Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)

Collins, Peter William; Baudo, F.; Knöbl, Paul; Lévesque, H.; Nemes, L.; Pellegrini, Fabio; Marco, Pascual; Tengborn, Lilian; Huth‐Kühne, Angela

doi:10.1182/blood-2012-02-409185

Cited by 296 publications

(483 citation statements)

References 23 publications

Supporting

Mentioning

423

Contrasting

Unclassified

Order By: Relevance

“…A high rate of infections and related mortality has been confirmed in the available studies (11%, 4%, and 12% of patients in a United Kingdom surveillance study 2 and in the EACH2 5 and SACHA 6 registries, respectively), whereas death due to bleeding, compared with historical data, substantially decreased in the last few years (from 22% to 2.9%).…”

mentioning

confidence: 56%

“…2 The likelihood of achieving CR was influenced by the presenting inhibitor titer and FVIII level. 5 The IST regimens are also variable in both the dosage and type of agents and with different outcome. There is no difference in inhibitor eradication or mortality between patients treated with steroids alone and combined therapy, 2,6 and stable CR is more likely with steroids and cyclophosphamide than with steroids alone.…”

mentioning

confidence: 99%

See 1 more Smart Citation

The problem of acquired hemophilia

Baudo¹,

Cataldo²

2015

Blood

Self Cite

View full text Add to dashboard Cite

The estimated average tumor purity in this study was 75%, which, although low by modern leukemia and lymphoma sequencing standards, is an improvement in signal-to-noise ratio for Hodgkin lymphoma given the incredible rarity of the Reed-Sternberg cell in its background nest of inflammatory infiltrate. Previous studies relying on laser capture microdissection could also increase the signal-to-noise ratio, but at the cost of time, effort, and still-low cell numbers.This work raises additional questions. Variant allele frequencies range from 2% to 100% within single cases. Does this imply intratumoral heterogeneity? Do individual or multiple HRS cells exist as clones and subclones with different alterations and characteristics within the same lymphoma, and if so, what would that mean for molecularly targeted therapy?Overall, this work is exciting for several reasons. It advances our knowledge of the genetic makeup of cHL but also demonstrates the feasibility of a new technique that could in the future be extended to provide additional information about transcription and potentially protein expression. The demonstration that restoration of B2M loss can reverse a characteristic phenotype is an important functional validation of a key finding and is timely and relevant because immune therapies are now being explored in Hodgkin lymphoma.

show abstract

mentioning

confidence: 56%

mentioning

confidence: 99%

The problem of acquired hemophilia

Baudo¹,

Cataldo²

2015

Blood

Self Cite

View full text Add to dashboard Cite

show abstract

“…Pemberian desmopressin tidak boleh menunda pemberian bypassing agent jika perdarahan tidak terkontrol. [13] International panel [8] menganjurkan pemberian desmopressin jika bypassing agent tidak tersedia. Dapat juga diberikan faktor FVIII dengan dosis yang besar.…”

Section: Terapi Untuk Mengatasi Perdarahanunclassified

“…[13] Yang et al [15] mendapatkan terapi dengan kortikosteroid atau kombinasi kortikosteroid dengan siklofosfamid memberikan remisi komplit sebesar 89%.…”

Section: Terapi Untuk Menghilangkan Antibodi Atau Inhibitorunclassified

See 1 more Smart Citation

Hemophilia A yang Didapat: Suatu Tinjauan tentang Diagnosis dan Manajemen

Bakta

2018

WMJ

View full text Add to dashboard Cite

AbstrakHemophilia A yang didapat atau acquired hemophilia A (AHA) adalah penyakit perdarahan yang didapat (acquired), suatu penyakit yang jarang, penyakit autoimun yang ditandai oleh autoantibodi terhadap faktor VIII. Yang khas, penderita tidak menunjukkan riwayat perdarahan sebelumnya, datang dengan keluhan utama perdarahan spontan, terutama perdarahan kulit atau mukosa. AHA lebih banyak terjadi pada umur tua, baik pada laki maupun wanita dan dihubungkan dengan berbagai penyakit, seperti penyakit autoimun (lupus erithematosus sistemik, penyakit limfoproliferatif, artritis rematoid), keganasan atau kehamilan. Sekitar setengah dari penderita bersifat idiopatik. AHA didiagnosis jika dijumpai penderita tanpa riwayat perdarahan sebelumnya, baik personal atau keluarga, dimana pemanjangan aPTT (activated plasma thromboplastin time) tidak dapat dikoreksi setelah pencampuran dengan sejumlah volume yang sama dari plasma penderita dan plasma normal yang diinkubasi selama 2 -4 jam pada suhu 37 o C. Kadar FVIII:C menurun. Aktivitas titer inhibitor FVIII:C yang spesifik dapat dideteksi dan diukur dengan Bethesda assay atau dengan modifikasinya Nijmegen assay. Dalam pengelolaan AHA, pengendalian terhadap perdarahan merupakan prioritas pertama. Pada penderita dengan kadar inhibitor yang rendah, desmopressin dapat digunakan. Terapi FV III bypassing agents (activated prothrombin complex concentrate atau recombinant FVIIa) diperlukan pada penderita dengan kadar inhibitor yang tinggi atau penderita dengan episode perdarahan yang berat. Secara bersamaan dilakukan eradikasi terhadap inhibitor (autoantibodi) untuk mengurangi risiko perdarahan berikutnya. Dianjurkan terapi lini pertama dengan kortikosteroid atau terapi kombinasi kortikosteroid dengan siklofosfamid. Untuk terapi lini kedua dapat dipakai rituximab, jika terapi lini pertama gagal atau terdapat kontraindikasi.

show abstract