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Öztaş M. et al: Giant Cell Arteritis with Concomitant Chronic Myelomonocytic LeukemiaIMAGES IN HEMATOLOGYDOI: 10.4274/tjh.galenos.2020.2020.0357Turk J Hematol 2021;38:226-227Giant Cell Arteritis with Concomitant Chronic MyelomonocyticLeukemiaEşzamanlı Kronik Myelomonositik Lösemi ile Dev Hücreli ArteritMert Öztaş 1 , Ilkın Muradov 2 , Abdülkadir Erçalışkan 3 , Ahu Senem Demiröz 4 , Şebnem Batur 4 , Ahmet Emre Eşkazan 3 ,Serdal Uğurlu 11İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Rheumatology, İstanbul, Turkey2İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Internal Medicine, İstanbul, Turkey3İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Hematology, İstanbul, Turkey4İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Pathology, İstanbul, TurkeyFigure 1. PET-CT showed aortic arch involvement and diffusebone marrow FDG uptake.PET-CT: Positron emission tomography-computed tomography, FDG:fluorodeoxyglucoseFigure 2. a) Bone marrow aspiration showed monocytic cells ofover 20% and monoblasts of 7%, with dysplasia in all cell lineages(Giemsa 1000 x ). b) Bone marrow biopsy showed hyperplasiaand dysplasia in all cell lineages (hematoxylin & eosin, 400 x ). c)Immunohistochemically, MPO is positive in very few cells (400 x ).d) CD33 showed that monocytic cells are increased (400 x ).©Copyright 2021 by Turkish Society of HematologyTurkish Journal of Hematology, Published by Galenos Publishing HouseAddress for Correspondence/Yazışma Adresi: Serdal Uğurlu, M.D., İstanbul University-Cerrahpaşa, CerrahpaşaFaculty of Medicine, Department of Rheumatology, İstanbul, TurkeyE-mail : serdalugurlu@gmail.com ORCID: orcid.org/0000-0002-9561-2282Received/Geliş tarihi: June 29, 2020Accepted/Kabul tarihi: July 23, 2020226
Turk J Hematol 2021;38:226-227Öztaş M. et al: Giant Cell Arteritis with Concomitant Chronic Myelomonocytic LeukemiaA 62-year-old man with no previous medical conditions washospitalized with constitutional symptoms, which had startedwithin the past 2 months. He described recurrent fever(>39 °C), weight loss, and headache. Initial physical examinationwas unremarkable except for pallor of conjunctiva andfever (39.3 °C). His hemoglobin was 7.5 g/dL (normal range:13-17.2 g/dL), while white blood cell (WBC) and plateletcounts were 22.8x10 9 /L (normal: 4.3-10.3x10 9 /L) and 58.3x10 9 /L(normal: 150-400x10 9 /L), respectively. He also had remarkablemonocytosis of 6.2x10 9 /L (normal: 0.3-0.9x10 9 /L). He hadelevated C-reactive protein of 150 mg/L (normal: <5 mg/L) andan erythrocyte sedimentation rate of 140 mm/h. His blood andurine cultures were sterile. On the suspicion of vasculitis andin order to exclude concomitant malignancy, positron emissiontomography-computed tomography (PET-CT) was ordered,which showed both aortic arch and ascending aorta involvementwithout any signs of solid tumors (Figure 1). Diffuse bonemarrow fluorodeoxyglucose uptake was also observed on PET-CT.Fragmentation of the lamina elastica interna was detected upontemporal artery biopsy without any sign of active vasculitis. Hewas diagnosed with giant cell arteritis (GCA) based on his age,initial erythrocyte sedimentation level, headache, fever, andlarge vessel involvement in PET-CT. He was further diagnosedwith chronic myelomonocytic leukemia (CMML) with peripheralblood monocytosis (≥1x10 9 /L and monocytes accounting for≥10% of the WBC differential count) [1]. Peripheral blood smearrevealed 8% blasts and bone marrow aspiration and biopsy wereconsistent with CMML-1 (Figure 2). Prednisolone was initiatedat 40 mg/daily and his constitutional complaints were resolved.Azacytidine was planned to be initiated for CMML, but thepatient decided to receive this treatment in his hometown.While the pathogenic link between GCA and CMML has not beenclearly established so far, an association is commonly observedbetween GCA and myelodysplastic syndromes [2].Keywords: Giant cell arteritis, Vasculitis, Chronic myelomonocyticleukemia, LeukemiaAnahtar Sözcükler: Dev hücreli arterit, Vaskülit, Kronikmyelomonositik lösemi, LösemiInformed Consent: Informed consent was obtained from theindividual participant included in this study.Author ContributionsConcept: M.Ö., A.E.E., S.U; Design: M.Ö.; Data Collection orProcessing: I.M., M.Ö., A.E., A.S.D, S.B.; Interpretation: M.Ö.,A.E.E., S.U.; Literature Search: M.Ö.; Writing: M.Ö., A.E.E., S.U.Conflict of Interest: No conflict of interest was declared by theauthors.Financial Disclosure: The authors declared that this studyreceived no financial support.References1. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM,Bloomfield CD, Cazzola M, Vardiman JW. The 2016 revision to the WorldHealth Organization classification of myeloid neoplasms and acuteleukemia. Blood 2016;127:2391-2405.2. Roupie AL, de Boysson H, Thietart S, Carrat F, Seguier J, Terriou L, Versini M,Queyrel V, Groh M, Benhamou Y, Maurier F, Decaux O, d’Aveni M, RossignolJ, Galland J, Solary E, Willems L, Schleinitz N, Ades L, Dellal A, Samson M,Aouba A, Fenaux P, Fain O, Mekinian A; on behalf of MINHEMON (FrenchNetwork of Dysimmune Disorders Associated with Hemopathies). Giant-cellarteritis associated with myelodysplastic syndrome: French multicenter casecontrol study and literature review. Autoimmun Rev 2020;19:102446.227
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