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

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Chloe Kupelian ◽  
Bindu Sathi ◽  
Deepika Singh
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Cervical Lymphadenopathy ◽  
Clinical Findings ◽  
Normocytic Anemia ◽  
Coronary Aneurysms

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.

Abstract 219: Long - Term Prognosis Of Patients With Kawasaki Disease Complicated By Significant Coronary Aneurysm (diameter ≥ 6 mm)

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Ji Seok Bang ◽  
Gi Beom Kim ◽  
Bo Sang Kwon ◽  
Eun Jung Bae ◽  
Chung Il Noh ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Artery Bypass ◽  
Artery Bypass ◽  
Coronary Aneurysm ◽  
Coronary Intervention ◽  
Surgical Interventions ◽  
Coronary Aneurysms ◽  
The Mean

Background: Some patients with Kawasaki disease (KD) develop large coronary aneurysms (diameter ≥ 6mm) and coronary stenosis, leading to ischemic heart disease. This study determined the long-term outcome for patients with Kawasaki disease complicated by significant coronary aneurysm. Methods and Results: From the database in Seoul National University Children’s Hospital between December 1986 and December 2013, medical records of 83 patients (61 men and 22 women) with large coronary aneurysms (diameter ≥ 6mm) were retrospectively reviewed. Information on patient demographics, catheter or surgical interventions, and most recent status was collected. From these data, we calculated the survival rate, cumulative coronary intervention rate, coronary artery bypass graft surgery rate. The mean age at onset was 4.28 ± 2.62 years, and the mean observational period was 13.2 ± 6.5 years. The maximum coronary artery internal diameter ranged from 6.1 to 25mm (median 9mm). Giant coronary aneurysm was 57 patients (68.7%) and large coronary aneurysm (6-8mm) was 26 patients (31.3%). Coronary aneurysms had progressed to coronary artery stenosis and/or complete occlusion in 42 patients (50.6%). The overall freedom from coronary intervention, coronary artery bypass surgery, or coronary thrombi was respectively 85.5%, 85.5%, and 90.3%. Catheter and surgical coronary interventions (median 1 intervention; range 1 to 5 interventions) were performed in 20 patients (24.1%) at 9 month to 18 years after onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8mm vs ≥8mm). Myocardial infarction occurred in 8 (8.98 %) patients. During this study period, 1 patient died and 98% survival rates was seen in our patients group. Conclusion: The long-term survival of patients with Kawasaki disease complicated by large coronary aneurysm is good even though a fourth of patients underwent multiple catheter or surgical interventions. Further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions in large coronary aneurysm (≥ 6mm).

Abstract 192: Long Term Coronary Angiography of Giant Coronary Aneurysms after Kawasaki Disease in Mexican Children

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Luis M Garrido-Garcia ◽  
Jose L Colin ◽  
Alfredo Bobadilla-Aguirre
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Angiography ◽  
Coronary Stenosis ◽  
Unknown Etiology ◽  
Coronary Artery Lesions ◽  
Mexican Children ◽  
Coronary Aneurysms

Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Coronary artery aneurysms (CAA), is one of the most important aspects of this disease. Some patients with KD develop giant coronary aneurysms (z-score > 10) and coronary stenosis, leading to ischemic heart disease. Objective: To determine the outcome of giant coronary artery lesions caused by KD and the value of coronary angiography in the evaluation and follow-up of coronary artery lesions in Mexican children. Materials and Methods: From our Institutional database, 34 patients (23 men and 11 women) who developed giant aneurysms from 1995 to December 2013 were identified. Information on patient demographics, catheter and surgical interventions, and most recent status was collected from medical records. Results: The average age at onset of KD was 13.5 months, and the median observational period was 70 mo. (5 to 163 mo.). During this period 11 patients showed CAA regression, 21 patients persist with CAA and 2 patients died at follow-up. In 9 patients with persistent giant CAA or coronary stenosis we performed cardiac catheterization to evaluate the coronary anatomy and findings of myocardial ischemia. Coronary bypass was performed to alleviate coronary ischemia in 1 patient, this patient developed dilated cardiomyopathy one year after the surgery and died. The overall survival rate in our series is 97%. Conclusions: Despite being a small series, the long-term survival of patients with KD complicated by giant coronary aneurysms in our center is relatively good. However further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions.

Abstract 203: Long-term Outcome Of Kawasaki Disease With Giant Coronary Aneurysms Which Waste Cardiac Follow-up; A Case Report

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Masanori Mizuno ◽  
Masahide Chikada ◽  
Yoshimitsu Tsuzuki ◽  
Hirokuni Ono ◽  
Masaki Arima ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Cardiopulmonary Arrest ◽  
Artery Aneurysm ◽  
Successful Resuscitation ◽  
Long Term Outcome ◽  
Stenotic Lesion ◽  
Coronary Aneurysms

We report 2 cases of Kawasaki disease (KD) which required surgical interventions long after initial diagnosis. Case 1: Twenty-one years-old male. He was diagnosed as KD at the age of 1. Despite he received intravenous gamma-globulin therapy, bilateral giant coronary aneurysms were formed. In addition, multiple aneurysms include abdominal, iliac and axillary arteries were formed simultaneously. Coronary angiography (CAG) was thought to be dangerous because of iliac artery aneurysm formation. He received medical follow-up with aspirin administration only. He referred to our department at the age of 13. Ischemic changes were confirmed on both treadmill exercise test and cardiac scintigraphy. CAG showed 75% right coronary artery (RCA) stenosis and 99% left main trunk stenosis. Coronary artery bypass grafting (CABG) was performed and his postoperative course has been uneventful. Case 2: Thirty-seven years-old male. He was diagnosed as KD at the age of 1. Left giant coronary aneurysms were formed and subsequently aneurysm was occluded. Aspirin was administrated, but he interrupted medication and follow-up for himself. He referred to our institution due to cardiopulmonary arrest during exercise. After successful resuscitation, CAG revealed total occlusion of RCA and 99% left coronary artery stenosis. Emergent CABG was performed and no postoperative sequel has occurred. Both cases didn’t receive sufficient assessment during the follow-up period. As a result, fatal cardiac event was occurred in case 2. Coronary aneurysm tends to change into combine aneurysmal and stenotic lesion. Long time follow up is necessary even if patients have no symptom. In addition, to decrease lost follow up patients, we should explain the risk of sudden cardiac death repeatedly and emphasis necessity of long term follow-up and medication.

scholarly journals Kawasaki disease: case report of a diagnostic dilemma and often a missed diagnosis

2020 ◽  
Author(s):  
Zaib Bin Jawaid ◽  
Jin Ling Du ◽  
Sohail Iqbal ◽  
Lei Zhang
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Cervical Lymphadenopathy ◽  
Blocking Agent ◽  
Diagnostic Dilemma ◽  
Total Occlusion ◽  
Percutaneous Balloon ◽  
Disease Case

Abstract Background  Management of cardiovascular sequelae to Kawasaki disease (KD) is challenging to adult cardiologists. Vasculitis of medium-sized arteries especially coronary arteries often leads to focal intimal thickening and aneurysmal dilatation of one or more coronary arteries. It needs special attention to recognize coronary artery involvement because of potential long-term morbidity and mortality. We present a case of diagnostic dilemma in young adult Chinese male with KD. Case summary  This asymptomatic patient was found to have deep Q waves in anterior leads on screening electrocardiography and was thought to have myocarditis after depiction of wall motion abnormality on echocardiography, later to be confirmed to have left anterior descending artery (LAD) territory infarct on cardiac magnetic resonance imaging. Coronary computed tomography angiogram depicted proximal LAD aneurysm with calcified plaque/thrombus. Additionally, there was an 18 mm giant right coronary artery (RCA) aneurysm with braid-like appearance and soft plaque (mural thrombus). His previous medical history included fever and cervical lymphadenopathy. Because of the high risk he was commenced on long-term low-dose aspirin and β-adrenergic-blocking agent to reduce myocardial oxygen consumption; however, 3 years later, he presented to the emergency department with acute inferior myocardial infarction. He was noted to have total occlusion of the proximal RCA and was treated aggressively with thrombectomy and percutaneous balloon angioplasty followed by medical management with β-blockers, sacubitril/valsartan, clopidogrel, and rivaroxaban without subsequent adverse cardiovascular events. Discussion  Kawasaki disease is one of the main causes of coronary artery disease in young adults and can be easily overlooked.

Kawasaki disease

2020 ◽  
pp. 4590-4597
Author(s):  
Brian W. McCrindle
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Clinical Signs ◽  
Cervical Lymphadenopathy ◽  
Primary Therapy ◽  
Unknown Aetiology ◽  
Coronary Artery Aneurysms ◽  
Persistent Fever ◽  
Intravenous Gammaglobulin

Kawasaki disease is an acute, self-limited, inflammatory vasculitis of unknown aetiology, with a peak incidence under five years of age. Coronary complications can present in adults. Its diagnosis requires persistent fever for five days or more and at least four of the following five clinical signs: (1) non-purulent conjunctivitis, (2) oropharyngeal inflammation, (3) cervical lymphadenopathy, (4) polymorphous exanthem, and (5) erythema of the palms and soles with subsequent desquamation. Coronary artery dilation and aneurysms occur in 15–25% of untreated patients. Its primary therapy is with intravenous gammaglobulin. Persistent and resolving coronary artery aneurysms are the predominant long-term morbidity. In adults, new presentations of myocardial ischaemia with coronary artery aneurysms may suggest a previous episode of Kawasaki disease during childhood.

scholarly journals NUDT15 polymorphism explains serious toxicity to azathioprine in Indian patients with chronic immune thrombocytopenia and autoimmune hemolytic anemia: a case series

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Anup J. Devasia ◽  
Raveen Stephen Stallon Illangeswaran ◽  
Infencia Xavier Raj ◽  
Biju George ◽  
Poonkuzhali Balasubramanian
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Case Series ◽  
Severe Toxicity ◽  
Toxic Side Effect ◽  
Case Presentation ◽  
Oral Ulcers ◽  
Asian Patients ◽  
Life Threatening

AbstractObjectivesAzathioprine (AZA) is a commonly used immunosuppressant in patients with autoimmune diseases. The toxic side effect to AZA (myelosuppression, hair loss, and oral ulcers) are highly unpredictable which can be life threatening if not identified earlier and dose adjustments made or the drug is withdrawn.Case presentationHere we report a case series of five patients with severe toxicity while on treatment with AZA for autoimmune hemolytic anemia (n=1) and Immune thrombocytopenia (n=4). The common thiopurine methyltransferase (TPMT) variants (TPMT*2, *3A, *3B) were not present in these patients. However, all these patients had the NUDT15 415C>T variant that has been reported to explain serious toxicity to thioguanine in Asian patients.ConclusionsOur report suggests pre-emptive genotype-based dosing of AZA could reduce adverse toxicity and hence better outcome.

Abstract 193: Hemophagocytic lymphohistiocytosis following kawasaki disease:Differential Diagnosis in IVIG Refractory Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hyun Ok Jun ◽  
Eun Kyung Cho ◽  
Jeong Jin Yu ◽  
So Yeon Kang ◽  
Chang Deok Seo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Skin Rash ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Symptoms ◽  
Early Recognition ◽  
Cervical Lymphadenopathy ◽  
Inflammatory Disorder ◽  
Laboratory Findings

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb<9.0), hypertriglyceridemia, high level of ferritin was shown and had splenomegaly on physical examination. In the suspicion of HLH, bone marrow biopsy was done and revealed hemophagocytosis, consistent with HLH. A second case of 11-month-old boy admitted for 8-day fever with Kawasaki feature. Although, he showed incomplete feature(fever, skin rash, conjunctival injection, cervical lymphadenopathy), echocardiography showed dilated left main coronary artery(3.5mm) and treated with IVIG. However, 2days after IVIG administration, he was still pyrexial. The laboratory findings fulfilled 5 diagnostic criteria of HLH; bicytopenia(anemia, thrombocytopenia), hypofibrinogenemia, hyperferritinemia, hemophagocytosis in bone marrow, raised level of soluble IL-2 receptor. In both cases, the patients treated according to the HLH protocol 2004, and after that clinical symptoms and laboratory findings were improved. Several causes of febrile illness, EBV, CMV, rubella, parvo-viral infection, for example, were excluded. Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.

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