Acquired Hemophilia: A Ten Year Population-Based Review of Incidence Rate, Patient Demographics, and Treatment Outcomes in Manitoba, Canada

Abstract Introduction: Acquired hemophilia A (AHA) is a rare disease characterized by inhibitory autoantibodies to coagulation factor VIII (FVIII). Although this entity is well described clinically, there are few population-based data available to allow accurate estimates of incidence or outcomes. The reported incidence is approximately 1.48/million/year.Up to 50% of cases of AHA may be associated with a variety of clinical conditions. Treatment typically involves immunosuppression to eradicate the FVIII inhibitor, as well as hemostatic therapy to control bleeding. Methods: We identified all unique cases of AHA in Manitoba, Canada between April 2006 and November 2015 from the records of the provincial reference hemostasis laboratory at the Health Sciences Center in Winnipeg. Since 2006, this laboratory has provided testing for the province's entire population. AHA was defined by a positive Bethesda inhibitor assay titre (> 0.5 B.U); patients with congenital hemophilia were excluded. The diagnosis was confirmed through chart review of medical records from CancerCare Manitoba (the sole provider of tertiary out-patient hematology care in Manitoba, and site of the Manitoba Bleeding Disorders Program), as well as from hospital admission records. We used a piloted case report form to gather patient and clinical demographics such as age, sex, and comorbidities; coagulation factor and inhibitor levels, pharmacotherapeutic treatments, blood product administration, adverse events, clinical outcomes, and survival. Results: 23 new cases of AHA were identified between 1 April 2006 and 1 November 2015, corresponding to an incidence rate for the Manitoba population of 1.98 cases per million per year. The median age at diagnosis was 75 years (range 49 to 88 years). The male to female ratio was 0.92:1. 22% (n=5) of patients had an underlying condition associated with AHA: 4 had autoimmune disease, 1had an active malignancy. Most patients initially presented to an emergency department (78%, n=18), with 70% of patients (n=16) requiring hospital admission. All patients had bleeding symptoms at the time of diagnosis. 57% (n=13) had a major bleed at the time of diagnosis, defined as a decrease in hemoglobin of ≥ 20 g/L from baseline or requiring transfusion of at least two units of red blood cells in 24 hours. Combined cyclophosphamide and prednisone was the most common immunosuppressive regimen used to eradicate the FVIII inhibitor (n = 18); two of these patients also received IVIG as part of their first line therapy. One patient was treated with prednisone alone due to concurrent esophageal adenocarcinoma, and plans for future chemotherapy. Three patients were not treated due to palliation, death prior to treatment initiation, and active tuberculosis. Data regarding immunosuppressive treatment is unknown in one patient. Activated recombinant factor VII was the most common first line hemostatic agent used (43%, n = 10). 13% of patients (n=3) were treated initially with tranexamic acid because AHA had not yet been recognized as the cause of bleeding. Adverse reactions to treatment occurred in almost all patients; the most common being mood disturbance and leukopenia. The median time to remission (Bethesda titre <0.5 B.U and/or normal FVIII level) was 62 days (range 16-166 days). The median survival after diagnosis was less than 5 years. The cause of death was not available for the majority patients, but at least one patient died as a result of bleeding. Disease relapse was rare, occurring in only 2 patients. Conclusions: The incidence of AHA identified in Manitoba, Canada is 1.98/million/year, slightly higher than previous estimates. Only 21% of patients had an associated comorbidity, which is lower than previous estimates, and may reflect a bias in non-population based series. Major bleeding requiring multiple transfusions is common, as is the need for hemostatic therapy and hospitalization. Adverse events related to treatment are common. There is high mortality seen in patients with AHA. Patients who die early are likely to be undercounted in non-population based series. Disclosures No relevant conflicts of interest to declare.

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Bleeding and Response to Hemostatic Therapy in Acquired Hemophilia A (AHA): Results from the GTH-AH 01/2010 Study

Blood ◽

10.1182/blood.2019003639 ◽

2020 ◽

Cited By ~ 1

Author(s):

Katharina Holstein ◽

Xiaofei Liu ◽

Andrea Smith ◽

Paul Knöbl ◽

Robert Klamroth ◽

...

Keyword(s):

Partial Remission ◽

Hemophilia A ◽

Coagulation Factor ◽

Individual Risk ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Bleeding Rate ◽

Risk Of Bleeding ◽

Fviii Activity ◽

Hemostatic Therapy

Acquired hemophilia A (AHA) is due to autoantibodies against coagulation factor VIII (FVIII) and most often presents with unexpected bleeding. In contrast to congenital hemophilia, the patient's residual FVIII activity does not seem to correlate with the risk of bleeding as suggested from previous studies. Risk factors for bleeding have not been described. We used data from the prospective GTH-AH 01/2010 study to assess the risk of bleeding and the efficacy of hemostatic therapy. FVIII activity was measured at baseline and weekly thereafter. Bleeding events were assessed by treating physicians. A total of 289 bleeds was recorded in 102 patients. 141 new bleeds starting after day 1 were observed in 59% of the patients, with a mean rate of 0.13 bleeds per patient-week in weeks 1 to 12, or 0.27 bleeds per patient-week before achieving partial remission. Weekly measured FVIII activity was significantly associated with the bleeding rate, but only achieving FVIII ≥50% abolished the risk of bleeding. A good WHO performance status assessed at baseline (score 0 vs. higher) was associated with a lower bleeding rate. Hemostatic treatment was reported to be effective in 96% of bleeds. In conclusion, the risk of new bleeds after a first diagnosis of AHA remains high until partial remission is achieved, and weekly measured FVIII activity may help to assess the individual risk of bleeding. These results will help to define future strategies for prophylaxis of bleeding in AHA.

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512 A CASE OF AN IDIOPATHIC ACQUIRED HEMOPHILIA A IN AN ELDERLY WOMAN

Age and Ageing ◽

10.1093/ageing/afab119.13 ◽

2021 ◽

Vol 50 (Supplement_2) ◽

pp. ii14-ii18

Author(s):

E E Phyu ◽

H P Than ◽

M Hayward

Keyword(s):

Hemophilia A ◽

Bleeding Episode ◽

Coagulation Factor ◽

Oral Prednisolone ◽

The Elderly ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Fviii Inhibitor ◽

Prolonged Aptt ◽

The Uk

Abstract Introduction Acquired Hemophilia is a bleeding diathesis caused by autoantibodies that interfere with factor VIII (FVIII). Reasons for autoantibodies production are not clear but may be related to gene polymorphisms and/or CD4+ T lymphocytes. 1.3 to 1.5 cases per million population per year are reported in the UK. Half of the cases are secondary to malignancy, pregnancy related conditions, connective tissue disorders or drug reactions while the rest are idiopathic. Case Report We report a case of an acquired hemophilia A in an 86-year-old lady with underlying type 2 diabetes, hypertension, and cognitive impairment, being treated as the left lower limb cellulitis with antibiotics. She was found to have a sudden hemoglobin drop and her CT (Abdomen) confirmed a spontaneous intra-abdominal hematoma. Clotting profile showed prolonged APTT to 168.5 seconds, being not corrected at mixing study, with normal PT and INR. The FVIII assay was reduced to 18.4 iU/dL with FVIII inhibitor concentration of 0.7 Bu. Viral and autoimmune screenings were negative. The idiopathic acquired hemophilia A was diagnosed. Red blood cell transfusions, bypassing agents (FEIBA) and oral tranexamic acid were given for acute bleeding episode. Concomitantly, oral prednisolone was used to reduce the inhibitor levels. Repeated FVIII assay showed 121 iU/dL and 199iU/dL on day 6 and 12, respectively. Steroid was continued for the next 4 weeks and then gradually tapered. No further bleeding episode was noted. Conclusion The diagnosis of acquired hemophilia should be considered in any elderly patient with prolonged APTT. Mixing study is to measure the presence of inhibitors of coagulation or to detect coagulation factor deficiency. Quantitative coagulation factor assays and Bethesda Assays are performed for definitive diagnosis. Immunosuppressive regimens are the mainstay treatment. However, premorbid conditions and co-morbidities should be taken into consideration before initiating the aggressive immunosuppressive therapy in the elderly patients.

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Diagnosis and Treatment of Acquired Hemophilia: One Single-Center Experience from PR. China

Blood ◽

10.1182/blood-2021-145618 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 4250-4250

Author(s):

Rong-Fu Zhou ◽

Yueyi Xu ◽

Wenjin Gao

Keyword(s):

Hemophilia A ◽

Conflicts Of Interest ◽

Clinical Manifestations ◽

Coagulation Factor ◽

Factor Vii ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Coagulation Factor Vii ◽

Prolonged Aptt ◽

Inhibitor Titer

Abstract Objective: To deepen the understanding of the clinical manifestations of acquired hemophilia A for timely and correctly treatment. Methods: The clinical data of the acquired hemophilia A patients diagnosed in the hospital from Jan 2006 to Mar 2021 were retrospectively analyzed, and the relevant literature was reviewed. Results: 17 patients with acquired hemophilia A, male: female =10: 7, median age 61 years (19 to 78 years), were diagnosed and treated in the hospital with the median time from the onset to diagnosis 21 days (2 days to 6 months). Six patients had comorbidity, including hepatitis B carrying, chronic myelomonocytic leukemia, diabetes, hypertension and positive autoantibodies, pemphigoid and gastric cancer, respectively. Other 11 patients were healthy before the onset. All patients had large large ecchymosis of skin, and one case was combined with hematuria, and one case with retroperitoneal hematoma. All patients had APTT extension (45s-144.7s) and the prolonged APTT could not be corrected with normal mixed plasma with and without incubation at 37℃ for 2 hours. FVIII activity was 1% - 8.9% and inhibitor titer 2 - 128 Bu/ml. All patients with bleeding were with prothrombin complex/recombinant activated coagulation factor VII, some of them with pd-coagulation factor FVIII preparations. Inhibitors were removed with prednisone acetate (1 case) + chemotherapy (1 case), prednisone acetate / + CTX (11 cases) + chemotherapy (1 case), prednisone acetate/prednisolone + mabthera (2 cases) + CTX (1 case), respectively. The removal time of inhibitor was from 8 days to 4 years. During the treatment process, two patients developed lower extremity venous thrombosis, and one patient was complicated with lung infection. Conclusion: Patients with unexplained bleeding and prolonged APTT should be conducted normal mixed plasma correction test, coagulation factor activity and inhibitor titer examination. After correctly diagnosis, bypass agents /coagulation factor VIII preparations should be given timely for hemostasis, protocol based on glucocorticoid + CTX/mabthera to remove the inhibitor and symptomatic treatment for patients with primary comorbidity disease at the same time. Disclosures No relevant conflicts of interest to declare.

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Emicizumab for the treatment of acquired hemophilia A

Blood ◽

10.1182/blood.2020006315 ◽

2020 ◽

Cited By ~ 1

Author(s):

Paul Knoebl ◽

Johannes Thaler ◽

Petra Jilma ◽

Peter Quehenberger ◽

Karoline Veronika Gleixner ◽

...

Keyword(s):

Hemophilia A ◽

Coagulation Factor ◽

Standard Of Care ◽

Early Discharge ◽

Severe Bleeding ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Breakthrough Bleeding ◽

Hemostatic Efficacy ◽

Bypassing Agents

Acquired hemophilia A (AHA) is a severe bleeding disorder caused by inhibiting autoantibodies to coagulation factor VIII (FVIII). For hemostatic treatment, bypassing agents, human or porcine FVIII are currently standard of care. Emicizumab is a bispecific, FVIII-mimetic therapeutic antibody, that reduced the annualized bleeding rates in congenital hemophiliacs. Here we report on 12 patients with AHA, 6 male, 6 female, age 74 yrs (64/80) (all data medians and IQR), treated with emicizumab. Initial FVIII was <1%, inhibitor 22.3BU/mL (range 3-2000). Eight patients had severe bleeding. Emicizumab was started with 3mg/kg sc. weekly for 2-3 doses, followed by 1.5mg/kg every 3 weeks to keep the lowest effective FVIII levels. For FVIII monitoring, chromogenic assays with human and bovine reagents were used. All patients received immunosuppression with steroids and/or rituximab. After the first dose of emicizumab, APTT normalized in 1-3 days, FVIII (human reagents) exceeded 10% after 11 (7.5/12) days. Hemostatic efficacy was obtained and bypassing therapy stopped after 1.5 (1/4) days. FVIII (bovine reagents) exceeded 50%, indicating complete remission, after 115 (67/185), and emicizumab was stopped after 31 days (15/79), in median 5 injections (range 3-9) were given. No patient died from bleeding or thromboembolism, and no breakthrough bleeding was observed after the first dose of emicizumab. In conclusion, emicizumab seems to be an effective hemostatic therapy for AHA, with the advantages of sc. therapy (every 1-3 weeks), good hemostatic efficacy, early discharge, reduction of immunosuppression and adverse events.

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Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature

Journal of Medical Case Reports ◽

10.1186/s13256-020-02505-7 ◽

2020 ◽

Vol 14 (1) ◽

Author(s):

Katarzyna A. Jalowiec ◽

Martin Andres ◽

Behrouz Mansouri Taleghani ◽

Albulena Musa ◽

Martina Dickenmann ◽

...

Keyword(s):

Factor Viii ◽

Plasma Cell ◽

Hemophilia A ◽

High Titer ◽

Coagulation Factor ◽

Laboratory Diagnostics ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Plasma Cell Neoplasm ◽

Cell Neoplasm

Abstract Background Acquired hemophilia A is a rare autoimmune disease with clinically often significant bleeding diathesis resulting from circulating autoantibodies inhibiting coagulation factor VIII. Half of acquired hemophilia A cases are associated with an underlying disorder, such as autoimmune diseases, cancer, or use of certain drugs, or occur during pregnancy and in the postpartum period. In the other half, no underlying cause is identified. An association of acquired hemophilia A with plasma cell neoplasm seems to be extremely rare. Case presentation We describe a case of a 77-year-old Swiss Caucasian man who was diagnosed with acquired hemophilia A and smoldering multiple myeloma as an underlying cause. Acquired hemophilia A was treated with prednisolone, cyclophosphamide, and immunoadsorption. Extensive workup revealed a plasma cell neoplasm as the only disorder associated with or underlying the acquired hemophilia A. For long-term control of acquired hemophilia A, we considered treatment of the plasma cell neoplasm necessary, and a VRD (bortezomib, lenalidomide, and dexamethasone) regimen was initiated. Due to multiple complications, VRD was reduced to VRD-lite after two cycles. After nine cycles of induction therapy and five cycles of consolidation therapy, the patient is in complete remission of his acquired hemophilia A and very good partial remission of the plasma cell neoplasm. We conducted a literature review to identify additional cases of this rare association and identified 15 other cases. Case descriptions, including the sequence of occurrence of acquired hemophilia A and plasma cell neoplasm , treatment, evolution, and outcome are presented. Discussion and conclusions Our case, together with 15 other cases described in the literature, underscore the possibility of plasma cell neoplasm as an underlying cause of acquired hemophilia A. Physicians should consider including protein electrophoresis, immunofixation, and analysis of free light chains in laboratory diagnostics when treating a patient with acquired hemophilia A. The occurrence of excessive and unexplained bleeding in patients diagnosed with plasma cell neoplasm should raise suspicion of secondary acquired hemophilia A and trigger the request for coagulation tests, particularly in patients treated with immunomodulatory drugs such as thalidomide or lenalidomide. Additionally, early intervention with immunoadsorption can be lifesaving in cases with high-titer factor VIII inhibitors, especially when surgical interventions are necessary.

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Treatment of Acquired Hemophilia a with Rituximab and Emicizumab

Blood ◽

10.1182/blood-2020-137262 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 18-19

Author(s):

Evan C. Chen ◽

William J. Gibson ◽

Paula Temoczko ◽

Nathan T. Connell ◽

Robert Handin ◽

...

Keyword(s):

Hemophilia A ◽

Case Series ◽

High Dose ◽

Recurrent Bleeding ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Private Company ◽

Fviii Inhibitor ◽

Fviii Activity

Background Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies that inhibit coagulation factor VIII (FVIII). The disorder is understudied given its rarity and there are no randomized prospective trials to guide therapy. In practice, treatment involves attaining hemostasis and eliminating the FVIII inhibitor, typically with high-dose steroids (1 mg/kg daily) and either cyclophosphamide or rituximab. However, current approaches carry risk of significant adverse events and delayed or inadequate responses. Emicizumab is a bispecific antibody that targets coagulation factors IXa and X to recapitulate the function of endogenous FVIII. We present a case series of patients with acquired hemophilia A who were successfully treated with a regimen consisting of rituximab and emicizumab. Methods We identified patients >18 years who were diagnosed with acquired hemophilia A and received treatment with rituximab and emicizumab at Brigham and Women's Hospital between 2019 and 2020. We performed a retrospective chart review. Data collected included the patients' clinical presentation, laboratory studies (including coagulation testing, FVIII activity, and FVIII inhibitor titer), and treatments received (including systemic therapies, recombinant factor VIIa [rFVIIa], red blood cell [RBC] transfusions, and vascular embolization). We recorded the time to normalization of the activated partial thromboplastin time (aPTT) and chromogenic FVIII activity following emicizumab and rituximab initiation, respectively. Activated prothrombin complex concentrate was avoided given the use of emicizumab. Results We identified 8 patients with acquired hemophilia A who received treatment with emicizumab and rituximab. The median patient age was 81 (range 47-93). All patients sought medical attention for extensive ecchymoses or bleeding and were found to have prolonged aPTT leading to FVIII inhibitor identification (Table 1). The median inhibitor titer was 18 Bethesda units (range 9.2-107.5). Patients concurrently received 4 weekly doses of rituximab 375mg/m2 and 4 weekly loading doses of emicizumab 3mg/kg. Patient (Pt) #1 continued emicizumab 3mg/kg every two weeks to complete three months of treatment. Pts #2, #3, and #8 received high-dose prednisone (1mg/kg) at the start of treatment for a range of 10-14 days. Pt #8 received 7 additional days of prednisone for an initial aPTT of 60.7 seconds before starting emicizumab and rituximab; she had no clinical response when treated with prednisone alone. Pts #2, #5, and #7 required vascular embolization. 7 patients (Pts #2 through #8) had aPTT retested within 1 week of starting emicizumab, and the aPTT for these patients normalized within 10 days of starting emicizumab (i.e. after only 1-2 doses; Figure 1). Except for Pt #5 who had recurrent hematuria from a persistent anatomic bladder defect that eventually required prostatic artery embolization, patients did not require rFVIIa or RBC transfusions for more than 7 days after starting emicizumab. Except for Pt #5 who required 28 doses of rFVIIa and 3 units of RBC transfusions after starting emicizumab, the median number of rFVIIa doses and RBC units given to the remaining 7 patients was zero (range 0-6 doses) and zero (range 0-4 units), respectively. Pts #2 and #3 had chromogenic FVIII levels obtained >30 days after starting rituximab with improvement in FVIII activity to 29% (day 71) and 86% (day 91), respectively. During a median follow-up of 102 days, no patients experienced recurrent bleeding. However, Pt #3 exhibited a slowly increasing aPTT that reached 46.3 seconds on day 233 of follow-up without symptoms; further diagnostic testing is pending. Conclusion Our case series demonstrates that the combination of rituximab and emicizumab can be an effective and safe regimen for the treatment of acquired hemophilia A. No thrombotic events or thrombotic microangiopathy occurred. Treatment with weekly emicizumab led to aPTT normalization after 1-2 doses and facilitated hemostasis, as reflected by a median usage of zero rFVIIA doses and zero RBC transfusions after starting emicizumab when excluding one patient with hematuria from an anatomic defect. This compares favorably to historical reports. While no patient has had recurrent bleeding, additional chromogenic FVIII activity testing for patients is needed to confirm long-term normalization of FVIII activity. Disclosures Gibson: Ampressa therapeutics: Current equity holder in private company; nference: Consultancy, Current equity holder in private company; ImmPACT-Bio: Consultancy; Boston Clinical Research Institute: Consultancy. Parnes:Bayer: Consultancy; I-Mab: Consultancy; Sunovion: Consultancy; UniQure: Consultancy; Sigilon: Consultancy; Shire/Takeda: Consultancy, Research Funding; Genentech: Research Funding; Geron: Current equity holder in publicly-traded company. OffLabel Disclosure: Emicizumab is used off-label in our case series for the treatment of acquired hemophilia A.

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Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

Blood ◽

10.1182/blood-2012-02-408930 ◽

2012 ◽

Vol 120 (1) ◽

pp. 39-46 ◽

Cited By ~ 170

Author(s):

Francesco Baudo ◽

Peter Collins ◽

Angela Huth-Kühne ◽

Hervé Lévesque ◽

Pascual Marco ◽

...

Keyword(s):

Hemophilia A ◽

Hemostatic Agent ◽

Symptomatic Therapy ◽

Bleeding Control ◽

First Line ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Acquired Haemophilia ◽

Bleeding Episodes ◽

Bypassing Agents

AbstractAcquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

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A case of rare inhibitory coagulopathy - acquired hemophilia A

Clinical Medicine (Russian Journal) ◽

10.18821/0023-2149-2016-94-10-775-779 ◽

2010 ◽

Vol 94 (10) ◽

pp. 775-779

Author(s):

V. I. Ershov ◽

Dar’ya A. Budanova ◽

I. Yu. Gadaev ◽

O. V. Bochkarnikova ◽

I. Ya. Sokolova ◽

...

Keyword(s):

Rare Disease ◽

Blood Clotting ◽

Hemophilia A ◽

Coagulation System ◽

Clotting Factor ◽

Factor X ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Hemostatic Therapy ◽

Blood Clotting Factor

Inhibitory coagulopathy is a rare variant of hemorrhagic syndrome. Acquired hemophilia A is caused by the formation of inhibitors (antibodies) to Factor VIII of the blood coagulation system leading to impaired activation of the key stage of blood clotting (factor X) and development of hemorrhagic syndrome of different severity. Acquired hemophilia A is a rare disease with an incidence of 1.38-1.48 per 1 million population per year. We report a case off severe idiopathic acquired hemophilia A in a 53 year-old woman manifest as skin hemorrhages, subcutaneous and intramuscular hematomas. Hemostatic therapy described in the article resulted in the elimination of hemorrhagic syndrome and complete remission. This case represents a rare disease the knowledge of which can be useful for preventing the development of debilitating complications, and sometimes saving the patient’s life.

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Acquired Hemophilia A Presenting as Massive Postoperative Bleeding in a Patient with Oral Squamous Cell Carcinoma

Case Reports in Otolaryngology ◽

10.1155/2020/8961785 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Susumu Oba ◽

Mitsuhiko Nakahira ◽

Yasunao Kogashiwa ◽

Yasuhiro Ebihara ◽

Masashi Sugasawa

Keyword(s):

Hemophilia A ◽

Postoperative Bleeding ◽

Laboratory Data ◽

Tumor Resection ◽

Coagulation Factor ◽

Factor Vii ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Spontaneous Bleeding ◽

Recombinant Activated Factor Vii

Acquired hemophilia A (AHA) is an extremely rare and serious bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Approximately, 10% of patients with AHA have an underlying malignancy. We report on a 46-year-old man with AHA and advanced oral cancer who presented with massive bleeding after surgery. Preoperative blood coagulation tests showed no abnormalities. He underwent radical tumor resection followed by reconstruction using a free rectus abdominal musculocutaneous flap. Massive subcutaneous hemorrhage developed in his neck and abdomen on the first postoperative day. The hemorrhage remained uncontrolled, despite embolization of the responsible vessels. Subsequent laboratory data showed prolonged activated partial thromboplastin time and decreased FVIII levels. On the basis of his clinical course and the presence of the FVIII inhibitor, we speculated that the patient suffered from AHA. We administered recombinant activated factor VII and prednisolone, after which the spontaneous bleeding stopped and the subcutaneous hemorrhage resolved. A review of the literature identified only three previous documented cases of AHA associated with head and neck cancer. This case indicates that AHA should not be ruled out in patients with uncontrolled postoperative bleeding, while attempting to ensure bleeding control and preventing potentially catastrophic fatal consequences.

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Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma

Case Reports in Neurological Medicine ◽

10.1155/2015/543927 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Yoshihide Sehara ◽

Yuka Hayashi ◽

Jun Mimuro

Keyword(s):

Subdural Hematoma ◽

Hemophilia A ◽

Coagulation Factor ◽

Activated Partial Thromboplastin Time ◽

Acute Subdural Hematoma ◽

Coagulation Test ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Rare Presentation ◽

Mild Brain Injury

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.

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