Acquired hemophilia A (AHA): underreported, underdiagnosed, undertreated medical condition

被引：6

作者：

El Demerdash, Doaa M. ^{[1
]}

Ayad, Alia ^{[1
]}

Tawfik, Noha ^{[1
]}

机构：

[1] Cairo Univ, Kasr Al Ainy Hosp, Fac Med, Internal Med Dept, Cairo, Egypt

来源：

EGYPTIAN JOURNAL OF INTERNAL MEDICINE | 2022年 / 34卷 / 01期

关键词：

Hemophilia; Acquired; Bleeding; Prolonged APTT; FACTOR-VIII; MANAGEMENT; INHIBITORS;

D O I：

10.1186/s43162-021-00074-9

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

BackgroundAcquired hemophilia A (AHA) is a rare acquired bleeding disorder occurred due to the formation of inhibitory antibodies neutralizing endogenous factor VIII.Main bodyAbout half the cases are idiopathic. Symptoms include severe and unexpected bleeding that could be life-threatening. High index of suspicion should be raised when unexplained subcutaneous or post-surgical bleeding with isolated prolonged APTT.ConclusionsAcquired hemophilia A is a rare underdiagnosed underreported acquired hemostatic disorder that presents with sudden usually life-threatening bleeding; it is crucial to raise awareness and suspicion index of clinicians for early diagnosis and treatment to avoid morbidity and mortality.

引用

页数：6

共 28 条

[1] Postpartum-Acquired Hemophilia A Presenting as Hemoperitoneum: A Case Report [J].

Azam, Khalid ;

Batool, Zainab ;

Malik, Ayesha ;

Chaudhry, Manahil ;

Abdullah, Mohammad .

CUREUS JOURNAL OF MEDICAL SCIENCE, 2020, 12 (12)

[2] Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry [J].

Baudo, Francesco ;

Collins, Peter ;

Huth-Kuehne, Angela ;

Levesque, Herve ;

Marco, Pascual ;

Nemes, Laszlo ;

Pellegrini, Fabio ;

Tengborn, Lilian ;

Knoebl, Paul ;

Aspoeck, G. ;

Heistinger, M. ;

Knobl, P. ;

Makipernaa, A. ;

André, H. ;

Aouba, A. ;

Bellucci, S. ;

Beurrier, P. ;

Borg, J. Y. ;

Darnige, L. ;

Devignes, J. ;

d'Oiron, R. ;

Gautier, P. ;

Gay, V. ;

Girault, S. ;

Gruel, Y. ;

Guerin, V. ;

Hézard, N. ;

Khellaf, M. ;

Koenig, M. ;

Lévesque, H. ;

Lifermann, F. ;

Marlu, R. ;

Ninet, J. ;

Peynet, J. ;

Quemeneur, T. ;

Rothschild, C. ;

Schleinitz, N. ;

Sigaud, M. ;

Trouillier, S. ;

Voisin, S. ;

Giebl, A. ;

Holstein, K. ;

Huth-Kuhne, A. ;

Loreth, R. M. ;

Steigerwald, U. ;

Tiede, A. ;

Theodossiades, G. ;

Nemes, L. ;

Radvanyi, G. ;

Schlammadinger, A. .

BLOOD, 2012, 120 (01) :39-46

[3]

Binet Q, 2017, CASE REP HEMATOL, V2017, DOI 10.1155/2017/2057019

[4]

BOUVRY P, 1994, HAEMATOLOGICA, V79, P550

[5] Management of acquired haemophilia A [J].

Collins, P. W. .

JOURNAL OF THROMBOSIS AND HAEMOSTASIS, 2011, 9 :226-235

[6] Consensus recommendations for the diagnosis and treatment of acquired hemophilia A [J].

Collins P. ;

Baudo F. ;

Huth-Kühne A. ;

Ingerslev J. ;

Kessler C.M. ;

Castellano M.E.M. ;

Shima M. ;

St-Louis J. ;

Lévesque H. .

BMC Research Notes, 3 (1)

[7] Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation [J].

Collins, Peter W. ;

Hirsch, Sybil ;

Baglin, Trevor P. ;

Dolan, Gerard ;

Hanley, John ;

Makris, Michael ;

Keeling, David M. ;

Liesner, Ri ;

Brown, Simon A. ;

Hay, Charles R. M. .

BLOOD, 2007, 109 (05) :1870-1877

[8] Safety of factor VIII inhibitor bypass activity (FEIBA®):: 10-year compilation of thrombotic adverse events [J].

Ehrlich, HJ ;

Henzl, MJ ;

Gomperts, ED .

HAEMOPHILIA, 2002, 8 (02) :83-90

[9]

Franchini M, 2007, MED SCI MONITOR, V13, pRA55

[10] ACQUIRED HEMOPHILIA A [J].

Franchini, Massimo ;

Lippi, Giuseppe .

ADVANCES IN CLINICAL CHEMISTRY, VOL 54, 2011, 54 :71-80

← 1 2 3 →