myelodysplasia bone marrow failure syndromes and other causes of pancytopenia

... acute lymphoid leukemia Some lymphomas of bone marrow Pancytopenia with Cellular Bone Marrow Primary bone marrow diseases Secondary to systemic diseases Myelodysplasia Systemic Paroxysmal nocturnal ... appear to evolve into another Many of these syndromes share an immune-mediated mechanism of marrow destruction and some element of genomic instability resulting in a higher rate of malignant transformation ... Differential Diagnosis of Pancytopenia Pancytopenia with Hypocellular Bone Marrow Acquired aplastic anemia Constitutional aplastic anemia (Fanconi's anemia, dyskeratosis congenita) Some myelodysplasia...

Ngày tải lên: 07/07/2014, 04:20

... hemoglobinuria (PNH; Chap 101) and to MDS, and in some cases a clear distinction among these disorders may not be possible Epidemiology The incidence of acquired aplastic anemia in Europe and Israel is two ... Israel is two cases per million persons annually In Thailand and China, rates of five to seven per million have been established In general, men and women are affected with equal frequency, but the ... age distribution is biphasic, with the major peak in the teens and twenties and a second rise in the elderly Etiology The origins of aplastic anemia have been inferred from several recurring clinical...

Ngày tải lên: 07/07/2014, 04:20

... cause of bone marrow failure Vast quantities of epidemiologic, clinical, and laboratory data link benzene to aplastic anemia, acute leukemia, and blood and marrow abnormalities The occurrence of ... between marrow failure and other chemicals is much less well substantiated Drugs (Table 102-3) Many chemotherapeutic drugs have marrow suppression as a major toxicity; effects are dose-dependent and ... translates, in a rare disease, to but a handful of drug-induced aplastic anemia cases among hundreds of thousands of exposed persons Table 102-3 Some Drugs and Chemicals Associated with Aplastic...

Ngày tải lên: 07/07/2014, 04:20

... time of presentation [and PNH cells are also seen in MDS (see below)]; frank hemolysis and thrombotic episodes occur in patients with large PNH clones (>50%) Functional studies of bone marrow ... manifestations, show evidence of defective hematopoiesis Patients with an initial clinical diagnosis of PNH, especially younger individuals, may later develop frank marrow aplasia and pancytopenia; patients ... painful induration of subcutaneous tissues (Chap 316) Pancytopenia with marrow hypoplasia can also occur in systemic lupus erythematosus Pregnancy Aplastic anemia very rarely may occur and recur during...

Ngày tải lên: 07/07/2014, 04:20

... A Normal bone marrow biopsy B Normal bone marrow aspirate smear The marrow is normally 30–70% cellular, and there is a heterogeneous mix of myeloid, erythroid, and lymphoid cells C ... lymphoid cells C Aplastic anemia biopsy D Marrow smear in aplastic anemia The marrow shows replacement of hematopoietic tissue by fat and only residual stromal and lymphoid cells An intrinsic stem ... damage and death on exposure to certain chemical agents Telomeres are short in a large proportion of patients with aplastic anemia, and mutations in genes of the telomere repair complex (TERC and...

Ngày tải lên: 07/07/2014, 04:20

... constitutional etiology of marrow failure Physical Examination Petechiae and ecchymoses are typical, and retinal hemorrhages may be present Pelvic and rectal examinations can often be deferred but, ... feature of aplastic anemia is the restriction of symptoms to the hematologic system, and patients often feel and look remarkably well despite drastically reduced blood counts Systemic complaints and ... should point to other etiologies of pancytopenia Prior drug use, chemical exposure, and preceding viral illnesses must often be elicited with repeated questioning A family history of hematologic...

Ngày tải lên: 07/07/2014, 04:20

... combination of pancytopenia with a fatty, empty bone marrow Aplastic anemia is a disease of the young and should be a leading diagnosis in the pancytopenic adolescent or young adult When pancytopenia ... applicable to the constitutional marrow failure states is available in some laboratories Chromosome studies of bone marrow cells are often revealing in MDS and should be negative in typical aplastic ... atypical presentations and among related hematologic diseases While pancytopenia is most common, some patients with bone marrow hypocellularity have depression of only one or two of three blood lines,...

Ngày tải lên: 07/07/2014, 04:20

... Development of MDS, with typical marrow morphologic or cytogenetic abnormalities, occurs in about 15% of treated patients, usually but not invariably associated with a return of pancytopenia, and some ... laboratory diagnosis of PNH can generally be made at the time of presentation of aplastic anemia by flow cytometry; recovered patients may have frank hemolysis if the PNH clone expands Bone marrow examinations ... suitable marrow donor, and immunosuppression is the treatment of choice Overall survival is equivalent with transplantation and immunosuppression However, successful transplant cures marrow failure, ...

Ngày tải lên: 07/07/2014, 04:20

... reasonable existence in the face of pancytopenia First and most important, infection in the presence of severe neutropenia must be aggressively treated by prompt institution of parenteral, broad-spectrum ... ceftazidime or a combination of an aminoglycoside, cephalosporin, and semisynthetic penicillin Therapy is empirical and must not await results of culture, although specific foci of infection such as ... fungal disease: Candida and Aspergillus are common, especially after several courses of antibacterial antibiotics, and a progressive course may be averted by timely initiation of antifungal therapy...

Ngày tải lên: 07/07/2014, 04:20

... (Diamond-Blackfan syndrome) Acquired pure red cell aplasia Thymoma and malignancy Thymoma Lymphoid malignancies (and more rarely other hematologic diseases) Paraneoplastic to solid tumors Connective ... Associations and Etiology PRCA has important associations with immune system diseases A small minority of cases occur with a thymoma More frequently, red cell aplasia can be the major manifestation of ... Self-limited Transient erythroblastopenia of childhood Transient aplastic crisis of hemolysis (acute B19 parvovirus infection) Fetal red blood cell aplasia Nonimmune...

Ngày tải lên: 07/07/2014, 04:20

... cessation of red cell production is not clinically apparent, and skin and joint symptoms are mediated by immune complex deposition Figure 102-2 Pathognomonic cells in marrow failure syndromes ... pronormoblast, the cytopathic effect of B19 parvovirus infection of the erythroid progenitor cell B Uninuclear megakaryocyte and microblastic erythroid precursors typical of the 5q– myelodysplasia syndrome ... showing perinuclear iron granules D Tumor cells present on a touch preparation made from the marrow biopsy of a patient with metastatic carcinoma ...

Ngày tải lên: 07/07/2014, 04:20

... blasts No Auer rods Isolated del(5q) Note: BM, bone marrow Source: Extracted from Jaffe ES et al (eds): Pathology and Genetics of Tumors of Haematopoietic and Lymphoid Tissues Lyon, IARC Press, 2001 ... with a dysmorphic (or abnormal appearing) and usually cellular bone marrow, and by consequent ineffective blood cell production A clinically useful nosology of these entities was first developed ... should be treated with immunosuppression and withdrawal of erythropoietin Myelodysplasia Definition The myelodysplasias (MDSs) are a heterogeneous group of hematologic disorders broadly characterized...

Ngày tải lên: 07/07/2014, 04:20

... abnormalities are not random (loss of all or part of 5, 7, and 20, trisomy of 8) and may be related to etiology (11q23 following topoisomerase II inhibitors); chronic myelomonocytic leukemia is often associated ... tel-PDGFβ gene The type and number of cytogenetic abnormalities strongly correlate with the probability of leukemic transformation and survival Mutations of N-ras (an oncogene), p53 and IRF-1 (tumor ... as radiation and benzene; other risk factors have been reported inconsistently Secondary MDS occurs as a late toxicity of cancer treatment, usually with a combination of radiation and the radiomimetic...

Ngày tải lên: 07/07/2014, 04:20

... of pancytopenia and not due to leukemic transformation; perhaps one-third will succumb to other diseases unrelated to their MDS Precipitous worsening of pancytopenia, acquisition of new chromosomal ... severe pancytopenia associated with monosomy 7; an International Prognostic Scoring System (Table 102-6) assists in making predictions Most patients die as a result of complications of pancytopenia ... Deficiencies of vitamin B12 or folate should be excluded by appropriate blood tests; vitamin B6 deficiency can be assessed by a therapeutic trial of pyridoxine if the bone marrow shows ringed...

Ngày tải lên: 07/07/2014, 04:20

... Current status of allogeneic bone marrow transplantation in acquired aplastic anemia Semin Hematol 37:30, 2000 [PMID: 10676909] Molldrem J et al: Treatment of bone marrow failure of myelodysplastic ... [PMID: 12011120] Young NS (ed): Bone Marrow Failure Syndromes Philadelphia, Saunders, 2000 ———, Calado RT: Telomere repair complex gene mutations in bone marrow failure syndromes Blood In preparation, ... features: proliferation of fibroblasts in the marrow space (myelofibrosis); the extension of hematopoiesis into the long bones and into extramedullary sites, usually the spleen, liver, and lymph nodes...

Ngày tải lên: 07/07/2014, 04:20

... biomaterials of bone graft is frequently neglected Therefore, the objective of this study was to evaluate the biocompatibility of the HA-PCL biomaterials to both bone marrow- derived osteogenic and endothelial ... of PCLHA scaffolds with bone marrow- derived endothelial cells, Page of (page number not for citation purposes) Journal of Orthopaedic Surgery and Research 2009, 4:5 Figure Characterizations of ... capability of selfrenewal and differentiation into organ-specific cell types [20] Our results indicated that MSCs and EPCs could be harvested from mice bone marrow and differentiate into osteoblasts and...

Ngày tải lên: 20/06/2014, 01:20

... region corresponds to strains of 3% to 8% In the fourth part of the curve (Rregion IV), macroscopic failure occurs because of the tensile failure of the fibers and shear failure between fibers (O’Brien ... “application of the principles and methods of engineering and life sciences toward fundamental understanding of structure-function relationship in normal and pathological mammalian tissues and the ... regeneration of tendon and nerve tissues BECON 2001 Repair Medicine: Growth Tissue and Organs, Jun 25-26, 2001, NIH, Maryland, USA Goh JCH, Ouyang HW, Mo XM, Teoh SH, Lee EH The efficacy of bone marrow...

Ngày tải lên: 17/09/2015, 17:18

... workers (OR=3.6 and 4.7 for 110 years and greater than 10 years of employment respectively), and for material handling and related equipment operators, dockers and freighthandlers (OR=3.9 and 8.1 for ... paper and wood pulp Any one of several types of particles and rays given off by radioactive material, high-voltage equipment, nuclear reactions and stars Alpha and beta particles, X-rays and gamma ... acid manufacture, Australian and New Zealand Journal of Public Health 2007; 31:13-18 69 Baker PJ and Hoel D Meta-analysis of standardized incidence and mortality rates of childhood leukaemia in...

Ngày tải lên: 14/02/2014, 21:20

... old, the rich and poor, men, women, and children “ Cancer represents a tremendous burden on patients, families, and societies It is one of the leading causes of death in the world and is still ... Physical activity, avoidance of overweight and frequent daily intake of fresh fruits and vegetables reduce the risk of breast, colon, oral cavity, lung, cervix, and other cancers Women Men WHO ... health-care systems and laws and regulations permit the use of opioids for medical purposes, and to develop plans of action, with a view to facilitating the supply and avaibility of narcotic drugs...

Ngày tải lên: 15/02/2014, 05:20

... security is offered and sold and/ or the issuer is located, the availability and nature of relevant public information, access to the management of the issuer and its advisers, the availability of pre-existing ... Definitions of Ratings and Other Forms of Opinion – Nov 2012 22 A.3 Other International Credit Ratings A.3.1 Bank Support and Viability Ratings A.3.1.1 Support Ratings The Purpose and Function of Support ... servicers of commercial and residential mortgages and other asset types Asset manager ratings opine on the relative operational and financial capabilities of asset managers, trustees and others...

Ngày tải lên: 16/02/2014, 03:20