SESSION TITLE: Infectious Disease Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a disorder characterized by benign proliferation of the mature histiocytes and uncontrolled phagocytosis of the platelets, erythrocytes, lymphocytes and their hematopoietic precursors in the bone marrow giving rise to cytopenias. It is associated with various stimuli-like infections, malignancies and immunocompromised states. Patients with miliary tuberculosis can progress to ARDS and tuberculosis can be one of the cause for hemophagocytic lymphohistiocytosis (HLH). The clinical, imageological and pathological findings of one such case is presented.
CASE PRESENTATION: A 29 year male who returned from USA 6 months back presented with history of high grade fever, shortness of breath, dry cough for the past one month to an another hospital. He was intubated and connected to ventilator in view of ARDS and respiratory failure. He responded to ventilator support and antibiotics for five days. Later again presented with fever and respiratory failure within ten days and reintubated. His investigations revealed leucopenia, thrombocytopenia, raised ferritin and triglycerides. His blood, sputum and urine cultures were sterile. The bone marrow aspiration revealed ill-defined granulomas. The chest x-ray revealed bilateral infiltrates. He was referred to our institute for further management. The HRCT revealed miliary nodules in both lungs. The patient was started on ATT with possibility of disseminated tuberculosis. He later developed spontaneous pneumothorax, ICD was placed and CT guided trucut biopsy of lung was performed. Hispathology revealed necrotizing granulomas with ZN stain showing AFB. Repeat CT showed features of ARDS and was reintubated due worsening respiratory failure. He succumbed to the illness after three weeks.
DISCUSSION: HLH can be familial or acquired (secondary). Secondary HLH can be associated with various stimuli including infections like tuberculosis. The presence fever, cytopenias, splenomegaly, hypertriglyceridemia and hyperferritinemia in present case fulfill the criteria laid down by the Histiocyte Society protocol (2004) for the diagnosis of HLH. Pulmonary tuberculosis was proven by biopsy findings of caseating granulomas with AFB and culture positivity for Mycobacterium tuberculosis. Tuberculosis is a potentially curable but uncommon cause of adult respiratory distress syndrome (ARDS) which is associated with very high mortality. These cases are being increasingly reported from several countries including India. The present case showed alveoli filled with dense exudative material and caseating granulomas which could have resulted in ARDS. The combination of HLH and ARDS in the background of miliary tuberculosis was responsible for rapid downhill course inspite treatment with anti-tuberculous drugs, steroids and ventilator support.
CONCLUSIONS: HLH is rare and fatal complication of military tuberculosis. Awareness, early diagnosis and appropriate treatment will reduce the mortality.
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Reference #2: Subhash HS, Sowmya S, Sitaram U, Cherian AM. Tuberculosis-associated haemophagocytic syndrome. J Postgrad Med 2001;47:220.
Reference #3: Goto S, Aoike I, Shibasaki Y, Morita T, Miyazaki S, Shimizu T, et al . A successfully treated case of disseminated tuberculosis-associated hemophagocytic syndrome and multiple organ dysfunction syndrome. Am J Kidney Dis 2001;38:E19.
DISCLOSURE: The following authors have nothing to disclose: Manmadha Rao Talluri, Shantveer Uppin, Vinathi Paritala, Narendra Kumar, Sundaram Challa, Nageshwar Rao, Bhaskar Kakarla, Paramjyothi Gk
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