Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Platelet crossmatching may provide a useful way of selecting donors for effective platelet transfusions in patients refractory to random donor platelet concentrates due to alloimmunization. We assessed the predictive value of a flow cytometric platelet immunofluorescence crossmatch test for the outcome of HLA matched platelet transfusions in a group of alloimmunized patients. Platelet immunofluorescence (PIFT) crossmatches were performed for 104 HLA-matched platelet transfusions administered to 30 patients. A negative PIFT crossmatch correctly predicted a successful platelet transfusion (1 h post-transfusion platelet recovery >20%) in 56/75 (75%) cases. We also considered non-immunological factors that, in combination with alloimmunization, might have contributed to an unsuccessful transfusion result, i.e. fever, septicaemia, splenomegaly, disseminated intravascular coagulation and bleeding. The predictive value of a negative PIFT crossmatch was better when these non-immunological factors were absent [48/59 (81%) correct predictions] than when these factors were present [8/16 (50%) correct predictions] (P=0.01; chi-square test). The effect of ABO incompatibility between donor and recipient on the predictive value of the PIFT crossmatch was also analysed. Positive PIFT crossmatches occurred more frequently in ABO incompatible donor-recipient combinations [in 18/28 (64%) cases] than in ABO-compatible donor-recipient combinations [in 11/76 cases (14%)] (P<0.001, chi-square test). Successful platelet transfusions were observed on 53/76 (70%) occasions in ABO compatible transfusions as compared to 16/28 (57%) in ABO incompatible transfusions. This difference was not statistically significant (P=0.23; chi-square test). Consequently, a negative PIFT crossmatch appeared to be non-predictive for the transfusion outcome in cases of ABO incompatibility between donor and recipient. We conclude that the PIFT crossmatch for platelet donor selection in addition to matching for HLA antigens, is predictive for the outcome of ABO compatible transfusions in alloimmunized recipients and prediction levels are increased when non-immunological causes for platelet refractoriness are absent.
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PMID:A flow cytometric platelet immunofluorescence crossmatch for predicting successful HLA matched platelet transfusions. 861 59

A 60-year-old Japanese woman was admitted to our hospital because of fatigue, weight loss and abdominal distension. Myelofibrosis was diagnosed, based on anemia, huge hepatosplenomegaly, leukoerythroblastosis and bone marrow fibrosis. Following treatment with ranimustine, anemia and splenomegaly improved. Seven months after initial therapy of ranimustine, however, polycythemia (RBC 7.39 x 10(6)/microliter; Hb 19.1 g/dl, Ht 65.9%) developed gradually, then RBC decreased to normal level following venesection (total 1,200 ml). After 32 months, blastic transformation occurred. The blasts were negative for myeloperoxidase. By flow cytometric analysis, the cells were positive for CD2, CD13, CD33 and HLA DR. Thus, AML (M0) was diagnosed. Despite of treatment with multicytotoxic agents, she died of DIC 36 months after the initial diagnosis of myelofibrosis. The progression from myelofibrosis to polycythemia is rare and only 15 cases have been reported so far. In addition, although a chromosomal abnormality, 46, XX, t(3; 12) (q25; p11), was present at the time of first diagnosis of myelofibrosis, the development of an additional abnormality, del(11) (q-), might be related to the transformation to AML.
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PMID:[A case of myelofibrosis that developed polycythemia vera following treatment with ranimustine and then acute myelogenous leukemia (M0)]. 882 83

A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.
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PMID:Mixed connective tissue disease associated with skin defects of livedoid vasculitis. 1105 28

We assessed the effect of rabbit antithymocyte globulin manufactured by Fresenius (ATG-F) on the hemostatic system in patients (n=12) with various hematological malignancies undergoing hematopoietic stem cell transplantation (HSCT) from HLA-matched unrelated donors. For this purpose, we monitored different parameters of coagulation before, during and after the administration of ATG-F. As a control group, we recruited patients (n=10) undergoing HSCT from their HLA-identical siblings who did not receive ATG-F as part of their preparative regimens. At 24 and 48 h after ATG-F treatment had been initiated, we found a temporary rise in D-Dimer, tissue factor, soluble thrombomodulin and thrombin-antithrombin III complex levels and a significant decrease of platelet counts in patients treated with ATG-F as compared to the control group. No differences between the two groups could be detected with regard to global coagulation tests as well as the incidence of bleeding manifestations, thromboembolic complications or the development of vascular-occlusive-disease of the liver. This temporary state of a stressed but compensated coagulation system under ATG-F therapy can be addressed as nonovert disseminated intravascular coagulation (DIC). The effect was independent from the different conditioning regimens and eased off after cessation of ATG-F. We conclude that ATG-F can induce nonovert DIC in patients receiving antithymocyte globulin as part of their conditioning regimen for HSCT.
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PMID:Non-overt disseminated intravascular coagulation in patients during treatment with antithymocyte globulin for unrelated allogeneic hematopoietic stem cell transplantation. 1273 91

Donor leukocyte infusion (DLI) is recognized as effective therapy for relapse after stem cell transplantation in patients with chronic myelogenous leukemia (CML). However, the clinical efficacy of DLI in the advanced phase of CML or other types of leukemia has not been clearly defined because of its varying degree of success. We describe a 22-year-old male patient with promyelocytic crisis of CML who had a relapse after peripheral blood stem cell transplantation, under reduced-intensity conditioning, from his HLA 2-antigen-mismatched mother. Complete hematologic remission was obtained after transplantation. However, a relapse that occurred on day 66 posttransplantion was characterized by an increase in number of leukemic promyelocytes with simultaneous exacerbation of disseminated intravascular coagulation (DIC). The patient received DLI containing 1 x 10(7)/kg CD3+ cells on day 73. Because rapid improvement of DIC paralleled the decrease in leukemic cells and because it was observed soon after DLI and before the development of acute graft-versus-host disease (GVHD), we hypothesized that leukemia-specific cells other than natural killer cells or cytotoxic T-cells unrelated to GVHD played a role in the graft-versus-leukemia effect observed in our patient. In addition, this may be the first report of effective correction of DIC by DLI after stem cell transplantation.
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PMID:Rapid improvement of disseminated intravascular coagulation by donor leukocyte infusions in a patient with promyelocytic crisis of chronic myelogenous leukemia after reduced-intensity stem cell transplantation from an HLA 2-antigen-mismatched mother. 1277 33

Streptococcal toxic shock syndrome (STSS) associated with a group A beta hemolytic streptococcal infection was described 18 y ago. Since then, although the pathophysiology of the syndrome has been clarified, mortality can be as high as 80%. A middle-aged female developed STSS associated with a group A streptococcal pneumonia. Laboratory studies confirmed respiratory and renal failure as well as disseminated intravascular coagulation with a striking reduction in endogenous procoagulants. The patient, probably due to her HLA DRB1*14 haplotype was unable to generate anti-streptococcal antibodies. She was treated with appropriate antimicrobial therapy together with intravenous gamma globulin and drotrecogin or activated protein C. Her response to this combined therapy was accompanied by a rapid resolution of the multiorgan failure and correction of the accompanying disseminated intravascular coagulation. This rapid response to treatment supports the hypohesis that several host factors including the immune response and loss of procoagulants determine the development and severity of the toxic shock syndromes. Further studies with this combined approach appear warranted.
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PMID:An early favorable outcome of streptococcal toxic shock syndrome may require a combination of antimicrobial and intravenous gamma globulin therapy together with activated protein C. 1714 61

A great variety of patient- and product-related factors influence the outcome of platelet transfusions. The patient-related factors are numerous, either physical factors as weight and height, or related to pathological being as splenomegaly, fever, infection, disseminated intravascular coagulation, previous HLA allo-immunization, or related to the treatment, as amphotericin. Major platelet factors that are associated with impaired responses are giving a decreased dose of platelets, ABO incompatible products, and platelets stored for more than 48 hours. When trying to prevent or to treat refractoriness and to finely tune platelet transfusions, all these factors have to be taken into account, and a good coordination between the blood bank and the clinician team is essential.
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PMID:[Factors affecting posttransfusion platelet efficiency "close relationship between patient and product"]. 1751 57

Familial hemophagocytic lymphohistiocytosis (FLH) is an autosomal recessively inherited multisystem disease. This defect in cellular cytotoxicity is a life threatening condition characterized by fever, rash, splenomegaly, cytopenias and neurologic manifestations. PRF1, UNC13D and STX11 gene defects underlie in about 40-50% of primary cases. Chemoimmunotherapy followed by hematopoietic stem cell transplantation improved disease outcome. We report a case of a 6-week-old boy who presented with a fever, diffuse rash, disseminated intravascular coagulation, hypofibrinogenemia, hypertrigliceridemia, hepatosplenomegaly, leukocytosis with 90% of lymphocytes, granulocytopenia, anemia, trombocytopenia, hyperferritinemia and pathological findings in cerebrospinal fluid. The patient had decreased frequency of NK cells and low NK cell activity in peripheral blood. Bone marrow aspiration analysis showed degenerative changes of histocyte cells, with preserved cytophages (lymphophages and erythrophages) consistent with hematophagocytic syndrome. Given that the molecular diagnosis of the known mutations in genes PRF1 and UNC13D showed a mutation in UNC13D, the diagnosis of familial hemophagocytic lymphohistiocytosis subtype 3 was established. HLH-2004 chemotherapy protocol was performed and partial remission with residual central nervous system disease was achieved. Hematopoietic stem cell transplantation was successfully performed with an unrelated HLA-matched donor. Familiar HLH is generally a progressive and fatal disease. Early diagnosis with molecular genetic analysis and chemoimmunotherapy followed by hematopoietic stem-cell transplantation is the best approach.
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PMID:Familial hemophagocytic lymphohistiocytosis in a 6-week-old male infant. 2069 42

The hantaviruses classified in Hantavirus genus of Bunyaviridae family, may cause two different types of clinical conditions, namely hemorrhagic fever with renal syndrome (HFRS) and hantavirus pulmonary syndrome (HPS). Mortality may reach up to 40% in these infections. Hantavirus subtypes (Sin Nombre, Hantaan, Seoul, Puumala, Dobrava, etc) with different virulences represent one of the most significant factors affecting the mortality. Additionally, many other factors including age, gender, humoral immune response, genetic factors, patient's clinical and laboratory findings, transfusion, mechanical ventilation requirement, antiviral treatment and immunotherapy administered to the patient are prognostically important. Increasing age had an unfavorable effect on mortality. While the disease is commonly observed in the male gender, mortality rate is higher in the female gender. The higher the emergent neutralizing antibody response, the virus spread, the number of the infected cells and the cytotoxic T lymphocyte-mediated injury will be lower. The requirement for dialysis is reported to be higher with a poorer prognosis in individuals with HLA-B8, -DR3, -DQ2 alleles, and those with HLA-B27 allele usually experience a milder clinical course. Clinically, the risk of mortality increases in patients with multiple, central nervous system hemorrhage, sepsis, disseminated intravascular coagulation (DIC) and secondary infection. The presence of adult respiratory distress syndrome (ARDS), the requirement for mechanical ventilation, the presence of dyspnea and hemoconcentration in HPS are reported to be the most important prognostic factors associated with death. The correlation of severity and the transfusion requirement with mortality was demonstrated. High serum levels of white blood cells, blood urea nitrogen (BUN), creatinine phophokinase (CPK), C-reactive protein (CRP), prothrombin time (PT), activated partial thromboplastin time (aPTT), D-dimer and INR (International normalized ratio) are prognostic factors that increase the mortality risk. Hemodialysis support is particularly important in cases infected with Hantaan and Dobrava viruses. Respiratory support and mechanical ventilation can be life-saving in HPS cases. Extracorporeal membrane oxygenation support has been demonstrated to have a favorable contribution to the patient survival in HPS. While there are some human and animal trials showing that ribavirin reduces the severity of HFRS, hemodialysis requirement and mortality, its efficacy for HPS has not yet been demonstrated. As a result, a proper evaluation of the prognostic factors will provide physicians a perspective with respect to the disease course and the necessary treatment approach.
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PMID:[Prognostic factors in hantavirus infections]. 2450 30


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