systemic sclerosis and related syndromes

... patients with systemic sclerosis Disease characteristic N = 49 Systemic sclerosis subset, number (percentage) Diffuse cutaneous systemic sclerosis (8%) Limited cutaneous systemic sclerosis 45 ... using IMT of the carotid artery as a marker of early atherosclerosis Lekakis and colleagues [22], Kaloudi and colleagues [23], and Bartoli and colleagues [24,25] found strongly increased IMT values ... Boxes indicate the median value and the markers interquartile ranges Lines indicate the minimum and maximum values Dotted bars represent systemic sclerosis patients and open bars represent controls...

Ngày tải lên: 09/08/2014, 10:23

Ngày tải lên: 26/05/2016, 13:15

... 102-1) While practical distinction among these syndromes usually is clear, they can occur secondary to other diseases, and some processes are so closely related that the diagnosis may be complex Patients ... two or three related diseases simultaneously, or one diagnosis may appear to evolve into another Many of these syndromes share an immune-mediated mechanism of marrow destruction and some element ... (idiopathic thrombocytopenic purpura or due to splenomegaly), and granulocytes (as in the immune leukopenias) Hematopoietic failure syndromes are classified by dominant morphologic features of...

Ngày tải lên: 07/07/2014, 04:20

... aplastic anemia in Europe and 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 ... related to both paroxysmal nocturnal hemoglobinuria (PNH; Chap 101) and to MDS, and in some cases a clear distinction among these disorders ... 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...

Ngày tải lên: 07/07/2014, 04:20

... epidemiologic, clinical, and laboratory data link benzene to aplastic anemia, acute leukemia, and blood and marrow abnormalities The occurrence of leukemia is roughly correlated with cumulative ... 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 ... suppression as a major toxicity; effects are dose-dependent and will occur in all recipients In contrast, idiosyncratic reactions to a large and diverse group of drugs may lead to aplastic anemia...

Ngày tải lên: 07/07/2014, 04:20

... Pancytopenia with marrow hypoplasia can also occur in systemic lupus erythematosus Pregnancy Aplastic anemia very rarely may occur and recur during pregnancy and resolve with delivery or with spontaneous ... monoubiquitination to play a role in the cellular response to DNA damage and especially interstrand cross-linking, a response that includes BRCA1, ATM, and NBS1 Dyskeratosis congenita is characterized by mucous ... Patients with Fanconi's anemia typically have short stature, café au lait spots, and anomalies involving the thumb, radius, and genitourinary tract At least 12 different genetic defects (all but one...

Ngày tải lên: 07/07/2014, 04:20

... 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 ... 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 aplastic ... bone marrow aspirate smear The marrow is normally 30–70% cellular, and there is a heterogeneous mix of myeloid, erythroid, and lymphoid cells C Aplastic anemia biopsy D Marrow smear in aplastic...

Ngày tải lên: 07/07/2014, 04:20

... restriction of symptoms to the hematologic system, and patients often feel and look remarkably well despite drastically reduced blood counts Systemic complaints and weight loss should point to other etiologies ... anemia patients and usually decline with successful immunosuppressive therapy; cytokine measurements show a T H1 immune response (interferon γ and tumor necrosis factor) Interferon and tumor necrosis ... 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,...

Ngày tải lên: 07/07/2014, 04:20

... metastatic cancer or systemic lupus erythematosus, or miliary tuberculosis on chest radiograph (Table 102-1) Diagnostic problems can occur with atypical presentations and among related hematologic ... of megakaryocytes and myeloid precursor cells, and typical cytogenetic abnormalities (see below) Prognosis The natural history of severe aplastic anemia is rapid deterioration and death Provision ... aplastic anemia is rapid deterioration and death Provision first of red blood cell and later of platelet transfusions and effective antibiotics are of some benefit, but few patients show spontaneous...

Ngày tải lên: 07/07/2014, 04:20

... relapse and malignant evolution Because of excellent results in children and younger adults, allogeneic transplant should be performed if a suitable sibling donor is available Increasing age and ... conventional sibling transplants but improving with higher-resolution HLA matching and more effective conditioning regimens and GVHD prophylaxis Patients will be at risk for late complications, especially ... transfusion and a leukocyte count adequate to prevent infection) in about 50% of patients The addition of cyclosporine to either ALG or ATG has further increased response rates to about 70% and especially...

Ngày tải lên: 07/07/2014, 04:20

... (Chap 109), and neutropenia without marrow myeloid cells in agranulocytosis (Chap 61) In general, and in contrast to aplastic anemia and MDS, the unaffected lineages appear quantitatively and qualitatively ... or recrudescent fever implies fungal disease: Candida and Aspergillus are common, especially after several courses of antibacterial antibiotics, and a progressive course may be averted by timely ... incriminating drug exposure) and amegakaryocytic thrombocytopenia are exceedingly rare and, like PRCA, appear to be due to destructive antibodies or lymphocytes and can respond to immunosuppressive...

Ngày tải lên: 07/07/2014, 04:20

... Thymoma and malignancy Thymoma Lymphoid malignancies (and more rarely other hematologic diseases) Paraneoplastic to solid tumors Connective tissue disorders with immunologic abnormalities Systemic ... Systemic lupus erythematosus, juvenile rheumatoid arthritis, rheumatoid arthritis Multiple endocrine gland insufficiency Virus Persistent B19 parvovirus, hepatitis, adult T cell leukemia virus, EpsteinBarr ... azathioprine, chloramphenicol, procainamide, isoniazid Erythropoietin Idiopathic Clinical Associations and Etiology PRCA has important associations with immune system diseases A small minority of cases...

Ngày tải lên: 07/07/2014, 04:20

... 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 A Giant pronormoblast, the ... effect of B19 parvovirus infection of the erythroid progenitor cell B Uninuclear megakaryocyte and microblastic erythroid precursors typical of the 5q– myelodysplasia syndrome C Ringed sideroblast...

Ngày tải lên: 07/07/2014, 04:20

... (CMML) The (RAEB-t), World Health and chronic Organization classification (2002) recognizes that the distinction between RAEB-t and acute myeloid leukemia is arbitrary and groups them together as acute ... 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 ... cyclophosphamide, and the monoclonal antibodydaclizumab, an antibody to the IL-2 receptor PRCA developing on erythropoietin therapy should be treated with immunosuppression and withdrawal of...

Ngày tải lên: 07/07/2014, 04:20

... other harmful alterations Cytogenetic 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); ... 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 ... symptomatic patients complain of the gradual onset of fatigue and weakness, dyspnea, and pallor, but at least half the patients are asymptomatic and their MDS is discovered only incidentally on routine...

Ngày tải lên: 07/07/2014, 04:20

... cytogenetic determination, and increase in the number of blasts are all poor prognostic indicators The outlook in therapy -related MDS, regardless of type, is very poor, and most patients will progress ... patients die as a result of complications of pancytopenia and not due to leukemic transformation; perhaps one-third will succumb to other diseases unrelated to their MDS Precipitous worsening of pancytopenia, ... transient More difficult are the distinctions between hypocellular MDS and aplasia or between refractory anemia with excess blasts and early acute leukemia The World Health Organization considers the...

Ngày tải lên: 07/07/2014, 04:20

... (both Mycobacterium tuberculosis and M avium), fungi, or HIV, and in sarcoidosis Intracellular lipid deposition in Gaucher disease and obliteration of the marrow space related to absence of osteoclast ... myelosuppression (worsening thrombocytopenia and neutropenia, necessitating blood count monitoring) and an increased risk of deep vein thrombosis and pulmonary embolism Other treatments for MDS ... extension of hematopoiesis into the long bones and into extramedullary sites, usually the spleen, liver, and lymph nodes (myeloid metaplasia); and ineffective erythropoiesis The etiology of the...

Ngày tải lên: 07/07/2014, 04:20

... disease Sera from patients with diffuse systemic sclerosis (dSSc), limited systemic sclerosis (lSSc), overlap syndromes, and undifferentiated systemic sclerosis patients were analyzed by line ... the criteria of the EUSTAR and DNSS network [13,14] Briefly, diffuse SSc (dSSc) and limited SSc (lSSc) were defined according to LeRoy and colleagues [16] and the DNSS and EUSTAR criteria based ... for citation purposes) KH and AK helped to provide preclinical analyses, statistics, and graphics and to write the manuscript CD, AJ, LK, and WM helped to develop the LIA and to perform the tests...

Ngày tải lên: 09/08/2014, 01:22

... systemic sclerosis patients at baseline factor (VEGF) concentrations in systemic sclerosis patients at baseline (n = 40; r = 0.43; P = 0.007) R312 Individualand (n = 20) and patients with systemic sclerosis ... Figure baseline and median values of serum sVCAM-1 sclerosis (n = at trol subjects after treatment with 60 mg nifedipine per day (SSc)in conIndividualand (n = 20) and patients with systemic concentrations ... fibrinolysis, and markers of endothelial dysfunction in systemic sclerosis Semin Arthritis Rheum 2003, 32:285-295 Altman RD, Medsger TA Jr, Bloch DA, Michel BA: Predictors of survival in systemic sclerosis...

Ngày tải lên: 09/08/2014, 01:23

... levels of (a) endostatin and (b) basic fibroblast growth factor (bFGF) in patients with established systemic sclerosis (SSc) and in healthy controls Levels of endostatin and bFGF were not enhanced ... endostatin and bFGF and other clinical parameters No correlation of VEGF, endostatin and bFGF levels with skin score, carbon monoxide diffusion capacity and the presence of teleangiectasias and other ... Preliminary criteria for the classification of systemic sclerosis (scleroderma) Arthritis Rheum 1980, 23:581-590 Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy...

Ngày tải lên: 09/08/2014, 06:22