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Hemophagocytic Lymphohistiocytosis Triggered by Ho ...
Hemophagocytic Lymphohistiocytosis Triggered by Hodgkin Lymphoma: A Diagnostic and Therapeutic Challenge
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This case describes hemophagocytic lymphohistiocytosis (HLH), a rare, rapidly progressive hyperinflammatory syndrome that can be triggered by hematologic malignancies such as Hodgkin lymphoma. HLH is difficult to diagnose because patients may not meet all classic criteria, especially when critically ill or malnourished. In adults, HLH commonly occurs with hematologic cancers, but Hodgkin lymphoma accounts for fewer than 10% of cases.<br /><br />A 42-year-old woman with recently diagnosed nodular sclerosing Hodgkin lymphoma presented after syncope with severe cachexia and malnutrition. She arrived hypotensive, tachycardic, and febrile, and was treated for distributive and hypovolemic shock with vasopressors and broad-spectrum antibiotics in the ICU. Her condition worsened with jaundice, lactic acidosis, thrombocytopenia, and respiratory failure requiring intubation.<br /><br />By day three, labs showed ferritin of 13,059 ng/mL, hemoglobin 8.7 g/dL, platelets 7,000/μL, fibrinogen 160 mg/dL, and triglycerides 106 mg/dL. Imaging revealed splenomegaly. Although hypertriglyceridemia was absent, clinicians suspected HLH and began chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine. She later developed anuric renal failure requiring plasmapheresis and continuous renal replacement therapy, and received multiple blood product transfusions. Despite aggressive treatment, she died. Postmortem testing showed markedly elevated interleukin-2 receptor alpha.<br /><br />The report emphasizes that HLH may present atypically, particularly in malnourished patients, and that absence of hypertriglyceridemia does not exclude the diagnosis. Early recognition, high clinical suspicion, and prompt empiric treatment are essential. Standard therapy includes corticosteroids, etoposide, and malignancy-directed chemotherapy when tolerated, though etoposide could not be given in this unstable patient.
Asset Subtitle
Mark Soliman
Meta Tag
Author List
Ardit Feinaj, Mark Soliman, Mohamed Swalih, Saif Memon
Category
Clinical Vignettes
Concept
Secondary hemophagocytic lymphohistiocytosis
Concept
HLH-04 criteria
Concept
Hodgkin Lymphoma
Concept
Corticosteroids
Concept
BEP Chemotherapy
Distinguished
Non-Finalist
Presenter Organization
Lakeland Regional Health
Presenting Author
Mark Soliman
Track
Adult
Keywords
hemophagocytic lymphohistiocytosis
HLH
Hodgkin lymphoma
hyperinflammatory syndrome
ferritin
splenomegaly
thrombocytopenia
malnutrition
critical illness
etoposide
Secondary hemophagocytic lymphohistiocytosis
HLH-04 criteria
Hodgkin Lymphoma
Corticosteroids
BEP Chemotherapy
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