Turkish Journal of Hematology Volume: 37 Issue: 1 / 2020

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Pantelic M, et al: Kasabach-Merritt Syndrome in an Adult Turk J Hematol 2020;37:53-54Figure 3. An exploratory laparotomy with biopsy was performed.Kasabach-Merritt syndrome (KMS) is a vascular diseasecharacterized by the presence of thrombocytopenia, anemia,disseminated intravascular coagulation (DIC), and vascularlesions. It was first described in 1940 by Kasabach-Merritt[1,2,3]. KMS often occurs during infancy and the neonatalperiod and rarely in adults [1,2,4]. KMS is commonly associatedwith kaposiform hemangioendothelioma and tufted angioma,which are rare vascular tumors produced by the lymph andcapillary endothelium with positive immunohistochemicalstaining for vascular markers (CD31, CD34) and focal positivityfor lymphatic markers (LYVE1, PROKS1, D2-40), while they arenegative for GLUT1 and Lewis Y antigen (markers specific tohemangiomas) [1,5].A 22-year-old woman presented to the emergency departmentwith abdominal pain, fever, and vomiting. Laboratoryevaluation showed moderate anemia, DIC (hypofibrinogenemia,thrombocytopenia, prolonged prothrombin, and activatedpartial thromboplastin time), and elevated D-dimer. Multislicecomputed tomography showed a large retroperitoneal tumor(Figures 1, 2). An exploratory laparotomy with biopsy wasperformed (Figure 3). Histopathological examination revealeda vascular lesion (positive for CD31 and CD34 positive; focallypositive for D2-40). The patient was transferred to anotherhospital where she was treated with corticosteroids (prednisoneat 40 mg daily), fresh frozen plasma, and cryoprecipitate, whichled to an improvement. After six months, magnetic resonanceimaging showed a regression in tumor size.Keywords: Kasabach-Meritt syndrome, Vascular lesion,Retroperitoneal tumor, Multislice computed tomographyAnahtar Sözcükler: Kasabach-Merritt sendromu, Vaskülerlezyon, Retroperitoneal tümör, Bilgisayarlı tomografiInformed Consent: A consent form was completed by allparticipants.Authorship ContributionsConcept: Mi.P., Ma.P., P.D., K.M., T.V., J.T.J; Design: Mi.P., Ma.P.,P.D., K.M., T.V., J.T.J; Data Collection or Processing: Mi.P., Ma.P.,P.D., K.M., T.V., J.T.J; Analysis or Interpretation: Mi.P., Ma.P., P.D.,K.M., T.V., J.T.J; Literature Search: Mi.P., Ma.P., P.D., K.M., T.V., J.T.J;Writing: Mi.P., Ma.P., P.D., K.M., T.V., J.T.J.Conflict of Interest: The authors of this paper have no conflictof interest, including specific financial interests, relationships,and/or affiliations relevant to the subject matter or materialsincluded.Financial Disclosure: The authors declared that this studyreceived no financial support.References1. Mahajan P, Margolin J, Iacobas I. Kasabach-Merritt phenomenon: classicpresentation and management options. Clin Med Insights Blood Disord2017;10:1-5.2. Li K, Tai M, Qin Z, Ge C. Clinical observations in mesh suture treatmentfor infants of Kasabach-Merritt phenomenon. J Paediatr Child Health2015;51:529-533.3. Kasabach HH, Meritt KK. Capillary hemangioma with extensive purpurareport of a case. Am J Dis Child 1940;59:1063-1070.4. Oak CY, Jun CH, Cho EA, Lee DH, Cho SB, Park CH, Joo YE, Kim HS, Rew JS,Choi SK. Hepatic hemangioma with Kasabach-Merritt syndrome in an adultpatient. Korean J Gastroenterol 2016;67:220-223.5. O’Rafferty C, O’Regan GM, Irvine AD, Smith OP. Recent advances in thepathobiology and management of Kasabach-Merritt phenomenon. Br JHaematol 2015;171:38-51.54
IMAGES IN HEMATOLOGYDOI: 10.4274/tjh.galenos.2019.2019.0171Turk J Hematol 2020;37:55-56Platelet SatellitismTrombosit SatellitizmiYasemin Ardıçoğlu Akışın 1 , Nejat Akar 21TOBB-ETU Faculty of Medicine, Department of Biochemistry, Ankara, Turkey2TOBB-ETU Faculty of Medicine, Department of Pediatrics, Ankara, TurkeyFigures 1 and 2. Platelet satellitism in a 5-year-old child with an upper respiratory tract infection accompanying asthma.Platelet satellitism is a rare in vitro phenomenon that occurswhen an immunoglobulinG antibody directed against theglycoprotein IIb/IIIa complex on the platelet membraneforms in ethylenediaminetetraacetic acid-treated peripheralblood at room temperature [1,2]. As the antibody coats theplatelets, platelets adhering to polymorphonuclear leukocytesshow a rosette-like appearance [3]. There is no definite causalassociation with any disease. Severe rosetting may lead to amisdiagnosis of thrombocytopenia unless peripheral smears areexamined [4].The images presented here were obtained from a 5-year-oldchild with an upper respiratory tract infection accompanyingasthma. Thrombocytopenia was not detected in complete bloodcount (Figures 1 and 2).Keywords: Platelet, Polymorphonuclear leukocytes, SatellitismAnahtar Sözcükler: Trombosit, Polimorfonükleer lökosit,SatellitizmInformed Consent: The patient’s family consented to thepublication of this study.©Copyright 2020 by Turkish Society of HematologyTurkish Journal of Hematology, Published by Galenos Publishing HouseAddress for Correspondence/Yazışma Adresi: Yasemin Ardıçoğlu Akışın, M.D., TOBB-ETU Faculty of Medicine,Department of Biochemistry, Ankara, TurkeyPhone : +90 533 336 74 32E-mail : yardicoglu@gmail.com ORCID: orcid.org/0000-0002-4109-0220Received/Geliş tarihi: April 30, 2019Accepted/Kabul tarihi: June 11, 201955
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