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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We are presenting a case of granulomatosis with polyangiitis &#40;GPA&#41; triggering acute myeloid leukemia &#40;AML&#41;&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 38-year-old woman&#44; without previous diseases&#44; went to emergency department &#40;ED&#41; for fever&#44; dyspnea and anosmia and she had these symptoms during the last 10 days&#46; Chest X-ray showed consolidations in the right upper lobe and left lower lobe &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Also&#44; laboratory tests revealed neutrophilia &#40;8&#46;17<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">9</span>&#47;L&#41; without leukocytosis or eosinophilia and as well as elevated acute phase reactants&#44; without renal failure&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Thoracic computed tomography &#40;CT&#41; confirmed the radiographic findings and manifested a necrotic core in consolidation in the right upper lobe &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A and B&#41;&#46; Moreover&#44; CT of paranasal sinuses evidenced extensive sinus occupation &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C&#41;&#46; Microbiological cultures were negative&#46; Antineutrophil cytoplasmic antibodies with cytoplasmic pattern &#40;c-ANCA&#41; were positive at 1&#47;320 titer and anti-proteinase 3 &#40;PR3&#41; antibodies had a concentration of 60<span class="elsevierStyleHsp" style=""></span>IU&#47;mL &#40;normal is less than 10<span class="elsevierStyleHsp" style=""></span>IU&#47;mL&#41;&#46; In addition&#44; a fibrobronchoscopy was performed with transbronchial biopsies showing vasculitis and granulomas with fibrinoid necrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>D&#41;&#46; After obtaining 13 points of the 2022 ACR&#47;EULAR diagnostic criteria&#44; the diagnosis of GPA was established &#40;score &#8805;5&#41;&#46; So&#44; induction treatment with prednisone 30<span class="elsevierStyleHsp" style=""></span>mg&#47;24<span class="elsevierStyleHsp" style=""></span>h was started and rituximab 604<span class="elsevierStyleHsp" style=""></span>mg weekly was administered weekly for 4 weeks&#46; After three months&#44; the patient went to ED for asthenia presenting leukocytosis &#40;19&#46;2<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">9</span>&#47;L&#41; with monocytosis &#40;8&#46;36<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">9</span>&#47;L&#41;&#46; Peripheral blood morphology evidenced 48&#37; of blasts confirming the diagnosis of AML&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Hematologic malignancies and ANCA-associated vasculitis &#40;AAVs&#41; are related&#46; Several studies have reported the increased incidence of bladder carcinoma&#44; AML&#44; cutaneous squamous cell carcinoma&#44; lung cancer&#44; colorectal carcinoma and lymphoma in AAVs due to immunosuppression&#44; treatment and tissue inflammation&#46; Furthermore&#44; AAVs may present as paraneoplastic vasculitis&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Cyclophosphomide has been replaced by rituximab in induction and maintenance treatment of AAVs because of its dose-dependent oncogenicity &#40;skin cancer&#44; myeloid malignancies and bladder cancer&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> Rituximab increases the risk of infection&#44; produces hypogammaglobulinemia and neutropenia&#44; but cumulative exposure does not increase the risk of malignancy&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#8211;5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Overall cancer risk is higher in patients with GPA than other AAVs&#46; It can be explained by PR-3 which is an autoantigen at the crosstalk of autoimmunity and hematopoietic proliferation and is overexpressed in several hematological malignancies&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> PR3 is the target antigen for c-ANCA and is essential for associating GPA and hematological malignancies&#46; It is endothelially expressed on mature neutrophils and monocytes and is inhibited by alpha-1 antitrypsin&#46; It induces endothelial apoptosis&#44; angiogenesis&#44; regulation of myeloid lineage differentiation and inhibition of T-lymphocyte proliferation&#46; In hematological malignancies&#44; there is overexpression of PR3 due to the action of G-CSF and defects in its suppression&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4&#44;5</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The association of GPA with AML has been described in few cases in the literature&#44; mainly in paraneoplastic vasculitis and patients treated with cyclophosphamide&#46; In our case&#44; the patient does not present initial alterations in the hemogram&#44; so it is not a paraneoplastic syndrome and the AML was later&#44; which confirms the exceptionality of the case&#46; PR3 plays a key role in the hematological oncogenesis of GPA&#46; The exceptional evolution presented in this case shows us the importance of close follow-up in search of hematologic malignancies in patients with AAVs&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0040" class="elsevierStylePara elsevierViewall">This work has not received funding of any kind&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of Interests</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors state that they have no conflict of interests&#46;</p></span></span>"
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                          "autores" => array:6 [
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                            2 => "C&#46; Sundstr&#246;m"
                            3 => "M&#46; H&#246;glund"
                            4 => "C&#46; Backlin"
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                            4 => "D&#46; Blockmans"
                            5 => "M&#46;C&#46; Cid"
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Case Report
Granulomatosis With Polyangiitis as a Trigger for Acute Myeloid Leukemia
Ignacio Boira Enrique
Corresponding author
nachoboiraenrique@hotmail.es

Corresponding author.
, Violeta Esteban Ronda, Eusebi Chiner Vives
Pneumology Department, University Hospital of Saint John of Alicante, Spain

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