Clinical outcomes in lymphocytopenic lymphatic malformation patients

To determine if lymphocytopenia in patients with lymphatic malformation (LM) is associated with rates of infection and poor clinical outcomes.

This is a retrospective case series at a tertiary pediatric hospital, of 21 consecutive patients (11 male and 10 female) undergoing LM treatment. Clinical data (i.e., age, clinical LM stage, presence of tissue hypertrophy, frequency/type of medical therapy, and number of hospitalizations) obtained from LM patients with lymphocytopenia (n = 6) was compared to LM patients without lymphocytopenia (n = 15).

The average age at the time of detailed leukocyte analysis was 67 months (Range 1-231). Six patients with lymphocytopenia (below 1500/cm(3)) were compared with 15 without lymphocytopenia (above 1500/cm(3)). All six patients with lymphocytopenia had large bilateral LM and normal neutrophil and platelet counts. The total number of hospital admissions was two times greater in lymphocytopenic patients (mean 8.3) compared to nonlymphocytopenic patients (mean 4.09) Chi square analysis revealed a statistical difference in lymphocytopenic patients. They were more likely to have had central line placement, central line infection, bacteremia, prophylactic antibiotics, admission at birth, infections distant from the lymphatic malformation and a treatment complication compared to nonlymphocytopenic patients. Univariate logistic regression revealed that, independent of LM stage, the use of prophylactic antibiotics, the need for a central line, the occurrence of a line infection, and the hospital admission rate were significantly increased in lymphocytopenic patients.

Patients with LM-associated lymphocytopenia have increased hospitalization requirements, rate of infection, and receive more intensive antibiotic therapy compared to nonlymphocytopenic LM patients.