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Albitar, Maher (1)
Alder, Hansjuerg (1)
Björkholm, Magnus (1)
Casella, James F. (1)
Cortes, Jorge (1)
Everett, Allen D. (1)
Fedorko, Daniel P. (1)
Ganz, Tomas (1)
Goldin, Lynn R. (1)
Heerema, Nyla (1)
Janik, John E. (1)
Kantarjian, Hagop (1)
Kristinsson, Sigurdur Y. (1)
Landgren, Ola (1)
Lee, Pauline (1)
Liu, Chang-Gong (1)
Ma, Wanlong (1)
Mahanty, Siddhartha (1)
Morris, John C. (1)
Munker, Reinhold (1)
Nemeth, Elizabeta (1)
Ramanathan, Roshan (1)
Savage, William J. (1)
Stewart, Donn M. (1)
Taccioli, Cristian (1)
Turesson, Ingemar (1)
Yeh, Chen-Hsiung (1)
Zhang, Zhong J. (1)
Year of Publication
Familial Aggregation of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia with Solid Tumors and Myeloid Malignancies
Kristinsson, Sigurdur Y.
Goldin, Lynn R.
Lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) is a B-cell disorder resulting from the accumulation, predominantly in the bone marrow, of clonally related lymphoplasmacytic cells. LPL/WM is a very rare disease, with an incidence rate of 3–4 cases per million people per year. Currently, the causes of LPL/WM are poorly understood; however, there are emerging data to support a role for immune-related factors in the pathogenesis of LPL/WM. In addition, data show that genetic factors are of importance in the etiology of LPL/WM. In this paper, we will review the current knowledge about familiality of LPL/WM and provide novel data on solid tumors and myeloid malignancies in first-degree relatives of LPL/WM patients.
Autoimmunity; Familial aggregation; Hematological malignancies; Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia; Monoclonal gammopathy of undetermined significance; Solid tumors; Susceptibility
Disseminated Strongyloides stercoralis Infection in HTLV-1-Associated Adult T-Cell Leukemia/Lymphoma
Stewart, Donn M.
Fedorko, Daniel P.
Janik, John E.
Morris, John C.
A 55-year-old woman with human T-cell lymphotropic virus type-1 (HTLV-1)-associated adult T-cell leukemia (ATL) and a history of previously treated Strongyloides stercoralis infection received anti-CD52 monoclonal antibody therapy with alemtuzumab on a clinical trial. After an initial response, she developed ocular involvement by ATL. Alemtuzumab was stopped and high-dose corticosteroid therapy was started to palliate her ocular symptoms. Ten days later, the patient developed diarrhea, vomiting, fever, cough, skin rash, and a deteriorating mental status. She was diagnosed with disseminated S. stercoralis. Corticosteroids were discontinued and the patient received anthelmintic therapy with ivermectin and albendazole with complete clinical recovery.
Adult T-cell leukemia; Alemtuzumab; Corticosteroid; Disseminated Strongyloides; HTLV-1; Human T-cell lymphotropic virus type-1
Plasma Glial Fibrillary Acidic Protein Levels in a Child with Sickle Cell Disease and Stroke
Savage, William J.
Everett, Allen D.
Casella, James F.
A 12-year-old boy with HbSS sickle cell disease (SCD) was admitted with an acute febrile illness and developed overt stroke 3 days later. Plasma glial fibrillary acidic protein levels were elevated, as compared to pediatric controls, 32 h prior to the clinical diagnosis of stroke, peaked immediately prior to the exchange transfusion, and remained elevated 1 year later despite chronic transfusion therapy. Stroke in SCD can occur in the setting of acute illness, and a biomarker that could predict the onset and triage ill children to therapeutic intervention more quickly would be useful.
Chronic transfusion; Glial fibrillary acidic protein; Sickle cell; Stroke
MicroRNA Profiles of Drug-Resistant Myeloma Cell Lines
The Role of Hepcidin in Iron Metabolism
Hepcidin is the central regulator of systemic iron homeostasis. Dysregulation of hepcidin production results in a variety of iron disorders. Hepcidin deficiency is the cause of iron overload in hereditary hemochromatosis, iron-loading anemias, and hepatitis C. Hepcidin excess is associated with anemia of inflammation, chronic kidney disease and iron-refractory iron deficiency anemia. Diagnostic and therapeutic applications of this new knowledge are beginning to emerge. Dr. Ernest Beutler played a significant role in advancing our understanding of the function of hepcidin. This review is dedicated to his memory.
Anemia of inflammation; Bone morphogenetic protein; Hemochromatosis; Hepcidin; Iron-loading anemia
Role of Matriptase-2 (TMPRSS6) in Iron Metabolism
Iron, an essential element for life, is regulated primarily at the level of uptake, storage, and transport in order to maintain sufficient availability for normal physiology. The key protein in iron homeostasis is a 25-amino-acid peptide, hepcidin, which modulates the amount of iron in the circulation by binding and promoting the degradation of the iron exporter ferroportin. Given the central importance of hepcidin, recent studies have focused on how iron is sensed and how the iron signal is transmitted to hepcidin. Mutations in a type II serine protease, matriptase-2/TMPRSS6, were recently identified to be associated with severe iron deficiency caused by inappropriately high levels of hepcidin expression. A key biologically relevant substrate for the proteolytic activity of matriptase-2/TMPRSS6 was found to be hemojuvelin, a cell surface protein that regulates hepcidin expression through a BMP/SMAD pathway. In this review, we discuss the putative role of matriptase-2/TMPRSS6 in iron homeostasis.
CUB; Hemojuvelin; Hepcidin; Iron; LDLa; Matriptase; TMPRSS; Type II serine protease
BCR-ABL Truncation due to Premature Translation Termination as a Mechanism of Resistance to Kinase Inhibitors
Zhang, Zhong J.
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