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)

This review covers the diagnosis and management of natural killer and peripheral T-cell lymphomas (PTCL). Problems with PTCL include their rarity, representing usually 10-15% of non-Hodgkin's lymphomas in the Western Hemisphere, morphologic heterogeneity, and lack of immunophenotypic markers for clonality. Additionally, their clinical behavior is variable and may not correlate with morphology. Dr. Kinney gives a general overview of the diagnosis of PTCL and NK cell neoplasms. Emphasis will be placed on extranodal T cell and natural killer (NK) cell lymphomas such as hepatosplenic lymphoma, subcutaneous panniculitis-like lymphoma and nasal/nasal type T/NK-cell lymphoma. The use of ALK gene regulation in the classification of anaplastic large cell lymphoma is also reviewed. Dr. Loughran describes current understanding of the pathogenesis of large granular lymphocyte (LGL) leukemia. The discussion focuses on LGL leukemia as an instructive model of dysregulated apoptosis causing both malignant and autoimmune disease. Current management options and mechanisms of therapeutic response are also described. Dr. Greer addresses whether PTCL should be treated differently from the more common diffuse large B cell lymphomas. He discusses the therapeutic options for anaplastic large cell lymphoma (ALCL), from a conservative approach for primary cutaneous ALCL to combination chemotherapy for the highly chemosensitive ALCL expressing anaplastic lymphoma kinase. He reviews therapy options for the extranodal subtypes of PTCL by drawing from series in adults, pediatrics, dermatology, and the Far East.
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PMID:T cell and NK cell lymphoproliferative disorders. 1172 88

Peripheral (post-thymic) T-cell lymphoma consists of a wide spectrum of disorders with marked differences in biology and behavior. Proper classification is pivotal for evaluating treatment results, and most studies performed a decade ago lump together different disease entities and cannot be interpreted. With improved use of immunophenotyping and molecular methods for these disorders, their exact nature is better defined in the Revised European-American Lymphoma and subsequent World Health Organization (WHO) classifications. The WHO classification of post- thymic T/natural killer (NK)-cell lymphoma consists of 15 entities, including about 30% that are unclassified cases. A wide range in incidence exists between different populations, but it is likely to be lower than previously estimated. Certain entities, like nasal/nasal-type T/NK-cell lymphoma and human T-cell leukemia/lymphoma virus 1, are much more prevalent in certain racial groups and show exquisite viral association. In these entities as a group, prognosis and treatment seem inferior to those of their B-cell counterparts, but treatment must be tailored to the exact pathologic diagnosis and prognostic index. Aggressive combination chemotherapy appears to be curative for certain entities (eg, anaplastic lymphoma kinase-positive), whereas purine analogues may be useful for low-grade entities. The role of autologous and allogeneic stem cell transplantation is still poorly defined. Specific antibody-based therapy is also on the horizon.
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PMID:Peripheral T-cell lymphoma. 1216 19

The World Health Organization (WHO) classification of malignant lymphoma presented a list of disease entities well defined by clinical, immunological and genetic features. Therefore, the current diagnosis of peripheral T/NK-cell lymphomas (PTNKLs) essentially requires the inclusion of anatomical sites of disease and phenotypical features. We analyzed 408 Japanese cases of PTNKLs in order to clarify the relationship between anatomical sites of disease and phenotypical features and to translate the functional subsets of T and NK cells into their diagnoses for further understanding lymphomatic biology. The T/NK-cell lymphoma entities were allocated into three categories: (i) cytotoxic memory T-cell and/or NK-cell lymphoma (n = 151) consisting of extranodal NK/T-cell tumors other than mycosis fungoides (MF); (ii) non-cytotoxic memory T-cell lymphoma (n = 142) consisting of nodal and cutaneous tumors such as angioimmunoblastic T-cell lymphoma, adult T-cell lymphoma/leukemia and MF; and (iii) anaplastic lymphoma kinase positive anaplastic large cell lymphoma (n = 110) that has unique features and might be regarded as cytotoxic 'naive' T-cell lymphoma. Overall, these three categories were significantly correlated with age of onset, anatomical sites, the level of expression of cytotoxic molecules and CD45RO, and association with Epstein-Barr virus. This concept might provide a new insight enabling further understanding of the interrelationships among WHO T/NK-cell disease entities.
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PMID:Phenotypic analysis of peripheral T/NK cell lymphoma: study of 408 Japanese cases with special reference to their anatomical sites. 1278 7

A chronic infectious mononucleosis-like illness caused by Epstein-Barr virus (EBV) is called 'chronic active EBV disease', which is defined as an EBV-associated lymphoproliferative disease. This lymphoproliferative disease is rare and predominantly occurs in Japanese children. Between 1998 and 2010, seven adult-onset cases (aged 20-45 years, median 39 years) were identified, which initially presented with inflammatory diseases, including hepatitis, interstitial pneumonitis, uveitis, nephritis and hypersensitivity to mosquito bites. They showed an EBV viral load in the peripheral blood and evidence of EBV infection of T or natural killer (NK) cells. Five cases (71.4%) developed EBV-positive T/NK-cell lymphoma/leukaemia at a median of 5 years (range 1-7 years) after the diagnosis. Although l-asparaginase-containing chemotherapy was effective for the lymphomas, only allogeneic haematopoietic cell transplantation eradicated EBV-infected cells. This observation indicates that persistent EBV infection of T or NK cells defines a distinct disease entity, which provides an underlying condition for EBV-positive T/NK-cell lymphoma/leukaemia.
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PMID:T/NK cell type chronic active Epstein-Barr virus disease in adults: an underlying condition for Epstein-Barr virus-associated T/NK-cell lymphoma. 2224 63