Topic
Pancytopenia
About: Pancytopenia is a research topic. Over the lifetime, 6151 publications have been published within this topic receiving 79948 citations. The topic is also known as: Pancytopenia (disorder) & Pancytopenia NOS.
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Washington University in St. Louis1, Northwestern University2, Mayo Clinic3, University of Pisa4, University of Melbourne5, Harvard University6, Stanford University7, Cornell University8, University of Nebraska Medical Center9, Memorial Sloan Kettering Cancer Center10, University of New Mexico11, University of Pennsylvania12, Tulane University13, National Institutes of Health14, United States Department of Veterans Affairs15
TL;DR: Overall, 52 patients with lymphoproliferative disorders, 2 patients with systemic lupus erythematosus, 1 patient with rheumatoid arthritis,1 patient with an idiopathic autoimmune pancytopenia, and 1 patientwith immune thrombocytopenIA developed PML after treatment with rituximab and other agents from 1997 to 2008.
818 citations
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TL;DR: In conclusion, awareness of the clinical symptoms and of the diagnostic criteria of HLH is crucial in order not to overlook HLH and to start life-saving therapy in time.
Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin and low fibrinogen. HLH occurs on the basis of various inherited or acquired immune deficiencies. Impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is shared by all forms of HLH. Genetic HLH occurs in familial forms (FHLH) in which HLH is the primary and only manifestation, and in association with the immune deficiencies Chediak-Higashi syndrome 1 (CHS 1), Griscelli syndrome 2 (GS 2) and x-linked lymphoproliferative syndrome (XLP), in which HLH is a sporadic event. Most patients with acquired HLH have no known underlying immune deficiency. Both acquired and genetic forms are triggered by infections, mostly viral, or other stimuli. HLH also occurs as a complication of rheumatic diseases (macrophage activation syndrome) and of malignancies. Several genetic defects causing FHLH have recently been discovered and have elucidated the pathophysiology of HLH. The immediate aim of therapy in genetic and acquired HLH is suppression of the severe hyperinflammation, which can be achieved with immunosuppressive/immunomodulatary agents and cytostatic drugs. Patients with genetic forms have to undergo stem cell transplantation to exchange the defective immune system with normally functioning immune effector cells. In conclusion, awareness of the clinical symptoms and of the diagnostic criteria of HLH is crucial in order not to overlook HLH and to start life-saving therapy in time.
654 citations
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01 Jan 1993
TL;DR: Two studies with mice, dogs, and monkeys established a “bone marrow syndrome” with death through the complications of pancytopenia after a total body dose of 5–7 Gy (500–700 rad), and were trailblazers for bone marrow transplantation as therapeutic modality in man.
Abstract: The development of nuclear arms at the end of World War II was a major stimulus for research into radiation damage to the hematopoietic system and into the treatment of such damage through bone marrow transplantation [1]. A large body of research with mice, dogs, and monkeys established a “bone marrow syndrome” with death through the complications of pancytopenia after a total body dose of 5–7 Gy (500–700 rad). Such animals could be salvaged by the infusion of syngeneic bone marrow [2] or allogeneic bone marrow [3,4]. These studies were trailblazers for bone marrow transplantation as therapeutic modality in man.
627 citations
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TL;DR: Insight is provided into the pathophysiology of HLH, new targets for specific therapeutic intervention in this fatal disorder are provided, and the excessive amount of IFNgamma seen in affected mice appears to be driven by increased antigen presentation to CD8+ T cells.
599 citations
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TL;DR: Characteristic laboratory findings in FHL are hypertriglyceridemia and hypofibrinogenemia, which are reversible with treatment, and Immunologic studies present evidence for a disturbed function of T lymphocytes, but a secondary immune defect seems to be more likely than primary immune deficiency.
Abstract: Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistocytic pleocytosis with increased protein levels in the cerebrospinal fluid. Characteristic laboratory findings in FHL are hypertriglyceridemia and hypofibrinogenemia, which are reversible with treatment. The disease has been rapidly fatal in most patients, but recently longterm remissions have been achieved with cytotoxic agents.
521 citations