Risk factors, treatment and survival rates of late‐onset acquired haemophilia A: A cohort study from the Shizuoka Kokuho Database

Shoji-Asahina, Aya; Nakatani, Eiji; Imaichi, Yutaro; Ohata, Emi; Oshima, M; Miyakoshi, Akinori; Ichikawa, Yoshikazu; Dote, Hisashi; Ubukata, Nanako; Funaki, Daito; Urano, Tetsumei

doi:10.1111/hae.14793

Cited by 4 publications

(3 citation statements)

References 34 publications

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“…In addition to malignant tumors and autoimmune diseases that have been reported so far, there have been an increasing number of reports of onset triggered by infectious diseases, such as reports related to COVID-19 ( 32-34 ). Furthermore, Alzheimer's dementia, hepatitis B and diabetes may also be important risk factors for AHA ( 35 ). In the case of the present study, AHA developed just after surgery for a malignant tumor, so it was difficult to collect information that may be related to the pathogenesis, but at least there was no background of Alzheimer's dementia, hepatitis B or diabetes.…”

Section: Discussionmentioning

confidence: 99%

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Takahashi,

Morita,

Hayashi

et al. 2023

Biomed Rep

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Acquired hemophilia A (AHA) is a rare disease that results from factor VIII inhibitors causing abnormal coagulation, and certain cases may develop after highly invasive surgery. The present case study reports on a 68-year-old male patient who developed AHA after undergoing a subtotal stomach-preserving pancreatoduodenectomy for distal cholangiocarcinoma. The patient experienced complications after surgery, requiring reoperation on postoperative day (PD) 5 due to rupture of the Braun's enterostomy. On PD 6, angiography was performed after bleeding was detected in the jejunal limb, but hemostasis occurred spontaneously during the examination. Bleeding was observed again on PD 8 and direct surgical ligation was performed. On PD 14, bleeding recurred in the jejunal limb and angiography was performed to embolize the periphery of the second jejunal artery. During the procedure, the prothrombin time was normal, but only the activated partial thromboplastin time was prolonged. A close examination of the coagulation system revealed a decrease in factor VIII levels and the presence of factor VIII inhibitors, resulting in the diagnosis of AHA. Administration of steroids was initiated on PD 15 and, in addition to daily blood transfusions, activated prothrombin complex concentrate was administered to achieve hemostasis. The patient was discharged from the intensive care unit on PD 36 but later developed an intractable labial fistula due to suture failure at the gastrojejunostomy site. As the use of factor VIII inhibitors continued despite the administration of steroids, cyclophosphamide (CPA) pulse therapy was added at PD 58. However, CPA was ineffective and the administration of rituximab was initiated on PD 98. After 12 courses of rituximab, the patient tested negative for factor VIII inhibitors on PD 219. On PD 289, labial fistula closure was performed with continuous replacement of factor VIII and the patient was discharged on PD 342.

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Section: Discussionmentioning

confidence: 99%

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Takahashi,

Morita,

Hayashi

et al. 2023

Biomed Rep

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“…The SKDB includes information on a regional population‐based longitudinal cohort of 2 398 393 Japanese individuals (women, n = 1 303 667, 54.4%) living in Shizuoka Prefecture (population: approximately 3.6 million) 10 and has been used as a data source for several studies. 11 , 12 , 13 Comprehensive personally linked data were collected and all individuals were uniquely identified. The dataset includes basic information from the subscriber list (sex, age, zip code, observation period, and reason for disenrollment, including death).…”

Section: Methodsmentioning

confidence: 99%

“…The SKDB includes information on a regional population-based longitudinal cohort of 2 398 393 Japanese individuals (women, n = 1 303 667, 54.4%) living in Shizuoka Prefecture (population: approximately 3.6 million) 10 and has been used as a data source for several studies. [11][12][13] Comprehensive personally linked…”

Section: Setting and Data Sourcementioning

confidence: 99%

Leukotriene antagonists reduce epileptic seizures‐related hospitalization in older adult populations with allergic rhinitis or asthma: A population‐based cohort study using the Shizuoka Kokuho database: The Shizuoka study

Imaichi,

Nakatani,

Fukutomi

et al. 2023

Epilepsia Open

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ObjectiveManaging the risk of epileptic seizures in older adults is increasingly important as the population ages. Leukotriene receptor antagonists (LTRAs) are commonly used to treat asthma or allergic rhinitis. Preclinical studies suggest that LTRAs have antiepileptic effects; however, few population‐based etiological studies on this topic have been available. Our study explored whether LTRAs reduce hospitalization risk associated with epileptic seizure in older individuals with asthma or allergic rhinitis.MethodsWe conducted a new‐user design analysis using the Shizuoka Kokuho database. We included all individuals aged 60–89 years who had at least one episode of allergic rhinitis or asthma during the study period. We compared individuals who newly started LTRAs with those who did not take LTRAs. Propensity score matching was used to balance the baseline characteristics of the participants. We compared the hazard ratios for seizure‐related hospitalization between new LTRA users and non‐users and performed subgroup analyses.ResultsOur matched cohorts consisted of 64,724 new users and non‐users of LTRAs who were aged 60–89 years and had asthma or allergic rhinitis. During the observation period, 377 (0.58%) and 595 (0.92%) incidents were observed in the LTRA new‐user and non‐user groups, respectively. The hazard ratio for seizure‐related hospitalization was 0.75 (95% confidence interval [CI]: 0.62–0.92) in the LTRA new‐user group compared with the non‐user group. Subgroup analysis revealed that the hazard ratio was weak in diabetic patients (1.31; 95% CI: 0.72–2.38).SignificanceThis study indicated that LTRAs reduced seizure‐related hospitalization in older adult patients with allergic rhinitis or asthma. We could not evaluate the severity and related diseases of epileptic seizure during LTRAs. Further studies, including observational studies, detailed multicenter prospective studies, and clinical trials, are needed to validate these findings.

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Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

Lehoczki,

Fekete,

Mikala

et al. 2024

GeroScience

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Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder characterized by the development of neutralizing autoantibodies (inhibitors) against coagulation factor VIII (FVIII). This review provides an in-depth exploration of AHA, covering its epidemiology, pathogenesis, clinical presentation, diagnosis, complications, and treatment strategies, focusing on recent advancements. AHA can manifest in both men and women with no prior bleeding history. The annual incidence is estimated to be approximately 1 case per million individuals in the general population. The incidence increases significantly with age: the incidence among individuals aged 60 years or older is approximately 3 to 4 cases per million individuals per year. Typically, patients present with an acquired bleeding disorder that is characterized by an isolated, prolonged activated partial thromboplastin time stemming from FVIII deficiency. Diagnosis relies on the detection of neutralizing antibodies using the Nijmegen-modified Bethesda assay. Hemostatic control strategies involve bypassing agents like recombinant activated factor VII, activated prothrombin complex concentrate, and recombinant porcine FVIII for bleeding patients. Emicizumab, a novel bypassing agent, exhibits several potential advantages. In the realm of immunosuppressive treatment for inhibitor eradication, the CyDRi regimen emerged as a remarkable advancement, significantly enhancing the outlook for the management of AHA even in the elderly frail population.

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Risk factors, treatment and survival rates of late‐onset acquired haemophilia A: A cohort study from the Shizuoka Kokuho Database

Cited by 4 publications

References 34 publications

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

Leukotriene antagonists reduce epileptic seizures‐related hospitalization in older adult populations with allergic rhinitis or asthma: A population‐based cohort study using the Shizuoka Kokuho database: The Shizuoka study

Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

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