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

Melanie Allison, DNP, MSN, RN, ACNP-BC

Executive Editorial Specialist, McGraw-Hill Education

Melanie Allison is the Executive Manager of Education & Learning with McGraw Hill. She earned her Doctor of Nursing Practice (DNP) degree and Post-Master’s Certificate in Nursing Education from The Johns Hopkins University. She earned her Master of Science in Nursing (MSN) degree, specializing as an acute care nurse practitioner (ACNP), from Vanderbilt University. Melanie has more than 20 years of experience as a registered nurse and nurse practitioner in adult cardiology and advanced lipid management. She is a part-time faculty member at a top school of nursing where she has taught for more than 15 years.

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