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Richard M. Tempero, MD, PhD; Mark Hannibal, MD, PhD; Laura S. Finn, MD; Scott C. Manning, MD; Michael L. Cunningham, MD, PhD; Jonathan A. Perkins, DO

Arch Otolaryngol Head Neck Surg. 2006;132:93-97.

Objective  To determine whether an immunologic abnormality exists in patients with lymphatic malformation (LM).

Design  Retrospective case series.

Setting  Tertiary care pediatric hospital.

Patients  Twenty-one consecutive patients (11 male and 10 female) undergoing LM treatment.

Interventions  Clinical data (ie, age, clinical LM stage, radiographic appearance, and histologic findings) were correlated with complete blood cell count and detailed lymphocyte differential. Complete blood cell counts and lymphocyte subsets were measured in 21 and 18 patients, respectively.

Results  The average age at the time of testing was 67 months (range, 1-231 months). The patients were categorized according to LM stage, including 4 (19%) with stage 1, 4 (19%) with stage 2, 4 (19%) with stage 3, 7 (33%) with stage 4, and 2 (10%) with stage 5 disease. Radiographic LM appearance was macrocystic in 6 patients (29%), mixed macrocystic and microcystic in 8 (38%), and microcystic in 7 (33%). Complete blood cell count data demonstrated lymphocytopenia in 6 patients (29%). The results of the lymphocyte subset tests showed concomitant T-, B-, and natural killer (NK)–cell deficiency in 6 (33%) of 18 patients. All 6 patients with T-cell lymphocytopenia had normal neutrophil and platelet counts. Spearman rank and ² analyses showed that LM stage 4 or 5 and microcystic LM were significantly associated with lymphocytopenia (P = .002 and P = .008, respectively). Histologic analysis did not demonstrate increased lymphocytes in any LM specimens.

Conclusion  We found T, B, and NK lymphocytopenia in patients with large bilateral or microcystic LM. Although the relationship between lymphocytopenia and infection was not addressed in this study, the recognition of lymphocytopenia in patients with LM may have important clinical and prognostic implications.

Author Affiliations: Departments of Otolaryngology–Head and Neck Surgery (Drs Tempero, Manning, and Perkins) and Pediatrics (Drs Hannibal and Cunningham) and Division of Genetics and Developmental Medicine (Dr Hannibal), University of Washington, and Department of Pathology (Dr Finn), Division of Pediatric Otolaryngology (Drs Manning and Perkins), and Children’s Craniofacial Center (Drs Cunningham and Perkins), Children’s Hospital and Regional Medical Center, Seattle.