Hemophagocytic Lymphohistocytosis is a rare, aggressive and life threatening condition characterized by excessive immune activation. In view of a variable clinical presentation, lack of specificity of..
Hemophagocytic Lymphohistocytosis is a rare, aggressive and life threatening condition characterized by excessive immune activation. In view of a variable clinical presentation, lack of specificity of the clinical and laboratory findings and outcomes limited by delay in identification warrants discussion to help in this challenging diagnosis. A 57 year old female initially presented for a CT of the chest after failed outpatient treatment for pneumonia; imaging denoted diffuse lymphadenopathy, she followed up with Hematology/Oncology, having two fine needle aspirations with inconclusive results. Approximately 2 months later she presented to the ED with progressive shortness of breath, hypoxia and chest pain associated with fatigue and cough. Physical exam was significant for only bilateral crackles at the lung bases. She was diagnosed with a subsegmental PE after imaging, pneumonia and lactic acidosis, and was subsequently admitted to the MICU and started on broad-spectrum antibiotics and heparin. General surgery was consulted for excisional biopsy of the lymph node, resulting in a T Cell Lymphoma with underlying B Cell Lympohma. In post-op she became tachycardiac, tachypneiac and hypoxic. Over the next 5 days, she became anemic, thrombocytopenic, febrile, hypercalcemic, hyperuricemiac and hypofirbinogenemic, and develope d splenomegaly with worsening lactic acidosis. HLH was ultimately identified with a severely elevated Ferritin level and elevated IL-2 (CD25) receptor, at which time she was also exhibiting tumor lysis syndrome and hemodynamic instability; subsequently leading to the patients’ death. Although rare, this case illustrates the necessity for early identification of HLH and initiation of treatment, especially in the setting of an unclear clinical picture delaying diagnosis.