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Cantini M

de Franceschi L

Delmonte L

Zamo A

ZAMO', Alberto

CANTINI, Maurizio

DE FRANCESCHI, Lucia

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An unusual case of sarcoidosis in an adult patient withsickle cell disease: Management with methotrexate andlow dose of steroidTo the editor: Sarcoidosis is a multisystem disease, which pathogenesis isstill only partially known. The prevalence of sarcoidosis is largely differentdepending on race and ethnicity [1]. The association of sarcoidosis andsickle cell disease (SCD) has been reported in few cases [2]. In June2011, a 52-year-old-man with sickle/b-thalassemia in a care of our servicesince 2005 presented with a 2 week history of cough and non-specificchest discomfort, without fever or typical SCD-related. He had beentreated with hydroxyurea (20 mg/kg/day) for the past 6 years following anacute chest syndrome; during this time he had sporadic episodes ofheadache with a brain magnetic resonance documenting silent infarctionin the left parietal brain area. The patient was clinically evaluated and bio-chemistry and hematological parameters were performed. Hematocrit was37%, hemoglobin levels were 11.7 g/dL, reticulocytes were 350,000/lL,lactate dehydrogenase was 708 U/L, and both transaminases wereslightly increased (aspartate transaminase, AST 64 U/L; alanine transami-nase, ALT: 55 U/L). The pulmonary vital capacity was significantlyreduced (<60%). The X-ray chest documented two lower zone pulmonaryopacities, while a chest and abdomen computed tomography (CT) scanrevealed lower zone pulmonary infiltrates and multiple lymph nodes in thesuperior mediastinum, two lymph nodes in parathracheal area, few lymphnodes at the celiac trunk and in the inguinal area. The map of enlargedlymph nodes was confirmed by positron emission tomography (PET)-CT.The histology of the removed PET-CT positive inguinal lymph node wasconsistent with angiolymphoma. Some of the mediastinal lymph nodeswere removed with mediastinoscopy. The mediastinal lymph node biopsyshowed the presence of partially confluent, non-caseating granulomaswith focal associated deposition of fibrous tissue. The lymph node biopsywas consistent with sarcoidosis (fungal and tuberculosis cultures werenegative). Hydroxyurea was stopped and erythrocytapheresis was initiatedto prevent severe SCD clinical manifestations [3,4]. Although glucocorti-coids are the most commonly used therapeutic agent for sarcoidosis,SCD patients incur the risk of developing severe sickle cell related clinicalmanifestation during treatment with steroids [5]. In sarcoidosis, methotrex-ate (MTX) has been proposed as a standard second-line agent in patientsthat cannot use or do not respond to steroids [6]. Limited data on MTXand SCD are present in the literature. In a group of SCD patients withrheumatic disorders treated with MTX, increased vaso-occlusive crisis hasbeen reported [5]. Therefore, the patient was treated with a low dose ofprednisone (25 mg/day) associated with MTX (7.5 mg weekly and folicacid 5 mg/week). After 6 months of treatment, the chest CT demonstrateda reduction in size of both lung infiltrates and mediastinal lymph nodes.Neither sickle cell related acute events nor significant changes in transa-minases levels were reported in a 10-month follow-up. This is the firstreported case of sarcoidosis in SCD patient treated with low dose gluco-corticoid and MTX, in which erythrocytapheresis has been used in clinicalmanagement of a SCD patient to prevent SCD acute manifestations.LETIZIA DELMONTE1ALBERTO ZAMO2MAURIZIO CANTINI3LUCIA DE FRANCESCHI1*1Department of Medicine, University of Verona and AOUI-Verona, Verona;Italy; 2Department of Pathology and Diagnostic, University of Verona andAOUI-Verona; Verona; Italy; 3Blood Bank, AOUI-Verona, Verona; ItalyConflict of interest: Nothing to report.*Correspondence to: Lucia De Franceschi, MD, Department of Medicine,Section of Internal Medicine, University of Verona and AOUI- Verona, VeronItaly and Policlinico GB Rossi, P.le L. Scuro, 10; 37134 Verona, ItalyE-mail: lucia.defranceschi@univr.itPublished online 21 December 2012 in Wiley Online Library(wileyonlinelibrary.com).DOI: 10.1002/ajh.23378References1. Hunninghake GW, Costabel U, Ando M, et al. ATS/ERS/WASOG statementon sarcoidosis. American Thoracic Society/European Respiratory Society/WorldAssociation of Sarcoidosis and other Granulomatous Disorders. SarcoidosisVasc Diffuse Lung Dis 1999;16:149–173.2. Lehmann LH, Riess H, Sturm I. Sickle cell disease, pulmonary hyperten-sion, and sarcoidosis. Ann Hematol 2008;87:591–592.3. de Franceschi L, Finco G, Vassanelli A, et al. A pilot study on the efficacyof ketorolac plus tramadol infusion combined with erythrocytapheresis in themanagement of acute severe vaso-occlusive crises and sickle cell pain. Hae-matologica 2004;89:1389–1391.4. De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and sickle celldisease. Semin Thromb Hemost 2011;37:226–236.5. Michel M, Habibi A, Godeau B, et al. Characteristics and outcome ofconnective tissue diseases in patients with sickle-cell disease: report of 30cases. Semin Arthritis Rheum 2008;38:228–240.6. Baughman RP, Koehler A, Bejarano PA, et al. Role of liver function tests indetecting methotrexate-induced liver damage in sarcoidosis. Arch Intern Med2003;163:615–620.CorrespondenceVC 2012 Wiley Periodicals, Inc.American Journal of Hematology 243 http://wileyonlinelibrary.com/cgi-bin/jhome/35105

An unusual case of sarcoidosis in an adult patient with sickle cell disease: Management with methotrexate and low dose of steroid

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An unusual case of sarcoidosis in an adult patient with sickle cell disease: Management with methotrexate and low dose of steroid

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