Hemophagocytic Lymphohistiocytosis (HLH) – The Great Mimicker: Case Report

Rationale and objective: HLH is the aggressive proliferation of activated macrophages and histiocytes phagocytosing blood cells. [1] This report demonstrates the clinical and laboratory approach and therapeutic management to a patient suspected of HLH. Case: This is a case of an 18-year old female with nine-month history of on and off fever. She was previously admitted and diagnosed with erythema nodosum probably secondary to pulmonary tuberculosis, congenital heart disease, atrial septal defect. She was discharged but there was still note of intermittent fever, tachycardia, progressive edema on upper and lower extremities, and increasing abdominal girth, hence readmission. Initial laboratory work up showed anisopoikilocytosis with a predominance of microcytic, hypochromic red blood cells, schistocytes and elliptocytes were seen with few burr cells and nucleated RBCs, normal WBC count, and with toxic granulation. Bone marrow aspiration was done revealing erythrophagocytosis and hemophagocytic lymphohistiocytosis. Patient was started with cotrimoxazole and fluconazole, induction therapy with corticosteroid-dexamethasone, chemotherapy with etoposide and cyclosporine, and blood transfusion. Clinical signs, symptoms, and laboratory parameters improved. Discussion and Summary: HLH does not have specific manifestations as it is capable of mimicking other diseases which contributes to its high mortality rate. With this, a high index of suspicion and a thorough clinical, immunological, and genetic workups are required. As in our patient, HLH should be considered when there is persistent high fever and cytopenias. With prompt recognition and appropriate treatment, prognosis may be improved.