scholarly journals Surgery-associated acquired hemophilia A: a report of 2 cases and review of literature

BMC Surgery ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Umar Zeb Khan ◽  
Xiangwu Yang ◽  
Matiullah Masroor ◽  
Abdul Aziz ◽  
Hui Yi ◽  
...  
Keyword(s):  
Mortality Rate ◽  
Factor Viii ◽  
Hemophilia A ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Bleeding Site ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Acquired Haemophilia ◽  
Upper Abdominal

Abstract Background Acquired Hemophilia A (AHA) is a rare bleeding diathesis in patients with no previous personal or family bleeding history. The diagnosis of this disease often delays due to unfamiliarity of physicians with it, which leads to its high mortality rate. Case presentation Two cases (one 12 years old female and another 18 years old male) were admitted for right upper abdominal mass and right upper abdominal pain respectively at different times. Pre-operative diagnosis of both cases was congenital choledochal cyst. They suffered continuous gastrointestinal bleeding (hematemesis and melena) with reduced hemoglobin to 54 g/L and 60 g/L after Roux-en-Y anastomosis respectively. To investigate the exact bleeding site, Digital subtraction angiography (DSA) of case 1 showed contrast overflow at small branch of proper hepatic artery but had unremarkable result for case 2, whereas gastroscopy of both cases showed unremarkable results. Multiple surgeries were also performed for hemostatic purpose but each time no active bleeding site was found. Finally, hematologists consultation was mandated in both cases and they were diagnosed as acquired haemophilia A. However, unfortunately case 1 patient could not survive because of sever hemorrhage and infection while Case 2 of 18 years old male survived after proper haemophilia treatment catalog. Conclusion Awareness about surgery associated acquired haemophilia A (SAHA) can facilitate quick diagnosis and lifesaving management because the mortality rate in SAHA is high due to lake of knowledge or late recognition of the disease. Bleeding always occurs at surgical sites and it can occur immediately within few hours after surgery in some cases. Hemorrhage may be severe or even life threatening and it presents a special challenge for diagnosis and treatment in a patient who has just undergone a surgical procedure. The treatment strategies for AHA include resumption of hemostasis with either recombinant porcine factor VIII (rpFVIII) or bypassing agents and immunosuppressive therapy to suppress the production of the factor VIII inhibitor.

scholarly journals Postpartum Acquired Hemophilia: A Rare Cause of Postpartum Hemorrhage

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Srikanth Seethala ◽  
Sumit Gaur ◽  
Elizabeth Enderton ◽  
Javier Corral
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Factor Vii ◽  
Normal Vaginal Delivery ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Viii Inhibitor

A 36-year-old female started having postpartum vaginal bleeding after normal vaginal delivery. She underwent hysterectomy for persistent bleeding and was referred to our institution. An elevation of PTT and normal PT made us suspect postpartum acquired hemophilia (PAH), and it was confirmed by low factor VIII activity levels and an elevated factor VIII inhibitor. Hemostasis was achieved with recombinant factor VII concentrates and desmopressin, and factor eradication was achieved with cytoxan, methylprednisolone, and plasmapheresis.

scholarly journals Case Series: Outcome and Cost Analysis of Rituximab Versus Non-Targeted Immunosuppression As First Line Inhibitor Eradication in Acquired Hemophilia a

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2399-2399
Author(s):  
Ellen Cusano ◽  
Adrienne Lee ◽  
Julia Hews-Girard
Keyword(s):  
Emergency Department ◽  
Mortality Rate ◽  
Cost Analysis ◽  
Factor Viii ◽  
Hemophilia A ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Bleeding Events ◽  
Inhibitor Titre ◽  
The Cost

Introduction Acquired hemophilia A (AHA) is a rare bleeding disorder where autoantibodies interfere with factor VIII activity. AHA causes severe bleeding in 90% of affected individuals and carries a high mortality rate (8-22%). The initial factor level and inhibitor titre do not correspond to bleeding severity, and the risk of severe and fatal hemorrhage persists until the inhibitor has been eradicated. Therefore, treatment must concurrently address bleeding (with hemostatic and bypassing agents) and inhibitor eradication. Immunosuppressive therapy (IST) aims to normalize factor VIII levels by achieving the latter. With a median age of 78 years at diagnosis, AHA patients typically have more comorbidities, lower reserve, and are more susceptible to adverse events secondary to IST. In 2015, local availability of Rituximab improved via the Short Term Exceptional Drug Therapy program, allowing upfront Rituximab treatment for AHA. Prior to 2015, standard IST was steroids and cyclophosphamide as published randomized control trials comparing IST regimens are lacking. The purpose of this study was to compare Rituximab (R) to non-Rituximab (non-R) IST. Primary outcomes were time to inhibitor eradication, number of infections, and number of bleeding events. Secondary outcomes included number of hospital and emergency department (ED) admissions, mortality and relapse rate, and cost analysis. Methods A retrospective chart review was performed on all AHA patients diagnosed and treated at the Southern Alberta Rare Blood & Bleeding Disorders Comprehensive Care Clinic (SARBBDC). Data was extracted from electronic and paper medical records from the time of diagnosis to the time of death, or December 2018, whichever occurred first. Descriptive statistics were used to summarize relevant demographics and outcomes. Mann-Whitney and t-tests were used to compare groups. Kaplan Meier curves and log-rank test was used for time to inhibitor eradication. Pricing for cost analysis was obtained from the local hospital billing office, Canadian Blood Services, and the Alberta Health Services Provincial Drug Formulary (current as of April 1, 2019). Results Between 1998 and 2018, 17 AHA patients were treated at the SAARBBDC. Ten (59%) were female and seven (41%) were male. In seven (41%) of the cases, there was an underlying cause: three related to autoimmune disease, three to malignancy, and one to pregnancy. All patients received prednisone as a part of initial IST. Seven patients received R IST and 10 received non-R. Table 1 describes IST regimens and patient characteristics. The average PTT and inhibitor titre at diagnosis were greater in the R group (p=0.006, p=0.02). There were no other statistically significant differences between the groups. Median time to inhibitor eradication was greater in the R group but was not statistically significant (Figure 1, p=0.69). There were more ED visits and infections per patient in the non-R group, although not statistically significant. There were more bleeding events per patient in the R group, but the cost of bleeding treatment per patient was greater in the non-R group. Overall, there were no deaths secondary to bleeding. Mortality rate was greater in the non-R group (10% v 0%). Relapse rate was greater in the R group (14% v 10%). There was a significantly different cost for IST per kilogram between non-R and R-groups ($23.20/kg v $167.71/kg, p=0.004); no significant difference in cost for bleeding treatment per kilogram ($1479/kg v $1406/kg, p=0.41). The overall average cost per patient was greater in the non-R group despite a significantly greater cost for IST in the R group. This was mainly driven by the cost of bleeding treatment. Conclusion Overall, median time to inhibitor eradication and number of bleeding events were greater in the R group. However, the number of infections, emergency department visits, and mortality rate was greater in the non-R group despite a younger median age, suggesting the toxicity of the IST may be clinically significant. Although the cost of Rituximab is significant, there is a $13,000 cost savings on average for the treatment of an AHA patient with Rituximab-containing IST. This evidence can be used to guide clinical decision making at the local level and has the potential to reduce overall burden on our health care system. Disclosures No relevant conflicts of interest to declare.

scholarly journals Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Makoto Saito ◽  
Hajime Senjo ◽  
Minoru Kanaya ◽  
Koh Izumiyama ◽  
Akio Mori ◽  
...  
Keyword(s):  
Cerebral Infarction ◽  
Factor Viii ◽  
Hemophilia A ◽  
Activity Level ◽  
High Dose ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Hemostatic Agents ◽  
Frequent Administration

A 74-years-old male who was a smoker and received treatment for hypertension, dyslipidemia, peripheral arterial disease and idiopathic interstitial pneumonia complained of subcutaneous hemorrhage of the right lower thigh. Marked anemia (hemoglobin 5.5 g/dL) and prolonged activated partial thromboplastin time (≥130 seconds) were noted. The factor VIII activity level was reduced to 1.2 %, and the factor VIII inhibitor titer was 285.3 BU/mL, a diagnosis of acquired hemophilia A (AHA) was made. Then, hematomas of 5 intra-muscles were recurred. Hemostasis became difficult despite frequent and high-dose administration of recombinant human coagulation factor VIIa (total: 18 days, 305 mg). Hemostasis was achieved by switching to activated prothrombin complex concentrate (for 3 days, 18,000 units), however, cerebral infarction occurred after 36 days. After the frequent administration of bypass hemostatic agents on elderly AHA patients with several risk factors for ischemic stroke, the risk of subsequent thrombotic events may persist for 1 month.

Acquired Hemophilia A: A Single Institution’s Experience.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4103-4103
Author(s):  
Devinderpal Randhawa ◽  
Ibrahim Sidhom ◽  
Gunwant Guron ◽  
Trevor Layne
Keyword(s):  
Factor Viii ◽  
Immunosuppressive Therapy ◽  
Hemophilia A ◽  
Treatment Modalities ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Mortality And Morbidity ◽  
Life Threatening ◽  
Viii Inhibitor

Abstract Background: Acquired Hemophilia A (AH) due to factor VIII inhibitor is a rare life threatening disorder. If not diagnosed and treated urgently, significant mortality and morbidity results. AH can occur in setting of old age, autoimmune diseases, pregnancy, medication, malignancy, and lymphoproliferatve disorders. In majority of cases it is idiopathic. Objective: Review the treatment modalities and outcome of AH patients at our institution. Methods: A retrospective review of the data pertaining to patients who were diagnosed with AH at our institution between 1993–2004. Results: There were 5 patients diagnosed with AH, 3 female and 2 male. The median age was 67 years (range 30–84 years) the setting for development of AH in these patients was as follows: 1- postpartum, 1-HIV, 3 idiopathic. All patients presented with varying degree of spontaneous hemorrhage. The median Factor VIII inhibitor level was 16 Bethesda Unit (BU)(range 7. 2–31). Acute control of hemorrhage was achieved in all patients using either FEIBA (Factor eight inhibitor bypass activity) alone (1 patient), FEIBA and Novo seven (VIIa)(4 patients). Chronic immunosuppressive therapy was given as follows: Steroid alone (2 patients), Steroid and IVIG (1 patients), Steroid and Cyclophospamide (1 patient) and Steroid, Cyclophospamide and Rituximab (1 patient). Complete remission (CR) was obtained in 4 patients and with the final patient still receiving treatment. In one patient, the dose of Cyclophospamide was decreased due to Leucopenia. The median time to elimination of inhibitors was 5 month (range 1–10 month). There have been no mortalities. Conclusions: AH is a life threatening condition if not promptly diagnosed and treated, mortality remains significantly high. Treatment with factors replacement and immunosuppressive therapy was effective in all our patients

Simultaneous Deep Venous Thrombosis and Acquired Factor VIII Inhibitor

2002 ◽  
Vol 8 (4) ◽  
pp. 375-379 ◽  
Author(s):  
Steven R. Deitcher ◽  
Teresa L. Carman ◽  
Kandice Kottke-Marchant
Keyword(s):  
Venous Thrombosis ◽  
Factor Viii ◽  
Deep Venous Thrombosis ◽  
Hemophilia A ◽  
Duplex Ultrasound ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Prolonged Aptt ◽  
Life Threatening

Acquired hemophilia A is a life-threatening immune-mediated hemorrhagic disorder that is most often found in individuals older than 50 who present with an unexplained activated partial thromboplastin time (aPTT) prolongation and clinically significant bleeding. The prolonged aPTT associated with acquired hemophilia A reflects factor VIII activity deficiency due to neutralizing or clearing autoantibodies. Deep venous thrombosis, in contrast, is a veno-occlusive disorder associated with several distinct hypercoagulable states that can result in significant morbidity and mortality due to pulmonary embolism, thrombus extension, and the post-thrombotic syndrome. A prolonged a PYI in the setting of thrombosis may reflect the presence of a lupus anticoagulant. In the absence of accurate diagnosis and the immediate institution of specific therapy, both disorders can be fatal. Three cases of acquired factor VIII inhibitors that included a prolonged aPTT, bleeding, and duplex ultrasound evidence of deep venous thrombosis are presented. The diagnostic and therapeutic challenges posed by these cases as well as a proposed mechanism by which pathologic thrombosis can develop in a patient with a life-threatening bleeding disorder are discussed.

scholarly journals A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tomohisa Kitamura ◽  
Tsuyoshi Sato ◽  
Eiji Ikami ◽  
Yosuke Fukushima ◽  
Tetsuya Yoda
Keyword(s):  
Factor Viii ◽  
Subdural Hematoma ◽  
Tooth Extraction ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Acute Subdural Hematoma ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

Background. Acquired hemophilia A (AHA) is a rare disorder which results from the presence of autoantibodies against blood coagulation factor VIII. The initial diagnosis is based on the detection of an isolated prolongation of the activated partial thromboplastin time (aPTT) with negative personal and family history of bleeding disorder. Definitive diagnosis is the identification of reduced FVIII levels with evidence of FVIII neutralizing activity. Case report. We report a case of a 93-year-old female who was diagnosed as AHA after tooth extraction at her home clinic. Prolongation of aPTT and a reduction in factor VIII activity levels were observed with the presence of factor VIII inhibitor. AHA condition is mild. However, acute subdural hematoma of this patient occurred due to an unexpected accident in our hospital. Hematoma was gradually increased and the patient died 13 days after admission. Discussion. Although AHA is mild, intracranial bleeding is a life-threatening condition. We also should pay attention to the presence of AHA patients when we extract teeth.

scholarly journals Acquired hemophilia A: A rare cause of gross hematuria

2015 ◽  
Vol 9 (11-12) ◽  
pp. 905
Author(s):  
Gregory W Hosier ◽  
Ross J Mason ◽  
K Sue Robinson ◽  
Gregory G Bailly
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Rare Condition ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Viii Inhibitor

Acquired hemophilia A is a rare condition caused by spontaneous development of factor VIII inhibitor. This condition most commonly presents with multiple hemorrhagic symptoms and isolated hematuria is exceedingly rare. Early diagnosis is important, as this condition carries a high mortality rate (13‒22%). We present a case of an 82-year-old man with isolated hematuria caused by a factor VIII inhibitor who was successfully treated with recombinant activated factor VII concentrate, as well as prednisone and cyclophosphamide.

scholarly journals Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Nicholas B. Abt ◽  
Michael B. Streiff ◽  
Christian B. Gocke ◽  
Thomas S. Kickler ◽  
Sophie M. Lanzkron
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Term Therapy ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Fviii Activity ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable.Methods. The patient’s plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours.Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity.Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered.

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