by light microscopy on the basis of their characteristic azurophilic granulation in Giemsa-stained plastic sections and by electron microscopy on the basis of their typical granules. Although the granules of all the tumor cells were negative for peroxidase activity, they all demonstrated chloroacetate-esterase and acid phosphatase activity. All the tumors reacted with cross-reacting antibodies against the CD3 antigen (epsilon chain) and did not react with a cross-reacting monoclonal antibody directed against epitopes on cytoplasmic domains of the CD20 antigen. Three tumors had a positive reaction with a monoclonal human CD57-like antibody. This is highly suggestive of either a cytotoxic T cell or a natural killer cell origin of the neoplasias. In three cats, although other abdominal organs were affected to a variable extent, the main neoplastic lesions were localized in the gastrointestinal tract and the jejunal lymph nodes. In contrast, in the other three cats, organ involvement was more widespread, affecting the lung (two), myocardium (two), precardiac mediastinum (one), salivary gland (one), and spinal cord (one); in addition, leukemia was present in two of these cats. The data presented indicate that tumors made up of large granular lymphocytes occur more frequently in cats than previously assumed and that they share many characteristic features with specific subtypes of clonal disorders of large granular lymphocytes in humans.
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PMID:Large granular lymphocyte leukemia/lymphoma in six cats. 975 42

Growth hormone (GH) and prolactin (PRL) quality as lymphohaemopoietic growth and differentiation factors, and so does insulin-like growth factor (IGF)-I, which mediates many of GH activities. Although there is only limited evidence that endocrine, paracrine or autocrine GH or PRL play a role in human leukaemia and lymphoma, the expression of these factors or their receptors may have diagnostic or therapeutic implications. Indeed, the participation of GH, PRL or IGF-I in the development or progression of certain haematological malignancies or to the antitumour immune response has been documented. Examples discussed in this review include a rat lymphoma in which the PRL receptor acts as an oncogene; the rat Nb2 lymphoma, which is dependent on PRL for growth; and experiments showing that PRL stimulates natural killer cell activity and the development of lymphokine-activated killer cells.
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PMID:A role for growth hormone and prolactin in leukaemia and lymphoma? 981 88


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