LETTER TO THE EDITOR Budd–Chiari Syndrome in a Child With Leukocyte Adhesion Deficiency—A Rare Association To the Editor: Thrombosis has rarely been reported in children with leukocyte adhesion deficiency (LAD) type I. We report a case of LAD type I with Budd–Chiari syndrome (BCS), a hitherto unknown complication. A 3.5-month-old female presented with abdominal distension. She was born of a non-consanguineous marriage with birth weight of 3.5 kg. At 15 days of life, she developed foul smelling purulent umbilical discharge and the umbilical cord separated by day 20. She was treated elsewhere with topical and oral drugs; however, the umbilical discharge continued unabated. At 2.5 months of age, she developed abdominal distension and later developed facial and limb edema. At admission, she was pale and tachypneic, and had anasarca. Her abdomen was distended with dilated veins (Fig. 1) and erythema was noted over umbilicus but there was no discharge. Fluid thrill was noted on physical examination but organomegaly could not be appreciated. Bilateral crepitations were noted on chest examination. Laboratory investigations revealed anemia (hemoglobin 50 g/L), persistent neutrophilic leukocytosis (total leukocyte count 52.52  10 9 /L, absolute neutrophil count 32.55  10 9 /L) and thrombocytopenia (platelet count 10  10 9 /L). Platelet counts improved during the hospital stay (123  10 9 /L). Bone marrow examination was normal. Immunological tests: IgM >3.97 g/L (0.2–1), IgG 16.57 g/L (2.4–8.8), IgA 1.88 g/L (0.1–0.5), CD3 90.4% (48–75), CD19 2% (14–39), CD56 9% (2–14). CD18 expression was absent on flow cytometry (0.1% in case vs. 90.8% in control, which was an age-matched, healthy child). Doppler examination of abdominal vessels showed normal portal veins. Magnetic resonance imaging of the abdomen with angiography (MRA) revealed thrombosis of hepatic veins. Computed tomography with pulmonary angiography excluded pulmonary thrombosis. Investigations for a pro-coagulant state did not show any protein S, protein C, or ATIII deficiency. There was no Factor V Leiden mutation, and b2-glycoprotein and anti-cardiolipin antibodies were not detected. She was treated with broad spectrum antimicrobials. Abdominal distension gradually decreased and after a stay of 6 weeks, she was discharged on oral antimicrobials and anti-coagulation. In LAD, the severity of infections is related to the severity of CD18 deficiency; cases with <1% expression are clinically severe, whereas those with 2.5–10% expression are mild to moderate.[1] This child represented the severe form of LAD. For diagnosis of BCS, colour doppler ultrasound examination is the initial investigation; however, it has a sensitivity of 75% and is operator dependent. MRA is superior and is the investigation of choice.[2] A child with LAD type I who developed ascites has been previously reported, but this was secondary to septicemia and renal failure.[3] Thrombotic complications have been reported in the phagocyte disorder chronic granulomatous disease,[4] but not in children with LAD. When children with LAD present with ascites, a possibility of Budd–Chiari syndrome should be considered in the differential diagnosis. Further studies are warranted to look for the pathogenic mechanism of this association. Sagar Bhattad, MD, Amit Rawat, MD, MAMS  , Anju Gupta, MD, Deepti Suri, MD, DNB, Surjit Singh, MD, DCP, FRCPCH Pediatric Allergy and Immunology Unit Advanced Pediatrics Centre Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India Biman Saikia, MD, MNAMS, Ranjana W. Minz, MD, DNB Department of Immunopathology Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India Kushaljit S. Sodhi, MD, MAMS Department of Radiodiagnosis Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India REFERENCES 1. Etzioni A. Genetic etiologies of leukocyte adhesion defects. Curr Opin Immunol 2009;21:481–486. 2. Zhou P, Ren J, Han X, Wu G, Zhang W, Ding P. Initial imaging analysis of Budd-Chiari syndrome in Henan province of China: Most cases have combined inferior vena cava and hepatic veins involvement. PLoS ONE 2014;9:e85135. 3. Kavehmanesh Z, Matinzadeh ZK, Amirsalari S, Torkaman M, Afsharpayman S, Javadipour M. Leukocyte adhesion deficiency: Report of two family related newborn infants. Acta Med Iran 2010;48:273–276. 4. De Ravin SS, Naumann N, Cowen EW, Friend J, Hilligoss D, Marquesen M. Chronic granulomatous disease as a risk factor for autoimmune disease. J Allergy Clin Immunol 2008;122:1097–1103. Fig. 1. Distended abdomen with dilated veins in the child with LAD. Conflict of interest: Nothing to declare Written informed consent taken from parents of child. Study approved by Department Review Board.  Correspondence to: Amit Rawat, Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgrad- uate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh 160012, India. E-mail: rawatamit@yahoo.com Received 6 May 2015; Accepted 21 May 2015  C 2015 Wiley Periodicals, Inc. DOI 10.1002/pbc.25630 (wileyonlinelibrary.com). Published online 7 July 2015 in Wiley Online Library Pediatr Blood Cancer 2015;62:2244