bone marrow failure syndromes ppt

... cooperate in a repair complex to maintain telomere length. In Shwachman-Diamond syndrome, marrow failure is seen with pancreatic insufficiency and malabsorption; most patients have compound ... thrombotic episodes occur in patients with large PNH clones (>50%). Functional studies of bone marrow from PNH patients, even those with mainly hemolytic manifestations, show evidence of ... an initial clinical diagnosis of PNH, especially younger individuals, may later develop frank marrow aplasia and pancytopenia; patients with an initial diagnosis of aplastic anemia may suffer...

Ngày tải lên: 07/07/2014, 04:20

... history of hematologic diseases or blood abnormalities may indicate a constitutional etiology of marrow failure. Physical Examination Petechiae and ecchymoses are typical, and retinal hemorrhages ... expression on CD34 cells, leading to apoptotic cell death; localization of activated T cells to bone marrow and local production of their soluble factors are probably important in stem cell destruction. ... presence of immature myeloid forms suggests leukemia or MDS; nucleated red blood cells suggest marrow fibrosis or tumor invasion; abnormal platelets suggest either peripheral destruction or...

Ngày tải lên: 07/07/2014, 04:20

Ngày tải lên: 07/07/2014, 04:20

... Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 1) Harrison's Internal Medicine > Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes Aplastic ... leukemia (AML) Some acute lymphoid leukemia Some lymphomas of bone marrow Pancytopenia with Cellular Bone Marrow Primary bone marrow diseases Myelodysplasia Paroxysmal nocturnal Secondary ... leukopenias). Hematopoietic failure syndromes are classified by dominant morphologic features of the bone marrow (Table 102-1). While practical distinction among these syndromes usually is clear,...

Ngày tải lên: 07/07/2014, 04:20

... Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 2) Aplastic Anemia Definition Aplastic anemia is pancytopenia with bone marrow hypocellularity. Acquired aplastic ... bone marrow hypocellularity. Acquired aplastic anemia is distinguished from iatrogenic marrow aplasia, marrow hypocellularity after intensive cytotoxic chemotherapy for cancer. Aplastic anemia ... typical physical anomalies and the development of pancytopenia early in life, can also present as marrow failure in normal-appearing adults. Acquired aplastic anemia is often stereotypical in its...

Ngày tải lên: 07/07/2014, 04:20

... Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 3) Radiation Marrow aplasia is a major acute sequela of radiation. Radiation damages ... a notorious 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. ... environment. The association 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;...

Ngày tải lên: 07/07/2014, 04:20

... Related Bone Marrow Failure Syndromes (Part 5) Pathophysiology Bone marrow failure results from severe damage to the hematopoietic cell compartment. In aplastic anemia, replacement of the bone ... responsible for benzene-induced tissue injury. Excessive generation of toxic intermediates or failure to detoxify the intermediates may be genetically determined and apparent only on specific ... severe damage to the hematopoietic cell compartment. In aplastic anemia, replacement of the bone marrow by fat is apparent in the morphology of the biopsy specimen (Fig. 102-1) and MRI of the...

Ngày tải lên: 07/07/2014, 04:20

... anemia. Genetic analysis 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 ... patients with bone marrow hypocellularity have depression of only one or two of three blood lines, sometimes showing later progression to more recognizable aplastic anemia. The bone marrow in constitutional ... ameliorated by suppression of the immune system to allow recovery of the patient's residual bone marrow function. Hematopoietic growth factors have limited usefulness and glucocorticoids are...

Ngày tải lên: 07/07/2014, 04:20

... Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 8) Hematopoietic Stem Cell Transplantation This is the best therapy ... suitable marrow donor, and immunosuppression is the treatment of choice. Overall survival is equivalent with transplantation and immunosuppression. However, successful transplant cures marrow failure, ... patients continue to have some degree of blood count depression, the MCV remains elevated, and the bone marrow cellularity returns toward normal only very slowly, if at all. Relapse (recurrent pancytopenia)...

Ngày tải lên: 07/07/2014, 04:20

... respond to immunosuppressive Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 9) Outcomes following both transplant and immunosuppression have improved ... Cell Aplasia Other, more restricted forms of marrow failure occur, in which only a single circulating cell type is affected and the aregenerative marrow shows corresponding absence or decreased ... regimen of 2 units every 2 weeks will replace normal losses in a patient without a functioning bone marrow. In chronic anemia, the iron chelators deferoxamine and deferasirox should be added at...

Ngày tải lên: 07/07/2014, 04:20

... Related Bone Marrow Failure Syndromes (Part 10) Definition and Differential Diagnosis PRCA is characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone ... characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow. The classification of PRCA is shown in Table 102-4. In adults, PRCA is acquired. An identical ... patients have etiologic mutations in a ribosomal RNA processing gene called RPS19. Temporary red cell failure occurs in transient aplastic crisis of hemolytic anemias due to acute parvovirus infection...

Ngày tải lên: 07/07/2014, 04:20

... preparation made from the marrow biopsy of a patient with metastatic carcinoma. Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 11) Persistent ... mediated by immune complex deposition. Figure 102-2 Pathognomonic cells in marrow failure syndromes. A. Giant pron ormoblast, the cytopathic effect of B19 parvovirus infection ... congenital, acquired, or iatrogenic immunodeficiency, persistent viral infection may occur. The bone marrow shows red cell aplasia and the presence of giant pronormoblasts (Fig. 102-2), which is...

Ngày tải lên: 07/07/2014, 04:20

... 102- 5 World Health Organization Classification of Myelodysplastic Syndromes Disease Freque ncy Blood Findings Bone Marrow Findings Prognos is Refractory anemia (RA) 5–10% Anemia ... characterized by cytopenias associated with a dysmorphic (or abnormal appearing) and usually cellular bone marrow, and by consequent ineffective blood cell production. A clinically useful nosology of ... hypolobated nuclei <5% blasts No Auer rods Isolated del(5q) Long survival Note: BM, bone marrow. Source: Extracted from Jaffe ES et al (eds): Pathology and Genetics of Tumors of Haematopoietic...

Ngày tải lên: 07/07/2014, 04:20

Ngày tải lên: 07/07/2014, 04:20

Ngày tải lên: 07/07/2014, 04:20

Ngày tải lên: 10/08/2014, 09:22

... resurrection after bone- marrow- derived Anastasiadis et al. Journal of Translational Medicine 2011, 9:12 http://www.translational-medicine.com/content/9/1/12 Page 4 of 5 Chronic ischemic heart failure epidemic. ... al.: Hybrid approach of ventricular assist device and autologous bone marrow stem cells implantation in end-stage ischemic heart failure enhances myocardial reperfusion. Journal of Translational ... Cardiol 2002, 39:1189-1198. 8. McMurray JV, Pfeffer MA: Heart failure. Lancet 2005, 365:1877-1889. 9. Gheorghiade M, Bonow RO: Chronic heart failure in the United States: a manifestation of coronary...

Ngày tải lên: 18/06/2014, 16:20

... experimental metastasis to bone and bone marrow. Cancer Res 1988, 48:6876-6881. 41. Argüello F, Baggs RB, Graves BT, Harwell SE, Cohen HJ, Frantz CN: Effect of IL-1 on experimental bone/ bone marrow metastases. ... as previously described [30]. For human BMSC isolation, bone marrow aspirates were obtained from patients under going bone marrow harvest for autologous bone marrow transplantation, after informed c onsent. ... in the prevention a nd treatment of bone metastases. List of abbreviations The abbreviations used are: COX-2: cyclooxygenase-2; BM: bone marrow; BMSC: bone marrow stromal cell; CM: conditioned...

Ngày tải lên: 18/06/2014, 22:20

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