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...

Full description

Saved in:
Bibliographic Details
Main Authors: Chloe Kupelian, Bindu Sathi, Deepika Singh
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2021/6640006
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832568580224319488
author Chloe Kupelian
Bindu Sathi
Deepika Singh
author_facet Chloe Kupelian
Bindu Sathi
Deepika Singh
author_sort Chloe Kupelian
collection DOAJ
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.
format Article
id doaj-art-7e1731b99d6643a7a226237dfa1ac0bf
institution Kabale University
issn 2090-6889
2090-6897
language English
publishDate 2021-01-01
publisher Wiley
record_format Article
series Case Reports in Rheumatology
spelling doaj-art-7e1731b99d6643a7a226237dfa1ac0bf2025-02-03T00:58:46ZengWileyCase Reports in Rheumatology2090-68892090-68972021-01-01202110.1155/2021/66400066640006Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki DiseaseChloe Kupelian0Bindu Sathi1Deepika Singh2Department of Pediatrics, Valley Children’s Healthcare, Madera, CA, USADepartment of Pediatric Hematology and Oncology, Valley Children’s Healthcare, Madera, CA, USADepartment of Pediatric Rheumatology, Valley Children’s Healthcare, Madera, CA, USAKawasaki 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.http://dx.doi.org/10.1155/2021/6640006
spellingShingle Chloe Kupelian
Bindu Sathi
Deepika Singh
Autoimmune Hemolytic Anemia and Immune Thrombocytopenia: A Unique Presentation of Kawasaki Disease
Case Reports in Rheumatology
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
url http://dx.doi.org/10.1155/2021/6640006
work_keys_str_mv AT chloekupelian autoimmunehemolyticanemiaandimmunethrombocytopeniaauniquepresentationofkawasakidisease
AT bindusathi autoimmunehemolyticanemiaandimmunethrombocytopeniaauniquepresentationofkawasakidisease
AT deepikasingh autoimmunehemolyticanemiaandimmunethrombocytopeniaauniquepresentationofkawasakidisease