SESSION TYPE: Critical Care Cases II
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Hemophagocytic Lymphohistiocytosis (HLH) also termed as hemophagocytosis has been associated with a variety of viral, bacterial, fungal, and parasitic infections, as well as collagen-vascular diseases and malignancies. HLH in association with Ehrlichia Chaffeensis has been reported in children but not in adults1. We present a case of Ehrlichiosis in an adult complicated with Hemophagocytosis.
CASE PRESENTATION: A 74 year old Caucasian male presents to emergency room with 1 week history of gradually worsening dyspnea, high fever, nausea and lethargy. Initial laboratory data apart from acute kidney injury (BUN:74 mg/dl, Creatinine: 3.4 mg/dl) was significant for coagulopathy (INR:7.8, PTT: 50, D dimer: 2.63 ug/ml, fibrinogen: 263 mg/dl), thrombocytopenia (Platelets:16,000), elevated liver enzymes (AST:261 U/L, ALT:78 U/L, ALP: 60 U/L, total bilirubin: 1.8 mg/dl, LDH: 1398 mg/dl) and anemia with leucopenia (Hb: 10 mg/dl, Hct: 30%, WBC:4100/uL). Further history revealed that he had a tick bite while camping 1 week prior to the symptom onset and hence, was started on doxycycline. Clinical suspicion of HLH was made based on very high levels of ferritin (12000 U) and high triglycerides (387 mg/dl). A bone marrow aspirate was obtained which showed the presence of reactive marrow along with hemophagocytosis (presence of macrophage with engulfed nucleated red blood cell) as shown in the Fig 1a. Fig 1b reveals a hemosiderrin laden macrophage. Tick borne serologies were reported positive for Ehrlichia PCR (Ehrlichia chaffeensis DNA. The patient’s combination of clinical features and laboratory evaluation fulfilled the revised diagnostic criteria for HLH2. His clinical condition improved with supportive management and doxycycline with no other proven therapies for HLH.
DISCUSSION: Hemophagocytic lymphohistiocytosis (HLH) is a rare complication of infectious diseases and has not been reported in adults with Ehrlichia Chaffeensis to best of our knowledge. (HLH) is a disease with major diagnostic and therapeutic difficulties.
CONCLUSIONS: The most typical features of HLH are fever, hepatosplenomegaly, lymphadenopathy, skin rash, jaundice, cytopenias, as well as hypertriglyceridemia, coagulopathy with low fibrinogen levels, liver dysfunction, and elevated levels of ferritin and serum transaminases. Pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leucocytes, platelets and their precursors) is a distinct clinical feature.
1) Hemophagocytic lymphohistiocytosis secondary to Ehrlichia chaffeensis infection: a case report. Burns S, Saylors R, Mian A ; J Pediatr Hematol Oncol 2010
2) Henter JI, Horne A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007
DISCLOSURE: The following authors have nothing to disclose: Sridhar Badireddi, Manish Joshi
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