Littoral cell angioma of the spleen revealed by a Kasabach-Merritt syndrome in a 22-month-old child

##plugins.themes.bootstrap3.article.main##

##plugins.themes.bootstrap3.article.sidebar##

Published Dec 30, 2017
DOI: https://doi.org/10.22551/2017.17.0404.10112
Marie-Amelyne Le Rouzic Julie Valduga Aurélie Phulpin-Weibel Ludovic Mansuy Audrey Contet Fanny Fouyssac Imane Athmani Marie-Agnès Galloy Michel Wassef Pascal Chastagner

Abstract

Introduction: Kasabach-Merrit syndrome (KMS) is characterized by the association of a vascular tumor, thrombocytopenia and potentially life-threatening coagulopathy. Observation: A 22-month-old child was referred to our center for incidental discovery of thrombocytopenia in a context of recurrent throat infections. Clinical examination showed petechial purpura, subicterus, no tumoral syndrome and good overall condition. His biological parameters showed regenerative hemolytic anemia (10.2 g/dL), thrombocytopenia (32 G/L), no schistocytes, moderate fibrinopenia and positive D-dimer test. The results of bone marrow analysis and auto-immune tests were normal. No treatment was undertaken given the absence of diagnosis and complication. The child was regularly followed-up in consultation. Complementary investigations ruled out Wilsons disease, thrombotic thrombocytopenic purpura and constitutional red blood cell disorders. The main hypothesis remained a thrombotic microangiopathy despite absence of schistocytes on repeated blood tests. Two months later, he was rehospitalized for acute clinical and biological worsening with splenomegaly and severe consumption coagulopathy leading to suspect a myeloproliferative syndrome, finally refuted. Steroids were started due to worsening hemorrhagic symptoms, daily transfusion requirements and increasing splenomegaly. Due to worsening health status and risk of threatening rupture, splenectomy was performed and histopathological analysis revealed a vascular lesion infiltrating the whole spleen, compatible with a littoral cell angioma. The child showed rapid clinical improvement and complete hematological normalization within 48 hours post-splenectomy. Conclusion: Littoral cell angioma is a very rare and benign vascular lesion which may be complicated by a life-threatening KMS. This diagnosis should be suspected in patients presenting with splenomegaly and features of thrombotic microangiopathy.

##plugins.themes.bootstrap3.article.details##

Keywords

Kasabach-Merritt syndrome, thrombotic microangiopathy, littoral cell angioma, child

References
1. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol 2002; 24(6):459-462.
2. Verheul HMW, Panigrahy D, Flynn E, et al. Treatment of the Kasabach-Merritt syndrome with pegylated recombinant human megakaryocyte growth and development factor in mice: elevated platelet counts, prolonged survival, and tumor growth inhibition. Pediatr Res 1999; 46:562-565.
3. ORafferty C, ORegan GM, Irvin AD, et al. Recent advances in the pathobiology and management of Kasabach-Merritt phenomenon. Br J Haematol 2015; 171(1):38-51.
4. Croteau SE, Liang MG, Kozakewich HP, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013; 162(1):142-147.
5. Falk S, Stutte HJ, Frizzera G. Littoral cell angioma: a novel splenic vascular lesion demonstrating histiocytic differentiation. Am J Surg Pathol 1991; 15(11):1023-1033.
6. Kasabach HH, Merritt KK. Capillary hemangioma with extensive purpura: report of a case. Am J Dis Child 1940; 59:1063-1070.
7. Haque PD, Mahajan A, Chaudhary NK, et al. Kasabach-Merritt syndrome associated with a large cavernous splenic hemangioma treated with splenectomy: a surgeons introspection of an uncommon, little read, and yet complex problem. Indian J Surg 2015; 77(1):166-169.
8. Louis TH, Sanders JM, Stephenson JS, et al. Splenic hemangiomatosis. Proc Bayl Univ Med Cent 2011; 24(4):356-358.
9. Enjolras O, Mulliken JB, Wassef M, et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000; 42(2):225-235.
10. Taeguyn K, Mi Ryung R, Kee Yang C, et al. Kasabach-Merritt Syndrom arising from a tufted angioma successfully treated with systemic corticosteroid. Ann Dermatol 2010; 22(4):426-430.
11. Hall GW. Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol 2001; 112(4):851-862.
12. Mizutani K, Umaoka A, Tsuda K, et al. Successful combination therapy of propranolol and prednisolone for a case with congenital Kasabach-Merritt syndrome. J Dermatol 2017; doi: 10.1111/1346-8138.13984.
13. Wang Z, Li K, Dong K, et al. Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma and Kasabach-Merritt syndrome. Pediatr Blood Cancer 2014; 61:1518-1519.
14. Wang Z, Li K, Dong K, et al. Refractory Kasabach-Merritt phenomenon successfully treated with sirolimus and a mini-review of the published work. J Dermatol 2015; 42(4):401-404.
15. Wang Z, Li K, Dong K, et al. Successful treatment of Kasabach-Merritt phenomenon arising from kaposiform hemangio-endotheliomas by sirolimus. J Pediatr Hematol Oncol 2015; 37(1):72-73.
16. Ji Y, Chen S, Xiang B, et al: Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: a multicenter retrospective study. Int J Cancer 2017; 141(4):848-855.
17. Delgado M, Pérez-Ruiz E, Alcade J, et al. Anti-angiogenic treatment (Sunitinib) for disseminated malignant haemangiopericytoma: a case study and review of the literature. Case Rep Oncol 2011; 4(1):55-59.
18. Phillips WG, Marsden JR. Kasabach-Merritt syndrome exacerbated by platelet transfusion. J R Soc Med 1993; 86(4):231–232.
19. Tee M, Vos P, Wiseman S, et al. Incidental littoral cell angioma of the spleen. World J Surg Oncol 2008; 6:87.
20. Bisceglia M, Sickel JZ, Giangaspero F, et al. Littoral cell angioma of the spleen: an additional report of four cases with emphasis on the association with visceral organ cancers. Tumori 1998; 84(5):595-599.
How to Cite
Le Rouzic, M.-A., Valduga, J., Phulpin-Weibel, A., Mansuy, L., Contet, A., Fouyssac, F., Athmani, I., Galloy, M.-A., Wassef, M., & Chastagner, P. (2017). Littoral cell angioma of the spleen revealed by a Kasabach-Merritt syndrome in a 22-month-old child. Archive of Clinical Cases, 4(4), Arch Clin Cases 2017; 4(4):186-190. https://doi.org/10.22551/2017.17.0404.10112
Issue
Vol. 4 No. 4 (2017)
Section
Case Reports

Archive of Clinical Cases is protected by copyright and may be used in accordance with copyright and other applicable laws. Content available at www.clinicalcases.eu and our digital applications is intended for personal noncommercial use.

Authors who submit a manuscript for publication in Archive of Clinical Cases agree to the following terms: a. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal. b. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal. c. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) only after the final version of the manuscript was accepted and published, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access). d. It is compulsory that before submission authors ensure that their work was not published in any other medical journals or pending acceptance for publication and that "Archives of Clinical Cases" is the only beneficiary at that moment if their work/case will be accepted by us.

Guidelines for linking to www.clinicalcases.eu a. The main purpose of the site linking to the Archive of Clinical Casess site should be educational. b. Links should be made to the Archive of Clinical Casess home page (www.clinicalcases.eu) or to the articles abstract. c. It is forbidden to use the Archive of Clinical Casess cover by outside organizations unless permission has been granted in advance, notifying our Secretary. d. Material owned by the Archive of Clinical Cases (including the name, logo, cover, and text) may not be used in any manner that may induce the idea or suggest that the Archive of Clinical Cases is in some way recommending a specific company, product or service. e. You must not use or allow others to access or use, all or any part of our Site or the contents and/or applications on it for commercial purposes without our permission. To seek permission to do anything prohibited by or not contained in these TERMS, or which requires our prior consent or agreement, you can contact us.

How to Cite

Le Rouzic, M.-A., Valduga, J., Phulpin-Weibel, A., Mansuy, L., Contet, A., Fouyssac, F., Athmani, I., Galloy, M.-A., Wassef, M., & Chastagner, P. (2017). Littoral cell angioma of the spleen revealed by a Kasabach-Merritt syndrome in a 22-month-old child. Archive of Clinical Cases, 4(4), Arch Clin Cases 2017; 4(4):186-190. https://doi.org/10.22551/2017.17.0404.10112