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Congenital CMV With LAD Type 1 and NK Cell Deficiency

Rai, Narendra MD; Thakur, Neha MD

Journal of Pediatric Hematology/Oncology: August 2013 - Volume 35 - Issue 6 - p 468–469
doi: 10.1097/MPH.0b013e31827e57b2
Original Articles

We report a rare case of congenital cytomegalovirus (CMV) in a patient who was subsequently diagnosed as leukocyte adhesion defect type 1 with natural killer cell deficiency. The clinical course was complicated by severe CMV pneumonitis during the newborn period. Thereafter the infant suffered from recurrent skin infections without pus formation, otitis media, and bronchopneumonia since 3 months of age. The patient had congenital CMV infection as urine and blood plasma was positive for CMV from day 12 onward. Neutrophil chemotaxis studies showed a decrease in directed chemotaxis. Neutrophils were dyspoetic and nonfunctional lacking HLA DR, CD11c, and CD18. Lymphocytes were polyclonal but lacked CD56, CD16, and surface membrane immunoglobulin.

*Department of Pediatrics, Army Hospital Research and Referral

Department of Pediatrics, Lady Hardinge Medical College, Kalawati Saran Children Hospital, New Delhi, India

The authors declare no conflict of interest.

Reprints: Narendra Rai, MD, 13A/11 First Floor, Old Rajinder Nagar, New Delhi 110060, India (e-mail:

Received January 29, 2012

Accepted November 15, 2012

Copyright © 2013 Wolters Kluwer Health, Inc. All rights reserved.