Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 75-year-old female known to have a chronic myelomonocytic leukaemia and an acquired FXI deficiency (FXI level, 5%) related to a FXI inhibitor (38 Bethesda units) was admitted to the hospital for acute pneumonia associated with a bulky pleural effusion. A therapeutic puncture was found to be essential for the patient. But, such a procedure is a haemostatic challenge which requires adequate preparation. A first treatment composed of intravenous immunoglobulins and immunosuppressive therapy failed to eradicate the inhibitor and to restore a normal FXI level. In this context, steroids or FXI concentrates were not recommended. Thus, small doses of recombinant activated factor VII were used to achieve haemostasis. The procedure was successful, the tolerance was good and no adverse events occurred.
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PMID:The use of recombinant factor VIIa (NovoSeven) in a patient with a factor XI deficiency and a circulating anticoagulant. 1168 43

Acquired hemophilia is a life-threatening condition associated with a high mortality, occurring mainly in elderly patients. We report on a 92-year-old male, administered two courses of 90 microg kg(-1) of recombinant activated factor VII (rFVIIa) due to failure of human factor VIII (FVIII) therapy, without side effects nor evidence of thrombotic complications. Despite the bleeding being completely under control, the patient died on day 11 of acute respiratory failure due to pneumonia. This case confirms previous reports on the complexity and severity of this disorder, showing that rFVIIa is an effective and safe agent for achieving hemostasis even in elderly patients with a FVIII inhibitor.
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PMID:Recombinant activated factor VII therapy in acquired hemophilia of the elderly. 1573 2

A 77-year-old man diagnosed with primary myelofibrosis (PMF), successfully controlled by thalidomide and prednisolone, was referred to us for massive subcutaneous bleeding involving the face, body, and all four limbs. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer, resulting in a diagnosis of acquired hemophilia A (AHA) for which he was treated with prednisolone and cyclophosphamide on admission. He developed right femoral intramuscular hemorrhage soon after immunosuppressive therapy and was treated with rituximab combined with activated prothrombin complex concentrates. Furthermore, he suffered complications of respiratory failure with increasing throat hemorrhaging. Recombinant activated factor VII (rFVIIa) was administered combined with methylprednisolone pulse therapy. Bleeding, including respiratory failure, was ameliorated with rFVIIa. Immunosuppressive rituximab therapy resolved AHA with marked efficacy. He died of Pneumocystis jiroveci pneumonitis. Autopsy showed transformation from PMF to acute myeloid leukemia.
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PMID:[Primary myelofibrosis complicated by acquired hemophilia A and subsequent development of acute myeloid leukemia]. 2445 52

We herein report the cases of two elderly patients with acquired hemophilia A (AHA) for whom treatment was difficult.An 89-year-old woman (Case 1) was admitted to our department with subcutaneous hemorrhage and melena. Her activated partial thromboplastin time (APTT), factor VIII activity, and factor VIII inhibitor level were 127.7 seconds, 1.0%, and 48 BU/mL, respectively, which was suggestive of AHA. The administration of prednisolone (PSL 0.5 mg/kg) was initiated. After 3 weeks, PSL was combined with cyclophosphamide (CPA 50 mg). Two months after the start of treatment, her factor VIII inhibitor level decreased to 3.4 BU/mL. However, hemorrhagic signs were repeatedly observed during the discontinuation of recombinant activated factor VII (rFVIIa) preparation; bleeding control became insufficient, and pneumonia developed, thus leading to a fatal outcome.An 81-year-old woman (Case 2) was admitted to our department with subcutaneous hemorrhage, anemia (Hb: 9.2 g/dL), and a prolonged APTT (78.7 seconds). Her factor VIII activity was reduced to 0.9%, and her factor VIII inhibitor level was markedly increased to 1,364.9 BU/mL, suggesting AHA. Treatment with PSL (0.5 mg/kg) was initiated. After one month, it was combined with CPA (50 mg); however, her hemorrhagic signs were protracted, and her Hb level decreased to 8.0 g/dL. Subsequently, pneumonia occurred. However, weekly rituximab therapy (375 mg/m2) for 4 weeks decreased her factor VIII inhibitor level, leading to the disappearance of the inhibitor at 1 year and 5 months. During this period, there were no episodes requiring the administration of bypassing agents, such as rFVIIa.
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PMID:Two elderly patients with difficult-to-treat acquired hemophilia A. 2788 31

Pneumonia is the most frequent cause of sepsis, and Klebsiella pneumoniae is a common pathogen in pneumonia and sepsis. Infection is associated with activation of the coagulation system. Coagulation can be activated by the extrinsic and intrinsic routes, mediated by factor VII (FVII) and factor XII (FXII), respectively. To determine the role of FVII and FXII in the host response during pneumonia-derived sepsis, mice were treated with specific antisense oligonucleotide (ASO) directed at FVII or FXII for 3 wk before infection with K. pneumoniae via the airways. FVII ASO treatment strongly inhibited hepatic FVII mRNA expression, reduced plasma FVII to ~25% of control, and selectively prolonged the prothrombin time. FXII ASO treatment strongly suppressed hepatic FXII mRNA expression, reduced plasma FXII to ~20% of control, and selectively prolonged the activated partial thromboplastin time. Lungs also expressed FVII mRNA, which was not altered by FVII ASO administration. Very low FXII mRNA levels were detected in lungs, which were not modified by FXII ASO treatment. FVII ASO attenuated systemic activation of coagulation but did not influence fibrin deposition in lung tissue. FVII ASO enhanced bacterial loads in lungs and mitigated sepsis-induced distant organ injury. FXII inhibition did not affect any of the host response parameters measured. These results suggest that partial inhibition of FVII, but not of FXII, modifies the host response to gram-negative pneumonia-derived sepsis.
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PMID:Inhibition of the extrinsic or intrinsic coagulation pathway during pneumonia-derived sepsis. 3013 9