bone marrow transplantation for inherited bone marrow failure syndromes
Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 1) ppsx
... aleukemic leukemia (AML) Some acute lymphoid leukemia Some lymphomas of bone marrow Pancytopenia with Cellular Bone Marrow Primary bone marrow diseases Secondary to systemic diseases Myelodysplasia Systemic ... 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, ... Myelophthisis Overwhelming infection Bone marrow lymphoma Alcohol Hairy cell leukemia Brucellosis Sarcoidosis Tuberculosis Leishmaniasis Hypocellular Bone Marrow ± Cytopenia Q fever Legionnaires'...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 2) ppsx
... aplastic anemia have been inferred from several recurring clinical associations (Table 102-2); unfortunately, these relationships are not reliable in an individual patient and may not be etiologic ... as a drug exposure Table 102-2 Classification of Aplastic Anemia and Single Cytopenias Acquired Inherited Aplastic Anemia Secondary Fanconi's anemia Radiation Dyskeratosis congenita Drugs and...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 3) pdf
... is 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 The ... necessarily reflect causation: a drug may have been used to treat the first symptoms of bone marrow failure (antibiotics for fever or the preceding viral illness) or provoked the first symptom of a preexisting ... 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;...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 4) pptx
... unproven explanation for the aplastic anemia/PNH syndrome is selection of the deficient clones because they are favored for proliferation in the peculiar environment of immune-mediated marrow destruction ... and 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 defective ... products 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...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 5) pdf
... A Normal bone marrow biopsy B Normal bone marrow aspirate smear The marrow is normally 30–70% cellular, and there is a heterogeneous mix ... 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 cell defect exists for the constitutional ... cellular macromolecules For example, derivative hydroquinones and quinolones are responsible for benzene-induced tissue injury Excessive generation of toxic intermediates or failure to detoxify the...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 6) ppt
... 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, when performed, ... 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 ... numbers may be normal or reduced The presence of immature myeloid forms suggests leukemia or MDS; nucleated red blood cells suggest marrow fibrosis or tumor invasion; abnormal platelets suggest either...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 7) docx
... 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 ... Diagnosis The diagnosis of aplastic anemia is usually straightforward, based on the combination of pancytopenia with a fatty, empty bone marrow Aplastic anemia is a disease of the young and should...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 8) potx
... hemolysis if the PNH clone expands Bone marrow examinations should be performed if there is an unfavorable change in blood counts Horse ATG is given at 40 mg/kg per day for days; rabbit ALG is administered ... suitable marrow donor, and immunosuppression is the treatment of choice Overall survival is equivalent with transplantation and immunosuppression However, successful transplant cures marrow failure, ... rates to about 70% and especially improved outcomes for children and for severely neutropenic patients Such combined treatment is now standard for patients with severe disease An early robust hematologic...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 9) docx
... Red 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 ... a regimen of units every 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 ... antibodies or lymphocytes and can respond to immunosuppressive therapies In all the single lineage failure syndromes, progression to pancytopenia or leukemia is unusual ...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 10) pot
... immune mechanism Cytotoxic lymphocyte activity restricted by histocompatibility locus or specific for human T cell leukemia/lymphoma virus I–infected cells, as well as natural killer cell activity...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 11) docx
... symptoms are mediated by immune complex deposition Figure 102-2 Pathognomonic cells in marrow failure syndromes A Giant pronormoblast, the cytopathic effect of B19 parvovirus infection of the ... sideroblast showing perinuclear iron granules D Tumor cells present on a touch preparation made from the marrow biopsy of a patient with metastatic carcinoma ...
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 12) pot
... Table 102-5 World Health Organization Classification of Myelodysplastic Syndromes Disease Freque ncy Blood Bone Findings Marrow Prognos is Findings Refractory 5–10% Anemia anemia (RA) Erythro ... 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 these ... Platelet hypolobated s nl or nuclei increased
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Chapter 102. Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes (Part 13) doc
... reported in some patients but likely occur late in the sequence leading to leukemic transformation Apoptosis of marrow cells is increased in MDS, presumably due to these acquired genetic alterations ... MDS, and a family history may indicate a hereditary form of sideroblastic anemia or Fanconi's anemia The physical examination is remarkable for signs of anemia; about 20% of patients have splenomegaly ... functionally deficient Circulating myeloblasts usually correlate with marrow blast numbers, and their quantitation is important for classification and prognosis The total white blood cell count is...
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