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Letter to the Editor
Acquired haemophilia A secondary to metastatic prostate cancer
Hemofilia A adquirida secundaria a cáncer de próstata metastásico
Alejandro Olivares-Hernándeza,b,
Corresponding author
aolivares@saludcastillayleon.es

Corresponding author.
, Luis Figuero-Péreza,b, Emilio Fonseca-Sáncheza,b,c
a Servicio de Oncología Médica, Hospital Universitario de Salamanca, Salamanca, Spain
b Instituto de Investigación Biomédica de Salamanca (IBSAL), Salamanca, Spain
c Facultad de Medicina, Universidad de Salamanca, Salamanca, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acquired haemophilia &#40;AH&#41; is a rare autoimmune disorder characterised by the production of autoantibodies that inactivate clotting factors&#44; with the most common being factor <span class="elsevierStyleSmallCaps">VIII</span> &#40;FVIII&#41; inhibitor&#46; The incidence of this disease varies between 1&#8211;2 cases per million inhabitants&#47;year&#46; There is a first age peak among young women during the postpartum period and another at 60 years of age&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The typical clinical manifestations of the acquired form are mucocutaneous and muscle bleeding&#44; unlike the congenital form in which hemarthrosis is the most common form of presentation&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The most common causes are autoimmune diseases&#44; pregnancy and neoplasms&#44; although 50&#37; are idiopathic&#46; The treatment consists of 2 differentiated parts&#58; one aimed at correcting the bleeding episodes and one aimed at controlling autoantibodies&#46; Mortality is very high&#44; ranging between 7 and 22&#37;&#46; We report the case of a 67-year-old patient who developed acquired haemophilia A secondary to metastatic castration-resistant prostate cancer &#40;mCRPC&#41; in the context of the article by Dr&#46; P&#225;ramo&#44;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> in which new haemophilia treatments with potential future implications for AH are discussed&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was diagnosed in 2016 with prostate cancer &#40;Gleason 4&#8201;&#43;&#8201;5&#41; stage <span class="elsevierStyleSmallCaps">IV</span> due to bone involvement&#46; Following the diagnosis&#44; he underwent various treatments such as complete androgen blockade with docetaxel&#44; radium-223 and abiraterone&#44; which he was receiving at the time of AH development&#46; He came to the emergency department with multiple haematomas predominantly in the parotid and cervical region&#44; with no associated trauma&#46; The blood test at the emergency department showed haemoglobin values of 9&#46;4&#8201;g&#47;dl and activated partial thromboplastin time &#40;aPTT&#41; of 101&#46;6&#8201;s&#46; In the aPTT study&#44; the factors of the intrinsic pathway were quantified by coagulative method&#44; with a FVIII&#58;C&#8201;&#61;&#8201;1&#46;54&#37;&#46; The presence of lupus anticoagulant was ruled out&#46; Factor VIII inhibitor titres were found to be elevated using the Bethesda method&#46; That is why the patient was diagnosed with acquired hemophilia A secondary to mCRPC&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Inpatient treatment with prednisone and cyclophosphamide was started&#44; as well as treatment of his underlying disease with docetaxel&#46; A few days later&#44; the patient developed a joint bleeding that required the administration of recombinant activated factor <span class="elsevierStyleSmallCaps">VII</span> &#40;rFVIIa&#41;&#44; with good subsequent control&#46; After the second cycle of chemotherapy&#44; a decrease in inhibitory factor values and an increase in FVIII&#58;C&#44; up to 46&#37;&#44; were observed&#44; for which treatment with cyclophosphamide and glucocorticoids was withdrawn&#46; The patient remained hospitalized until he received a total of 4 cycles of chemotherapy and was discharged after the stabilization of FVIII&#58;C levels&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">10&#37; of AH are due to malignant neoplasms&#44; mainly solid tumours of the prostate&#44; lung or colon&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In most cases&#44; the diagnosis of AH precedes or occurs at the same time as that of neoplasm&#46; Due to the rare condition of the disease&#44; the diagnosis is complex&#44; requiring a high degree of suspicion&#46; Initial laboratory tests usually show an elongated aPTT&#44; a normal platelet count and decreased FVIII or FIX levels&#46; It is important to rule out the use of oral anticoagulants&#44; heparins or liver function disorders in the differential diagnosis&#46; To confirm the diagnosis&#44; clinical practice guidelines recommend testing for the absence of antiphospholipid antibodies and confirming the presence of clotting factor inhibitor in the patients&#8217; plasma&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> All these causes of AH have been ruled out in the differential diagnosis of our patient&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Treatment of the underlying malignancy has not been shown to influence the control of HA and the disappearance of inhibitory factor titres&#44; however&#44; in our patient&#8217;s case&#44; by initiating a new line of treatment&#44; remission of inhibitory factor titres was possible&#46; This is why&#44; in addition to treatment of the underlying disease&#44; new lines of research with other treatments such as new clotting factors&#44; non-substitutive drugs or gene therapy will be necessary in the future&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0030" class="elsevierStylePara elsevierViewall">None&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interests</span><p id="par0035" class="elsevierStylePara elsevierViewall">None to declare&#46;</p></span></span>"
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                            0 => "M&#46; Napolitano"
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ISSN: 23870206
Original language: English
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