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Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease

Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, muc...

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Autores principales: Kupelian, Chloe, Sathi, Bindu, Singh, Deepika
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7936906/
https://www.ncbi.nlm.nih.gov/pubmed/33728087
http://dx.doi.org/10.1155/2021/6640006
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author Kupelian, Chloe
Sathi, Bindu
Singh, Deepika
author_facet Kupelian, Chloe
Sathi, Bindu
Singh, Deepika
author_sort Kupelian, Chloe
collection PubMed
description Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans' syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings.
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spelling pubmed-79369062021-03-15 Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease Kupelian, Chloe Sathi, Bindu Singh, Deepika Case Rep Rheumatol Case Report Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans' syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings. Hindawi 2021-02-27 /pmc/articles/PMC7936906/ /pubmed/33728087 http://dx.doi.org/10.1155/2021/6640006 Text en Copyright © 2021 Chloe Kupelian et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kupelian, Chloe
Sathi, Bindu
Singh, Deepika
Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title_full Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title_fullStr Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title_full_unstemmed Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title_short Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
title_sort autoimmune hemolytic anemia and immune thrombocytopenia: a unique presentation of kawasaki disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7936906/
https://www.ncbi.nlm.nih.gov/pubmed/33728087
http://dx.doi.org/10.1155/2021/6640006
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