Acquired Hemophagocytic Lymphohistiocytosis (HLH)?

Acquired Hemophagocytic Lymphohistiocytosis (HLH)?
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What You Need to Know:

ESPN college football reporter, Edward Aschoff died December 24th following hospitalization for a presumed diagnosis of hemophagocytic lymphohistiocytosis (HLH) and pneumonia. HLH is an aggressive, life-threatening, rare disease that is either familial (25%) or acquired (75%). Diagnosis of familial HLH is typically made in infancy or early childhood, whereas acquired HLH may manifest in adulthood simultaneously with an infection such as Epstein Barr Virus (EBV), cancer, or in patients with a weakened immune system. It is unknown whether or not Aschoff suffered from additional health problems that could have contributed to his untimely death.

The hallmark of HLH is continuous activation of CD8+ T lymphocytes and macrophages that cause liver, bone marrow, central nervous system, and other organ damage. Symptoms of HLH are vague and mimic many other diseases, so misdiagnosis or delayed diagnosis is common. The most prevalent symptoms include fever and splenomegaly. Additional symptoms may include enlarged liver, skin rash, jaundice, lymphadenopathy, cough, shortness of breath, nausea, vomiting, diarrhea, headaches, visual disturbances, ataxia, and generalized weakness. Lab tests may reveal elevated liver enzymes, blood cytopenia, hypertriglyceridemiahypofibrinogenemia, elevated ferritin, and increased markers of T cell activation. Hemophagocytosis can be seen in bone marrow aspirate or cerebrospinal fluid. 


Read More About Acquired HLH:

Fitzpatrick’s Dermatology, 9e: Chapter 117. Histiocytosis

Williams Hematology, 9e: Chapter 71. Inflammatory and Malignant Histiocytosis

Principles and Practice of Hospital Medicine, 2e: Chapter 170. Disorders of the White Cell

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