Amit Dey

Tuberculosis with Hemophagocytic Lymphohistiocytosis in an Infant

To the Editor: Tuberculosis (TB) with Hemophagocytic Lymphohistiocytosis (HLH) has been rarely reported in infants. [1] A 2-mo-old boy born of third degree consanguineous marriage presented with fever for 15 d, distention of abdomen and increased respiratory activity for 3 d. There was no contact with a patient having TB. On examination, he had tachycardia, tachypnea with subcostal retractions. On systemic examination, he had hepatosplenomegaly. His initial investigations showed hemoglobin of 8.3 g%, white cell count of 1580/cumm (35 % polymorphs, 65 % lymphocytes), platelet count of 1,57,000/cumm, C-reactive protein (CRP) of 141 mg/L, SGOT of 572 IU/L, SGPT of 350 IU/L, LDH of 8750 mg/L. Blood gases showed PaO2 of 52.6 and oxygen saturation of 84.1 without acidosis. Chest radiograph showed miliary pattern (Fig. 1). Ultrasound abdomen was suggestive of hepatosplenomegaly with mild ascitis. HIV ELISA was negative. Bone marrow examination showed hemophagocytes engulfing nucleated red blood cells and platelets. There were no granulomas and no acid fast bacilli (AFB) on the marrow. Plasma fibrinogen was 108 mg/dL (normal=82–382 mg/dL), serum ferritin was 52269 ηg/mL (normal=32–233 ηg/mL), serum triglycerides were 297.75 mg/dL (normal=42–279 mg/dL). D-dimers were 6.96 μg/mL (normal 0.11–0.49 μg/mL) and serum perforin was 85 %. Bronchoscopy could not be done due to poor condition of the patient. He was started on cyclosporine (6 mg/kg/d) and dexamethasone (10 mg/m/d). On the next day, child had respiratory failure, was intubated and put on ventilator. Bronchial lavage was taken from endotracheal tube which showed 2+ AFB on smear. Patient was started on 4 drug antituberculous therapy (ATT) consisting of ethambutol, ofloxacin and streptomycin. Isoniazid, Rifampicin, Pyrazinamide were avoided in view of liver dysfunction. However, he succumbed to his illness on day 3 of hospitalization. Several cases of tuberculosis have been reported as cause of HLH with most of the patients having underlying conditions like cancer, diabetes and other immunocompromised states. [2] The mechanism of tuberculosis causing HLH is not clearly understood. It is presumed that during phagocytosis of mycobacteria cytokines are released which perpetuates the host immune response and leads to the recruitment of lymphocytes, neutrophils and monocytes which when gets magnified leading to a impaired immune system leading to HLH. [1] Tuberculosis associated hemophagocytic syndrome carries a higher mortality rate than virus related HLH. It is noted that survival rate is only 16.7 % among HIV infected patients along with tuberculosis associated HLH. [3] Similarly in our patient, he succumbed to his illness.

Disseminated tuberculosis with varied paradoxical reactions

Paradoxical reactions are immune-mediated exacerbations of disease triggered by tuberculosis (TB) treatment. We describe a case of varied paradoxical reactions in a 9-year-old girl who was diagnosed with mediastinal TB and tuberculous ascites. The development of paradoxical reaction was gradual with pericardial effusion occurring first followed by pleural effusion and subsequently bilateral papilledema.

Infantile Budd Chiari and Response to Balloon Dilatation

To the Editor: Angioplasty in infants with Budd-Chiari syndrome (BCS) is difficult due to small vessels and has poor prognosis [1]. Two infants presented with acute onset ascites and hepatomegaly without splenomegaly. Both were confirmed to have BCS on Magnetic resonance (MR) or computed tomography (CT) angiography, were started on low molecular weight (LMW) heparin and had successful outcome with angioplasty. Investigations are depicted in Table 1. Patient 1 underwent venoplasty of middle hepatic vein (MHV) as right hepatic vein (RHV) and left hepatic vein (LHV) could not be cannulated. Currently patient 1 is 2 1⁄2 y of age. Patient 2 underwent right and left hepatic venoplasty as MHV could not be cannulated. He was shifted to warfarin but INR remained below 1.5 despite dose escalation. Six months later, he again had narrowing in proximal portion of RHV. He underwent a repeat angiography that showed 90 % re-stenosis of RHV and 95 % re-stenosis of LHV with multiple intrahepatic collaterals. Again left and right hepatic venoplasty was done and he was put on low molecular weight heparin and is doing well till last follow-up at 3 y of age. BCS is a rare, life-threatening disease caused by obstruction of hepatic venous outflow usually without significant liver parenchyma dysfunction. Doppler ultrasound by an experienced examiner, is the most effective and reliable diagnostic modality. MR or CT imaging confirms the diagnosis, being most useful in the absence of an experienced doppler ultrasound examiner [2]. A step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPSS), and orthotopic liver transplantation (OLT) provides good long-term survival in adults [3]. In a study of 16 children with BCS with a median age of 22mo, one of the hepatic vein patency could be established by TIPSS or angioplasty in 11 children [4]. In another study, out of 13 children with BCS, 6 underwent angioplasty and 3 underwent TIPSS [5]. However, intervention in infancy is difficult owing to the small blood vessel size and issue of restenosis. A strong suspicion of BCS in infants presenting with acute onset ascites should be considered. Early intervention with anticoagulation and angioplasty may lead to good prognosis.