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"Pregnancy-Related Acquired Hemophilia-A", The Nightmare of Non-Hematologists. A New Case Report

Author: M.K. Ramadan
Publisher: Zenodo
DOI: 10.5281/zenodo.17288016
Source: https://zenodo.org/records/17288016/files/MAROY476.pdf
M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
“P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-
Hema ologis s. A New Case Repo
M.K. Ramadan 1, 2*, K. Dimachkieh 1, H. Me hi 3, G. S. Wehbe 1, I. Chehadeh 3.
1. Depa men o Obs e ics and Gynecology, Ra ik Ha i i Uni e si y Hospi al, Bei u -Lebanon
2. Depa men o Obs e ics and Gynecology, Di ision o Ma e nal-Fe al-Medicine, Ra ik Ha i i Uni e si y
Hospi al
3. Depa men o he In e nal Medicine, Di ision o Hema ology-Oncology, Ra ik Ha i i Uni e si y
Hospi al.
*Co espondence o: M.K. Ramadan MBBCh-FACS, Clinical p o esso , Depa men o Obs e ics and
Gynecology, Lebanese Uni e si y, Facul y o Medical Sciences. Head o Ma e nal-Fe al Medicine uni ,
Makassed Gene al Hospi al, and Ra ik Ha i i Uni e si y Hospi al, Bei u -Lebanon.
Copy igh .
© 2025 M.K. Ramadan This is an open access a icle dis ibu ed unde he C ea i e Commons A ibu ion
License, which pe mi s un es ic ed use, dis ibu ion, and ep oduc ion in any medium, p o ided he o iginal
wo k is p ope ly ci ed.
Recei ed: 30 Sep 2025
Published: 06 Oc 2025
MAR Oncology and Hema ology (2025) 5:09
Case Repo
M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
Abs ac
Acqui ed Hemophilia-A (AHA) is a a e bleeding condi ion, seconda y o he sudden de elopmen
o au oan ibodies agains FVIII. A ec ed indi iduals a e p edisposed o spon aneous as well as
pos auma ic hemo hage. P egnancy- ela ed AHA (P-AHA) accoun s o 7-21% o all AHA
cases, usually eme ging in he pos pa um pe iod, bu can excep ionally be seen du ing
p egnancy. We he eby, desc ibe he clinical cou se o a 29-yea -old, heal hy mul ipa ous woman
who p esen ed wi h isola ed pos pa um hemo hage 5 days a e an une en ul aginal deli e y.
She de eloped in e mi en episodes o hea y aginal bleeding necessi a ing he pe o mance o
se e al su gical in e en ions, p olonged ICU s ay, and mul iple blood ans usions be o e
diagnosis 25 days la e . One o he ob ious pa icula i ies o P-AHA is he subs an ial p opo ion
ecei ing ini ial ca e by obs e icians/gynecologis s. The ea men o pos pa um P-AHA is
undamen ally simila o ha o AHA o o he e iologies, none heless, ea men du ing p egnancy
emains enigma ic due o he lack o guidelines and he a i y o he condi ion. Ea ly accu a e
diagnosis is c i ical in P-AHA pa icula ly hose p esen ing wi h isola ed pos pa um hemo hage,
as his can educe mo ali y and aba e he equen ly pe o med hys e ec omies among young
women o low pa i y. Unexplained massi e pos pa um hemo hage should ins iga e he
coagula ion p o ile assessmen and he u iliza ion o bypassing hemos a ic agen s, a e
consul a ion wi h he hema ologis , o he e e al o he pa ien o a specialized hema ology
cen e . Enhancing amilia i y, pa icula ly among obs e icians, and he coo dina ed managemen
wi h expe ienced hema ologis s, can subs an ially imp o e he ou come o his a e ye po en ially
p eca ious bleeding condi ion.
Keywo ds: Isola ed pos pa um hemo hage, massi e pos pa um hemo hage, pos pa um AHA,
P egnancy- ela ed-AHA, FVIII-inhibi o s.
M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
In oduc ion
Acqui ed Hemophilia A (AHA) is a a e diso de ha de elops when he immune sys em suddenly p oduces
au oan ibodies ha inhibi he unc ions o FVIII. Bleeding can a y om majo li e- h ea ening hemo hages
o mino clinically insigni ican bleeds. This condi ion has been epo ed o exis concomi an ly wi h ce ain
medical condi ions in abou 50% o cases including ce ain malignancies, espi a o y, de ma ologic,
au oimmune condi ions, p egnancy, and d ug/alle gic eac ions, wi h an e e -expanding lis ha included
ecen ly COVID-19 in ec ion and pos COVID- accina ion [1-KNOBLE-2012] In he emaining 50%, FVIII
inhibi o s can appea spon aneously wi hou any concomi an clinical condi ions. AHA is an ex emely a e
bleeding condi ion wi h an incidence o 0.2-1 pe million indi iduals/yea . [2-FRANCHINI-2006] This igu e,
howe e , migh no accu a ely e lec he ue incidence, owing o he lack o popula ion-based sc eening
s udies, unawa eness o he condi ion, and he exis ence o asymp oma ic cases. Mo eo e , i is mos ly
unde epo ed, and any ela ed mo ali y has been a ibu ed o concomi an condi ions as seen wi h
malignancies o ea ly pos pa um hemo hage. I usually a ec s he elde ly popula ion (80%) wi h equal
gende p e alence o less commonly i migh a ec younge age g oups, mos ly emales, in associa ion wi h
p egnancy, up u ed co pus lu eum cys , and OHSS. [3-BAUDO-2003, 4-MATSUOKA-2017, 5-MELO-
2021, 6-TANAKA-2003] Acco ding o he la ges egis y da abase om Eu ope, AHA was p esen in 8.4%
du ing he pos pa um pe iod and is es ima ed o a ec 1 in 350,000 bi hs. [7-TENGBORN-2012] We he eby
desc ibe he clinical cou se o a mul ig a id woman who p esen ed wi h hea y aginal bleeding 5 days
pos pa um. Simila o mos women p esen ing wi h isola ed pos pa um hemo hage, she was subjec ed o a
se ies o in asi e su gical p ocedu es and ecei ed mul iple ans usions be o e eaching he diagnosis 25 days
la e . She showed a d ama ic imp o emen a e he ini ia ion o I.V. Me hylp ednisolone and was discha ged
less han a week la e on o al P ednisone. Comple e e adica ion o he inhibi o was documen ed 3 days a e
discha ge. Gi en he a i y o P-AHA, we op ed o desc ibe he clinical cou se and discuss he managemen
ca ea s encoun e ed in his “nea -miss case”, pa icula ly o ellow obs e icians who a e he p incipal on -
line p o ide s when AHA p esen s as isola ed pos pa um hemo hage.
Case Desc ip ion
A 29-yea -old woman, G4P4A0, was discha ged home one day a e a no mal une en ul aginal deli e y a
a di e en heal h acili y. She p esen ed o ou hospi al ou days la e wi h hea y aginal bleeding o se e al
hou s’ du a ion. A admission, (21/11/2021) he pa ien looked pale, achypneic, and dizzy. Pulse:123 BPM,
BP:100/70 mmHg, a eb ile, spO2: 91 %. Hb: 9.2gm/dl, Hc : 28.1%, Pl s: 372 X 109/L, WBC: 13,250,
INR:1.14, PT:13.4s, aPTT: 58.9s. She ga e no his o y o easy b uising, pe sonal o amilial bleeding
M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
endencies. The Pel ic examina ion e ealed an in ac bi h canal wi h blood seen pou ing om he u e ine
ce ix. La ge mul iple blood clo s we e emo ed om he u e ine ca i y and uppe agina. The abdomen was
so while he u e ine undus was well-con ac ed and non- ende . Eme gen D&C was done o a p esumed
RPOC suspec ed on he in e p e a ion o a bedside ul asound examina ion. Minimal issues we e e ie ed.
Hea y bleeding esumed so an in au e ine 3-way Foley was in oduced and kep in si u o amponading.
Following his, and a e gi ing 2 uni s o PRBCs, he pa ien became s able wi h minimal bleeding. Tes s o
malignancy o au oimmune diso de s we e nega i e. The abno mal aPTT was misin e p e ed as a p obable
consequence o hea y bleeding (ea ly DIC). Bleeding became in e mi en and luc ua ed be ween mild and
hea y wi hou no ice. D&C was epea ed wo mo e imes o e he ollowing ew days, hen a hys e ec omy
was done along wi h bila e al hypogas ic a e y liga ion. Hea y bleeding ecu ed se e al days la e , which
manda ed wo explo a o y lapa o omies and pel ic packing o secu e hemos asis, wi hou majo success,
despi e ecei ing coun less uni s o PRBCs and FFPs. CT-Scan showed e ope i oneal and iliopsoas
hema omas. Bleeding was inally cu bed wi h he use o FVIIa on 4 consecu i e days. Concomi an ly,
p olonged aPTT was empi ically ea ed wi h I.V. Me hylp ednisolone o he suspicion o an au oimmune
p ocess, as ecommended by he hema ologis pending he esul s o FVIII-inhibi o and coagula ion ac o s
sen o an ou side labo a o y. Two days la e Labo a o y esul s disclosed an FVIII:c o 22%, and an FVIII-
inhibi o o 1.5 BU con i ming he diagnosis o P-AHA. S e oids con inued ollowing which she showed a
d ama ic clinical imp o emen wi h no maliza ion o aPTT wi hin 5 days and was discha ged one week la e
on a ape ed P ednisone egimen ha was wi hd awn a he disposal o he hema ologis a ew weeks la e .
Th ee days a e discha ge, no inhibi o was de ec ed and FVIII ac i i y was 100%. She was ollowed egula ly
by he hema ologis and se ial labo a o y exams e ealed sus ained comple e emission o he condi ion. Six
weeks la e , she was admi ed o emo al o a Double-J-ca he e . Six mon hs a e discha ge she unde wen
une en ul he nio haphy. In bo h ins ances, he coagula ion p o ile was no mal. Upon con ac , one mon h
la e (7 mon hs a e ini ial discha ge) by he obs e ician, she disclosed leading a heal hy li e wi h he in an .
She was counseled abou he isk o ecu ence o AHA in u u e p egnancy and he need o p ac ice eliable
con acep ion o a leas one yea o s able comple e emission.
Discussion
De-No o au oan ibodies de elopmen agains coagula ion ac o s is a a e condi ion, and he majo i y o cases
de elop agains FVIII. [8-FRANCHINI-2011] Clinically, he e is an eme gence o bleeding among indi iduals
wi hou a pe sonal o a amily his o y o bleeding dia hesis, and wi hou ecei ing an icoagulan medica ion.
I s mani es a ions a y widely be ween mino clinically insigni ican o se e e li e- h ea ening bleeding.
Fu he mo e, AHA migh also be asymp oma ic, as FVIII inhibi o s we e iden i ied inciden ally a ou ine
M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
p eope a i e assessmen o he coagula ion p o ile among 20% o heal hy indi iduals. [9-IGIMAN-1992]
AHA can excep ionally in 8.5% be ela ed o p egnancy. [[7-TENGBORN-2012]. The managemen ca ea s
in his case we e also desc ibed by o he case epo s. The o emos impo an one was he managemen by
obs e icians a p esen a ion. P-AHA is usually unde diagnosed o se e al easons, p incipally due o
unawa eness o his condi ion among non-hema ologis s. Because o he ex eme a i y o his condi ion, i is
belie ed ha many obs e icians/gynecologis s wo ldwide may be un amilia wi h he specialized labo a o y
assessmen s necessa y o con i m he diagnosis. [1-KNOEBL-2012] Pa ien s wi h AHA may p esen ini ially
o physicians in di e en special ies, who may no ha e expe ience wi h his a e diso de . [10-JAMES-2017].
In his case, he diagnosis and ini ia ion o app op ia e ea men we e unduly delayed. None o ou en i e
obs e ic s a had a p e ious encoun e wi h a simila case. This pe mi ed a se ies o eme gen hemos a ic
su gical in e en ions wi h all ela ed complica ions, oge he wi h he exposu e o successi e massi e
ans usions, all o which could ha e been aba ed i only he possibili y o AHA was en e ained and he cause
o isola ed p olonged aPTT had been judiciously elucida ed. A simila managemen app oach and sequence
o complica ions ha e been obse ed in se e al cases p esen ing wi h isola ed ea ly o la e pos pa um
hemo hage and managed by obs e icians. [11-See hala-2013, 12-Paidas-2013, 13-El Deme dash-2022]
Ano he se ious e en , ha migh ha e delayed diagnosis and compounded he bleeding endency, was he
se ies o eme gen hemos a ic su gical p ocedu es pe o med despi e p olonged aPTT, assuming ha su ge y
would p omp ly a es he hemo hage. Pe o ming su ge y in he absence o app op ia e hemos a ic and
immunosupp essi e ea men can esul in ca as ophic consequences. [5-MELO-2022] I seems ha e ading
su ge y i possible is sa e , bu when su ge y is ine i able, he pe iope a i e adminis a ion o a BPA becomes
manda o y.
A BPA in he o m o FVIIa, as o de ed by he hema ologis , was used o 4 consecu i e days, 7 days be o e
he con i med diagnosis, ollowing which bleeding became minimal. These agen s ha e been used e ec i ely
as a second-line ea men o a es massi e pos pa um hemo hage and o he bleeding incidences i espec i e
o he speci ic de ec in coagula ion. [14-HOSSAIN-2007] These agen s a e no expec ed o es o e he no mal
pa hway o o co ec a p olonged aPTT, as hei main ac ion is on ex insic and common cascade pa hways
bu can gap he de ec in he p ocess o ac i a ing Fac o X c ucial o he p ope coagula ion. BPA a e
moni o ed clinically wi h he assessmen o bleeding se e i y. In a simila case, Bin Waqa e al desc ibed he
use o hese hemos a ic agen s o a es epidu al and e ope i oneal bleeding be o e he con i med diagnosis
o P-AHA. [15-WAQAR-2021] I would be sa e i BPA we e adminis e ed o a es massi e bleeding, e en
be o e con i ma ion o AHA, and pa icula ly be o e eme gen in asi e hemos a ic p ocedu es.
IST in he o m o Me hylp ednisolone was also ied wo days be o e diagnosis (day 23) as ecommended by
he hema ologis , and his has esul ed in a d ama ic co ec ion o aPTT. We ound his app oach bene icial

M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
a e ailing all classical medical and su gical in e en ions o a es pos pa um hemo hage e en be o e
eaching a de ini e diagnosis.
A delayed diagnosis seems o be common among pos pa um P-AHA cases, pa icula ly hose p esen ing wi h
isola ed PPH, p omp ing obs e icians o p o ide con en ional hemos a ic p ocedu es and medica ions
commonly used in he managemen o PPH. These women we e consequen ly exposed o mul iple in asi e
p ocedu es and su ge y- ela ed complica ions such as hema omas, hemope i oneum, aginal aul hema omas,
and wound hema omas. In a ew un o una e cases, ca as ophic complica ions such as hemo hagic, sep ic
shock, ARDS, li e dys unc ion, AKI, MOF, and e en dea h ollowed hese has y in e en ions. [11, 12] All
hese sho comings could ha e been a e ed i AHA had been excluded be o e doing any hemos a ic su gical
in e en ions.
On he o he hand, bleeding a o he si es such as skin, mucosa, hema omas, se osa, GI, o in ol ing mul iple
si es, usually o ien ed ca e p o ide s o suspec a bleeding dia hesis and seek ea ly hema ologis ad ice.
The coagula ion p o ile is ou inely assessed be o e cesa ean deli e y and he odds o iden i ying AHA o
o he bleeding diso de s a e high. Ea ly pos pa um hemo hage ollowing aginal deli e y, howe e , usually
does no p omp he assessmen o he coagula ion p o ile and he usual esponse is o sea ch o common
causes o PPH such as u e ine a ony, bi h canal lace a ions, and RPOC. The con ibu ion o congeni al o
acqui ed bleeding dia hesis, including AHA, as he e iology o ea ly PPH, is es ima ed o be in <1%.
Consequen ly, and gi en i is a a e cause, i is cu en ly ecommended ha AHA should be suspec ed only
a e he ailu e o ou ine classical hemos a ic measu es o a es bleeding. We belie e ha in cases wi h
pe sis en o massi e hemo hage, as sugges ed by Da ey e al, i migh be p uden o suspec p omp ly he
exis ence o AHA. [16-DAVEY-2018].
Conclusion
This case highligh s he need o imp o e awa eness o AHA, pa icula ly among obs e icians, and
demons a es he impo an ole o expe ienced hema ologis s in he diagnosis and managemen o a e
bleeding diso de s. The clinical cou se imp o ed d ama ically only ollowing he la e consul a ion o he
hema ologis .
Funding
No g an was ecei ed o his s udy.
Consen o Publica ion
W i en in o med consen was ob ained om he pa ien o publica ion o his case epo .
M.K. Ramadan, MAR Oncology and Hema ology (2025) 5:09.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
A New Case Repo . MAR Oncology and Hema ology. (2025) 5:09
Con lic o in e es s
The au ho s indi idually decla ed no compe ing in e es s.
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M.K. Ramadan. (2025). “P egnancy-Rela ed Acqui ed Hemophilia-A”, The Nigh ma e o Non-Hema ologis s.
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