Splenic sarcoidosis: An unusual aetiology of agranulocytosis
2010; Wiley; Volume: 85; Issue: 11 Linguagem: Inglês
10.1002/ajh.21661
ISSN1096-8652
AutoresPeggy Cuillière-Dartigues, Marie‐Caroline Meyohas, Pierre Balladur, Norbert Claude Gorin, Paul Coppo,
Tópico(s)Autoimmune and Inflammatory Disorders
ResumoAmerican Journal of HematologyVolume 85, Issue 11 p. 891-891 Images in HematologyFree Access Splenic sarcoidosis: An unusual aetiology of agranulocytosis† Peggy Cuilliere-Dartigues, Peggy Cuilliere-Dartigues Service d'Anatomie et Cytologie pathologiques, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorMarie-Caroline Meyohas, Marie-Caroline Meyohas Service de Maladies Infectieuses et Tropicales, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorPierre Balladur, Pierre Balladur Service de Chirurgie Générale et Digestive, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorNorbert Claude Gorin, Norbert Claude Gorin Service d'Hématologie Clinique et de Thérapie Cellulaire, AP-HP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris and UPMC Univ Paris 6, Paris, FranceSearch for more papers by this authorPaul Coppo, Corresponding Author Paul Coppo paul.coppo@sat.aphp.fr Service d'Hématologie Clinique et de Thérapie Cellulaire, AP-HP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris and UPMC Univ Paris 6, Paris, FranceService d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, UPMC Univ Paris 06, 184 rue du Faubourg Saint-Antoine, Assistance Publique—Hôpitaux de Paris, 75012 Paris, FranceSearch for more papers by this author Peggy Cuilliere-Dartigues, Peggy Cuilliere-Dartigues Service d'Anatomie et Cytologie pathologiques, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorMarie-Caroline Meyohas, Marie-Caroline Meyohas Service de Maladies Infectieuses et Tropicales, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorPierre Balladur, Pierre Balladur Service de Chirurgie Générale et Digestive, APHP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris, FranceSearch for more papers by this authorNorbert Claude Gorin, Norbert Claude Gorin Service d'Hématologie Clinique et de Thérapie Cellulaire, AP-HP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris and UPMC Univ Paris 6, Paris, FranceSearch for more papers by this authorPaul Coppo, Corresponding Author Paul Coppo paul.coppo@sat.aphp.fr Service d'Hématologie Clinique et de Thérapie Cellulaire, AP-HP, Hôpital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75012 Paris and UPMC Univ Paris 6, Paris, FranceService d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, UPMC Univ Paris 06, 184 rue du Faubourg Saint-Antoine, Assistance Publique—Hôpitaux de Paris, 75012 Paris, FranceSearch for more papers by this author First published: 26 January 2010 https://doi.org/10.1002/ajh.21661Citations: 6 † Potential conflict of interest: Nothing to report. AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat A 18-year-old French Caribbean male with no medical past history was referred for night sweats and severe neutropenia (absolute neutrophil count <0.2 G/L). There was no fever or wasting, and physical examination was unremarkable. There was no atypical cell or excess of large granular lymphocytes on peripheral blood smear and peripheral blood immunophenotyping found a normal lymphocytes subpopulations count. Bone marrow smear and bone marrow biopsy disclosed a mild granulocytic hypoplasia with a normal representation of granulocytic progenitors at all stages of differentiation without malignant cells. Antineutrophil antibodies were negative. Serologies for human immunodeficiency virus, human T-cell leukemia virus-1, cytomegalovirus, and B and C hepatitis virus were negative and Epstein-Barr virus serology was of IgG-type. Total body computerized tomography scan revealed a mild splenomegaly (15 cm height) with diffuse nodular lesions, with no lymph node enlargement. (18)F-fluoro-2-deoxy-D-glucose positron emission tomography showed an intense splenic uptake. A laparoscopic splenectomy was therefore performed ( Image 1). The spleen weighted 480 g; on gross sections besides the clearly discernable white pulp, several nodules measuring 1–3 cm, were randomly scattered. These reddish nodules were more easily observed after formalin fixation. On histology, the white pulp was normal with reactive germinal centers without lymphoma infiltration. The reddish nodules were made of multiple epithelioid and giant-cell granulomas (black arrow); fibrinoid necrosis was rarely observed. Comparable granulomas were present in splenic hilar lymph nodes. Special stains for acid-fast bacilli and fungi were negative. The diagnosis of sarcoidosis was supported by the pathological findings. Figure Image 1Open in figure viewerPowerPoint Exhaustive explorations found no other organ involvement and serum calcium and angiotensin convertase enzyme levels were normal. Serum protein electrophoresis disclosed a polyclonal hypergammaglobulinemia (22 g/L) with no monoclonal component on immunofixation. The patient's status did not require any further therapy, as night sweats did not recur and neutrophil count recovered completely some weeks after splenectomy. Sarcoidosis is an unusual etiology of agranulocytosis [1, 2]. In the absence of lung or other systemic involvement, splenic sarcoidosis can mimic more ominous neoplastic or infectious disease [3] and in this context only splenectomy allows to establish the diagnosis. Interestingly, splenectomy may also allow the total and rapid resolution of agranulocytosis, as it was observed for our patient. This observation supports clear evidence that sarcoidosis granulomas are involved in the occurrence agranulocytosis, probably through either a direct or indirect secretion of mediators of neutropenia. References 1 Vezendi S,Dobran I. Hematologic changes in sarcoidosis. Orv Hetil 1990; 131: 679– 681. CASPubMedGoogle Scholar 2 Zia H,Zemon H,Brody F. Laparoscopic splenectomy for isolated sarcoidosis of the spleen. J Laparoendosc Adv Surg Tech A 2005; 15: 160– 162. CrossrefPubMedWeb of Science®Google Scholar 3 Warshauer DM. Splenic sarcoidosis. Semin Ultrasound CT MR 2007; 28: 21– 27. CrossrefPubMedWeb of Science®Google Scholar Citing Literature Volume85, Issue11November 2010Pages 891-891 FiguresReferencesRelatedInformation
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