Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.
Acute Kidney Injury ; Anemia, Hemolytic ; Comorbidity ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Enterohemorrhagic Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension, Malignant ; Mortality ; Plasma Exchange ; Plasmapheresis ; Polymerase Chain Reaction ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombospondins ; Thrombotic Microangiopathies

Placental abruption is a condition that should be carefully considered in perinatal management because it is associated with serious events in both the mother and neonate, such as intrauterine fetal death, cerebral palsy, obstetric critical bleeding, and uncontrollable bleeding. The concomitant presence of disseminated intravascular coagulation (DIC) more easily causes critical bleeding that may necessitate hysterectomy or multi-organ failure resulting in maternal death. Therefore, early management should be provided to prevent progression to serious conditions by performing both hemostatic procedures and DIC treatment. To take measures to improve the outcomes in both the mother and neonate, health guidance for pregnant women, early diagnosis, early treatment, development of the emergency care system, and provision of a system for transport to higher-level medical institutions should be implemented.
Abruptio Placentae ; Cerebral Palsy ; Dacarbazine ; Disseminated Intravascular Coagulation ; Early Diagnosis ; Emergency Medical Services ; Female ; Fetal Death ; Fibrinogen ; Hemorrhage ; Humans ; Hysterectomy ; Infant, Newborn ; Maternal Death ; Mothers ; Obstetric Surgical Procedures ; Pregnancy ; Pregnant Women

BACKGROUND/AIMS: This study aimed to investigate the risk factors associated with provoked pulmonary embolism (PE). METHODS: This retrospective cohort study included 237 patients with PE. Patients that had transient risk factors at diagnosis were classified as having provoked PE, with the remaining patients being classified as having unprovoked PE. The baseline clinical characteristics and factors associated with coagulation were compared. We evaluated the risk factors associated with provoked PE. RESULTS: Of the 237 PE patients, 73 (30.8%) had provoked PE. The rate of respiratory failure and infection, as well as the disseminated intravascular coagulation score and ratio of right ventricular diameter to left ventricular diameter were significantly higher in patients with provoked PE than in those with unprovoked PE. The protein and activity levels associated with coagulation, including protein C antigen, protein S antigen, protein S activity, anti-thrombin III antigen, and factor VIII, were significantly lower in patients with provoked PE than in those with unprovoked PE. Multivariate analysis showed that infection (odds ratio [OR], 3.2; 95% confidence interval [CI], 1.4 to 7.4) and protein S activity (OR, 0.97; 95% CI, 0.95 to 0.99) were significantly associated with provoked PE. CONCLUSIONS: Protein S activity and presence of infection were important factors associated with provoked PE. We should pay attention to the presence of infection in patients with provoked PE.
Cohort Studies ; Diagnosis ; Disseminated Intravascular Coagulation ; Factor VIII ; Humans ; Multivariate Analysis ; Protein C ; Protein S ; Pulmonary Embolism* ; Respiratory Insufficiency ; Retrospective Studies ; Risk Factors*

Disseminated intravascular coagulation (DIC) is a major complication in sepsis patients. We compared the performance of five DIC diagnostic criteria, focusing on the prediction of mortality. One hundred patients with severe sepsis or septic shock admitted to intensive care unit (ICU) were enrolled. Routine DIC laboratory tests were performed over the first 4 days after admission. The overall ICU and 28-day mortality in DIC patients diagnosed from five criteria (International Society on Thrombosis and Haemostasis [ISTH], the Japanese Association for Acute Medicine [JAAM], the revised JAAM [R-JAAM], the Japanese Ministry of Health and Welfare [JMHW] and the Korean Society on Thrombosis and Hemostasis [KSTH]) were compared. Both KSTH and JMHW criteria showed superior performance than ISTH, JAAM and R-JAAM criteria in the prediction of overall ICU mortality in DIC patients (odds ratio 3.828 and 5.181, P = 0.018 and 0.006, 95% confidence interval 1.256–11.667 and 1.622–16.554, respectively) when applied at day 1 after admission, and survival analysis demonstrated significant prognostic impact of KSTH and JMHW criteria on the prediction of 28-day mortality (P = 0.007 and 0.049, respectively) when applied at day 1 after admission. In conclusion, both KSTH and JMHW criteria would be more useful than other three criteria in predicting prognosis in DIC patients with severe sepsis or septic shock.
Asian Continental Ancestry Group ; Dacarbazine ; Diagnosis ; Disseminated Intravascular Coagulation* ; Hemostasis ; Humans ; Intensive Care Units ; Mortality* ; Prognosis ; Sepsis* ; Shock, Septic ; Thrombosis

Systemically sustained thrombin generation in vivo is the hallmark of disseminated intravascular coagulation (DIC). Typically, this is in response to a progressing disease state that is associated with significant cellular injury. The etiology could be infectious or noninfectious, with the main pathophysiological mechanisms involving cross-activation among coagulation, innate immunity, and inflammatory responses. This leads to consumption of both pro- and anticoagulant factors as well as endothelial dysfunction and disrupted homeostasis at the blood vessel wall interface. In addition to the release of tissue plasminogen activator (tPA) and soluble thrombomodulin (sTM) following cellular activation and damage, respectively, there is the release of damage-associated molecular patterns (DAMPs) such as extracellular histones and cell-free DNA. Extracellular histones are increasingly recognized as significantly pathogenic in critical illnesses through direct cell toxicity, the promotion of thrombin generation, and the induction of neutrophil extracellular trap (NET) formation. Clinically, high circulating levels of histones and histone–DNA complexes are associated with multiorgan failure, DIC, and adverse patient outcomes. Their measurements as well as that of other DAMPs and molecular markers of thrombin generation are not yet applicable in the routine diagnostic laboratory. To provide a practical diagnostic tool for acute DIC, a composite scoring system using rapidly available coagulation tests is recommended by the International Society on Thrombosis and Haemostasis. Its usefulness and limitations are discussed alongside the advances and unanswered questions in DIC pathogenesis.
Blood Platelets/cytology/pathology ; Disseminated Intravascular Coagulation/*diagnosis/pathology ; Fibrin Fibrinogen Degradation Products/analysis ; Humans ; Immunity, Innate ; Laboratories, Hospital ; Partial Thromboplastin Time ; Prothrombin Time ; Thrombelastography

Prompt malaria diagnosis is crucial so antimalarial drugs and supportive care can then be rapidly initiated. A 15-year-old boy who had traveled to Africa (South Africa, Kenya, and Nigeria between January 3 and 25, 2011) presented with fever persisting over 5 days, headache, diarrhea, and dysuria, approximately 17 days after his return from the journey. Urinalysis showed pyuria and hematuria. Blood examination showed hemolytic anemia, thrombocytopenia, disseminated intravascular coagulation, and hyperbilirubinemia. Plasmapheresis and hemodialysis were performed for 19 hospital days. Falciparum malaria was then confirmed by peripheral blood smear, and antimalarial medications were initiated. The patient's condition and laboratory results were quickly normalized. We report a case of severe acute renal failure associated with delayed diagnosis of falciparum malaria, and primary use of supportive treatment rather than antimalarial medicine. The present case suggests that early diagnosis and treatment is important because untreated tropical malaria can be associated with severe acute renal failure and fatality. Physicians must be alert for correct diagnosis and proper management of imported tropical malaria when patients have travel history of endemic areas.
Acute Kidney Injury* ; Adolescent ; Africa ; Anemia, Hemolytic ; Antimalarials ; Delayed Diagnosis* ; Diagnosis ; Diarrhea ; Disseminated Intravascular Coagulation ; Dysuria ; Early Diagnosis ; Fever ; Headache ; Hematuria ; Humans ; Hyperbilirubinemia ; Kenya ; Malaria* ; Male ; Nigeria ; Plasmapheresis ; Plasmodium falciparum ; Pyuria ; Renal Dialysis ; Renal Insufficiency ; Thrombocytopenia ; Urinalysis

BACKGROUND/AIMS: This study investigated whether patients with acute promyelocytic leukemia (APL) truly fulfill the diagnostic criteria of overt disseminated intravascular coagulation (DIC), as proposed by the International Society on Thrombosis and Haemostasis (ISTH) and the Korean Society on Thrombosis and Hemostasis (KSTH), and analyzed which component of the criteria most contributes to bleeding diathesis. METHODS: A single-center retrospective analysis was conducted on newly diagnosed APL patients between January 1995 and May 2012. RESULTS: A total of 46 newly diagnosed APL patients were analyzed. Of these, 27 patients (58.7%) showed initial bleeding. The median number of points per patient fulfilling the diagnostic criteria of overt DIC by the ISTH and the KSTH was 5 (range, 1 to 7) and 3 (range, 1 to 4), respectively. At diagnosis of APL, 22 patients (47.8%) fulfilled the overt DIC diagnostic criteria by either the ISTH or KSTH. In multivariate analysis of the ISTH or KSTH diagnostic criteria for overt DIC, the initial fibrinogen level was the only statistically significant factor associated with initial bleeding (p = 0.035), but it was not associated with overall survival (OS). CONCLUSIONS: Initial fibrinogen level is associated with initial presentation of bleeding of APL patients, but does not affect OS.
Adult ; Aged ; Biomarkers/blood ; Chi-Square Distribution ; Disseminated Intravascular Coagulation/blood/diagnosis/*etiology/mortality ; Female ; Fibrinogen/analysis ; Hemorrhagic Disorders/blood/diagnosis/*etiology/mortality ; Humans ; Kaplan-Meier Estimate ; Leukemia, Promyelocytic, Acute/blood/*complications/diagnosis/mortality ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Proportional Hazards Models ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

Cardiac arrest one day after cesarean section is extremely rare. Obstetrical clinicians have low experience to these serious situations necessitating immediate first aid and knowledge of its differential diagnosis. A 33-year-old woman underwent elective repeat cesarean section at 38 weeks of gestation under spinal anesthesia. The patient underwent uneventful course on that day. Loss of consciousness occurred one day after cesarean section during her first ambulation. Immediate cardiac compression was performed and eventually resulted in good recovery of her heartbeat. Her condition was suitable disseminated intravascular coagulation (DIC). She developed acute ischemic pancreatitis after cardiac arrest. We describe the consideration of amniotic fluid embolism with DIC as most appropriate in this case. To our knowledge, our case is one of the most dangerous conditions after the cesarean section. Here, we report our case with a review of literatures.
Adult ; Anesthesia, Spinal ; Cesarean Section ; Cesarean Section, Repeat ; Dacarbazine ; Death, Sudden, Cardiac* ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Embolism, Amniotic Fluid ; Female ; First Aid ; Heart Arrest ; Humans ; Pancreatitis ; Pregnancy ; Pulmonary Embolism* ; Unconsciousness ; Walking

Hemophagocytic lymphohistiocytosis (HLH) is a condition caused by excessive activation and expansion of T lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity. It is a life-threatening condition, and the reported mortality rates reach 20% to 30%. It is usually associated with infection, malignancy, or autoimmune disease, but rarely with rheumatoid arthritis (RA). We recently experienced a case of HLH with rapid progression resulting in mortality in a 38-year-old female patient with long-standing RA. She visited the clinic for evaluation of a common cold-like illness. She had hypotension, liver enzyme elevation, and pancytopenia. After admission, her hypotension continued and disseminated intravascular coagulation and metabolic acidosis developed and progressed with the appearance of azotemia. Despite supportive management, she died on the fifth hospital day. HLH should be considered as a differential diagnosis when patients with RA show acute illness with fever, cytopenia, hepatic failure, and coagulopathy.
Acidosis ; Adult ; Arthritis ; Arthritis, Rheumatoid* ; Autoimmune Diseases ; Azotemia ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Female ; Fever ; Histiocytes ; Humans ; Hypotension ; Liver ; Liver Failure ; Lymphohistiocytosis, Hemophagocytic* ; Mortality ; Pancytopenia ; T-Lymphocytes

OBJECTIVETo study the effect of thrombelastography (TEM) in the diagnosis of disseminated intravascular coagulation (DIC) in children.

METHODThe data of 117 children suffering from DIC in the pediatric intensive care unit (PICU) and Cardiologic ICU (CICU) in the authors' hospital from January 2010 to June 2012 were collected. Ninety-four children without DIC were enrolled into the control group. The platelet count, prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB), D-dimers and TEM were determined. The sensitivity and specificity of TEM were measured and the relevance of TEM and DIC was investigated to evaluate the effect of TEM and the conventional tests of the coagulation system in the diagnosis of DIC in children.

RESULTThe average R reaction time in the DIC group was significantly longer than that in the control group[(13.3 ± 3.3)s vs. (4.5 ± 2.6)s, P = 0.000 5], and the average α-angle in the DIC group was smaller than that in the control group significantly (37.2° ± 1.4° vs. 55.6° ± 3.8°, P = 0.001 0). There was significant decrease in the maximal amplitude (MA) and amplitude (A) in the DIC group, compared with the control group. The OR value (95%CI) of the R reaction time,α-angle and MA was 3.538 (1.298-5.389), 2.472 (1.820-2.224) and 0.256 (0.263-0.831) respectively, which suggests good correlation with the existence of DIC (all P < 0.01). The specificity of R reaction time, α-angle and MA was higher than that of PT, APTT and D-dimers (85.7%, 73.5% and 72.9% vs. 27.0%, 42.1% and 68.2%) . The average R reaction time of children suffering from hemorrhage of severe liver disease(n = 36) was significantly longer than that of 40 healthy children [(9.2 ± 2.7) vs. (2.3 ± 1.8)s, P = 0.001 0], while the α-angle (42.8° ± 7.6° vs. 59.2° ± 10.8°, P = 0.040 0) and the MA value [(33.9 ± 5.1) vs.(56.0 ± 8.1) mm, P = 0.020 0] were significantly smaller. The average R reaction time of children suffering from congenital coagulopathy was significantly longer than that of healthy children [(6.8 ± 3.1) vs. (2.3 ± 1.8)s, P = 0.003 0], too.

CONCLUSIONTEM, which has high specificity, is beneficial to the diagnosis of DIC in children.

Blood Coagulation ; Case-Control Studies ; Child ; Child, Preschool ; Critical Illness ; Disseminated Intravascular Coagulation ; blood ; diagnosis ; Female ; Fibrin Fibrinogen Degradation Products ; analysis ; Humans ; Intensive Care Units ; Logistic Models ; Male ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time ; ROC Curve ; Sensitivity and Specificity ; Thrombelastography