Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

BACKGROUND The objective of the current study was to guide the early clinical treatment strategies by assessing the recovery of abnormal coagulation in acute promyelocytic leukemia (APL) patients during induction therapy. MATERIAL AND METHODS Retrospective analysis was performed in 112 newly-diagnosed patients with APL during induction treatment. RESULTS The early death (ED) rate in our study was 5.36% and the main cause was fetal hemorrhage. The presence of bleeding symptoms was significantly correlated with low platelet and fibrinogen levels. The values of white blood cell (WBC), lactate dehydrogenase (LDH), prothrombin time (PT), fibrinogen, and bone marrow leukemic promyelocyte in the high-risk group were significantly different from those in the low/intermediate-risk groups. Coagulation variables significantly improved after dual induction therapy. No significant difference was found in changes of platelet (PLT), prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimers, and fibrinogen among different risk groups after induction therapy. D-dimer levels were initially high and remained well above normal after 4 weeks of induction therapy. CONCLUSIONS Aggressive prophylactic transfusion to maintain high platelet and fibrinogen transfusion thresholds could reduce hemorrhage in APL patients. Immediately starting induction therapy effectively alleviated coagulopathy in APL patients. Hyperfibrinolysis was a more important event in the APL hemorrhagic diathesis.
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PMID:Hyperfibrinolysis Is an Important Cause of Early Hemorrhage in Patients with Acute Promyelocytic Leukemia. 2977 70

BACKGROUND Acquired hemophilia A (AH) is a rare hemorrhagic diathesis, characterized by the presence of autoantibodies directed against the pro-coagulant activity of factor VIII. It is associated with rheumatoid arthritis (RA) in 4% to 8% of cases and its prognosis remains severe. CASE REPORT A 66-year-old patient has been followed up for 20 years for deforming and severe RA, which was in low-disease activity. However, the patient presented a polyarticular flare involving the metacarpophalangeal and the proximal interphalangeal joints, the left elbow, and the right knee, which was warm and swollen. Articular puncture of this knee yielded a hematic fluid that did not coagulate. Its cytological analysis showed significant presence of red blood cells, which were also abundantly present in the other cell lines. Activated partial thromboplastin time was lengthened and not corrected by the addition of control plasma. Prothrombin time (Quick's test), fibrinogen level, and vitamin K-dependent factors were without abnormalities. In contrast, factor VIII was collapsed at 7% and the anti-factor VIII antibody was positive. The diagnosis of AH with anti-factor VIII inhibitor was thus retained. With regard to RA, the Disease Activity Score was 6.32 and exhibited a very active RA. Rituximab with methotrexate was begun and the evolution was favorable. After 6 months, the reappearance of the anti-factor VIII inhibitor was found, thus justifying a second cycle of rituximab. CONCLUSIONS AH is not exceptional in RA. Rituximab remains a relevant alternative for managing simultaneous AH with inhibitor and RA.
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PMID:Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature. 2978 Jan 57

Two pet rabbits were presented with an acute decrease in appetite and activity. Rabbit 1 showed severe hypothermia, bradycardia, arrhythmias, a heart murmur, dyspnea, occlusion of the nares with secretions, icterus, dehydration, and gaseous gastrointestinal dilation. The urine was dark yellow. Rabbit 2 was overweight, apathetic, and dehydrated; this animal presented with a heart murmur, gastric dilation, and intermittent nystagmus with dorsal strabismus in the right eye. Blood gas, electrolyte, hematology, plasma clinical biochemistry analysis, coagulation profile, plasma protein electrophoresis, urinalysis, and radiographic examinations were performed. The main shared findings were moderate thrombocytopenia, markedly decreased aspartate aminotransferase and alanine aminotransferase activities and fibrinogen concentrations, prolonged prothrombin and activated partial thromboplastin times, profoundly increased alkaline phosphatase and gamma-glutamyl transferase (GGT) activities, and high bile acid and bilirubin concentrations. Rabbit 1 also had respiratory acidosis, marked hypoglycemia, hyperphosphatemia, and a profoundly increased creatine kinase activity. Gastric dilation was observed on both radiographic exams. A low urinary pH of 5-6, marked bilirubinuria and proteinuria, and high urinary GGT levels were present in both patients. Marked icterus developed before death, which occurred within 22 and 30 hours post admission in rabbits 1 and 2, respectively. The necropsy of rabbit 1 showed a markedly accentuated hepatic lobular pattern, pulmonary hemorrhages, pericardial effusion with adhesions, peritoneal petechiae, and icteric and hemorrhagic abdominal fat. Histopathologic findings included hemorrhagic diathesis, severe centroacinar and midzonal hepatocellular necrosis, severe necrosuppurative bronchopneumonia, and moderate cardiomyocyte necrosis. A liver PCR assay was positive for Rabbit Hemorrhagic Disease Virus (RHDV) 2 (RHDV2) and negative for classic RHDV. This is the first description of the gross clinicopathologic abnormalities associated with naturally occurring RHDV2 infection in pet rabbits.
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PMID:Clinicopathologic findings of naturally occurring Rabbit Hemorrhagic Disease Virus 2 infection in pet rabbits. 3086 86

BACKGROUND Acquired hemophilia A (AHA) is a rare hemorrhagic disorder that is caused by producing autoantibodies against factor VIII. It is usually characterized by severe, spontaneous bleeding, which can be life-threatening. The current standard treatments for bleeding prophylaxis are highly effective but accompanied with some disadvantages such as frequent intravenous infusions, high cost, and risk of thromboembolic complications. Emicizumab is a bispecific antibody with a therapeutic FVIII-mimetic nature. Emicizumab has shown a reduction in annualized bleeding rate in congenital hemophilia patients with and without inhibitors. The pathophysiological concepts and preclinical data suggest that Emicizumab can be effectively used for treating AHA. CASE REPORT We present the case of an 87-year-old woman admitted for symptomatic anemia and large chest wall and pelvic hematomas confirmed by imaging, without history of trauma. Her coagulation studies showed isolated prolonged activated partial thromboplastin time (aPTT), low factor VIII activity level, and high levels of factor VIII inhibitor. She was successfully treated with activated prothrombin complex concentrate (aPCC), which was transitioned to Emicizumab on discharge. No recurrent bleeding episodes or adverse events related to Emicizumab were reported during the 2-month follow-up period. CONCLUSIONS A subcutaneous weekly or biweekly injection of Emicizumab, a recombinant monoclonal antibody, offers several advantages: less frequent infusions, good hemostatic efficacy, possible outpatient therapy, and even more cost-effective than bypassing agents. More clinical studies should be conducted to compare Emicizumab with the current standards of care.
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PMID:Emicizumab Use in Treatment of Acquired Hemophilia A: A Case Report. 3131 50

Acquired hemophilia A (AHA) is a rare and life-threatening autoimmune hemorrhagic disorder where autoantibodies are developed against factor VIII. An early diagnosis is challenging and mandatory: an immediate hemostatic control is required to reduce morbidity and mortality. Laboratory features of AHA are: presence of autoantibodies against factor VIII, prolonged activated partial thromboplastin time (with normal prothrombin time and thrombin time) and decreased factor VIII levels. In some cases, the results of laboratory tests may be incorrect due to errors in analysis, blood extraction or manipulation of samples; also worth of consideration are limitations in the measurement range and low sensitivity of the tests. This review highlights the importance of adequate screening in patients with suspected AHA to make an adequate diagnosis and reduce overall fatal outcomes.
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PMID:[Adequate diagnosis of acquired hemophilia A]. 3134 71


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