scholarly journals A Pediatric Case of Acquired Hemophilia A: The Usefulness of the Activated Partial Thromboplastin Time (APTT) Cross-Mixing Test for Early Diagnosis

2018 ◽  
Vol 40 (4) ◽  
pp. 331-337
Author(s):  
Hiroshi ASAI ◽  
Rie SHIRAYAMA ◽  
Koichi OSHIDA ◽  
Yuko HONDA ◽  
Tetsuji SATO ◽  
...  
2020 ◽  
Vol 3 (2) ◽  
pp. 79
Author(s):  
Ibnu Purwanto

<p>Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.</p><p>Acquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.</p>


2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Yoshihide Sehara ◽  
Yuka Hayashi ◽  
Jun Mimuro

An 80-year-old man was admitted for acute subdural hematoma caused by a mild brain injury. His coagulation test showed an isolated prolongation of activated partial thromboplastin time (aPTT). Though the subdural hematoma did not progress, oozing bleed from the wound of tracheostomy continued. Failure of correction on aPTT mixing test supported the presence of an inhibitor to a coagulation factor. Once the diagnosis of acquired hemophilia A (AHA) was made, steroid therapy was performed, which leads him to complete remission of AHA. Isolated prolongation of aPTT can be the key to diagnose a rare coagulopathy, such as AHA.


2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Quentin Binet ◽  
Catherine Lambert ◽  
Laurine Sacré ◽  
Stéphane Eeckhoudt ◽  
Cedric Hermans

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.


2013 ◽  
Vol 46 (4) ◽  
pp. 135-139
Author(s):  
Chia-Wei Chang ◽  
Jiun-Ting Yeh ◽  
Shang-Yu Wang ◽  
Chun-Hsiang Ouyang ◽  
Chien-Hung Liao ◽  
...  

2005 ◽  
Vol 40 (1) ◽  
pp. 58 ◽  
Author(s):  
Sun Young Park ◽  
Jin Seok Kim ◽  
Yuri Kim ◽  
In Hae Park ◽  
June-Won Cheong ◽  
...  

2011 ◽  
Vol 26 (9) ◽  
pp. 1247 ◽  
Author(s):  
Ji Hyun Lee ◽  
Dong-Hwan Kim ◽  
Kiyoung Yoo ◽  
Yongmook Choi ◽  
Sun-Hee Kim ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document