Hemophagocytic lymphohistiocytosis [HLH] is a reactive disorder characterized by generalised non-malignant histiocytic proliferation with prominent hemophagocytosis. It may be either primary [familial (FLH)] or secondary [infection or malignancy associated]. Organisms incriminated for infection associated hemophagocytic syndrome (IAHS) include viruses, bacteria, spirochetes, fungi and parasites. Reports of IAHS associated with tuberculosis in neonates are rare. The authors report a case of perinatal tuberculosis presenting as hemophagocytic lymphohistiocytosis.

A newborn presented at 14 hrs of age with right sided clonic seizures and shrill cry. Magnetic Resonance Imaging of the brain showed left cerebral hemiatrophy with cystic changes in left fronto-parietal lobe and parasylvian region. The Magnetic Resonance Angiography revealed hypoplasia of left supraclinoid Internal Carotid Artery and hypoplasia and irregularity of vessel wall affecting the left Middle Cerebral Artery. Such an early presentation of this rare disorder has not been reported previously. Recognition of this anomaly has important implications during carotid and trans-sphenoidal surgery, in thromboembolic disease, and in the surveillance and detection of associated cerebral aneurysms.

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