All That Childhood Coronary Artery Aneurysms Are Not Kawasaki Disease!
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SCIENTIFIC LETTER
All That Childhood Coronary Artery Aneurysms Are Not Kawasaki Disease! Darshan Rangaswamy 1 & Jaikumar Govindaswamy Ramamoorthy 1 & Sainadh Mukarjee 1 & Dhandapany Gunasekaran 1 & Avinash Anantharaj 2 & Prabhu Manivannan 3 & Venkataraman Ranjith Kumar 1 Received: 6 July 2020 / Accepted: 17 August 2020 # Dr. K C Chaudhuri Foundation 2020
To the Editor: A 10-y-old boy presented with high grade hectic pattern fever (104 °F) and left hypochondrial abdominal pain for 5 d. On examination, moderate hepatosplenomegaly (6 cm below costal margin) without significant lymphadenopathy was observed. Hemoglobin was 8.7 g/dl, platelet counts of 47 × 103/μL and total leucocyte count (TLC) was 3.75 × 10 3 /μL with 40% neutrophils in complete hemogram. Peripheral smear was non-contributory. Blood and urine culture, evaluation for tropical infections like typhus fever, dengue and malaria, serological tests for Epstein-Barr virus and parvo virus and polymerase chain reaction for cytomegalovirus were all non-contributory. Erythrocyte sedimentation rate was 70 mm/h and C-reactive protein was 6 mg/dl. The fever was persistent and the TLC had decreased to 0.18 × 103/μL with absent blast cells in smear examination. Bone marrow evaluation showed: 93% periodic acid Schiff positive lymphoid blasts and pre-B cell acute lymphoblastic leukemia (ALL). Echocardiogram performed on day 10 of fever for pre-chemotherapy cardiac evaluation showed left main coronary artery (LMCA) aneurysm (2.5 Z-score) with normal cardiac contractility (Fig. 1).
* Jaikumar Govindaswamy Ramamoorthy [email protected] 1
Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry 605006 India
2
Department of Cardiology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry India
3
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry India
Coronary artery aneurysm (CAA) is diagnostic of Kawasaki disease (KD) with 98% specificity and age ≥ 10 y and presence of thrombocytopenia are significant risk factors to develop CAA [1]. Increased levels of matrix metalloproteinase-9 (MMP-9) is considered pathogenetic of CAA in KD [2]. MMP-9 is secreted by lymphoid blasts and it is believed, MMP-9 secreted disrupts the blood-brain-barrier and facilitates leukemic progression [3]. Coronary artery dilatation or CAA has been rarely observed in children with non-kawasaki febrile illness (NKFI). In a cohort of 43 children, 1 child with osteomyelitis had CAA (2.8 Z-score) [4]. Three children with febrile systemic onset juvenile idiopathic arthritis have been reported with CAA and 1 of the child had isolated LMCA aneurysm (3.2 Z-score) similar to our case [5]. In our child, CAA could have developed because of increased MMP-9 secretion by lymphoid blast or as a compensatory response to fever induced escalated myocardial oxygen demand or an immune response to pathogenic protein bound to coronary endothelium [2, 4].
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