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Double Trouble: Lymphocyte-Depleted Classical Hodg ...
Double Trouble: Lymphocyte-Depleted Classical Hodgkin Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis
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This case describes a 65-year-old woman who presented with three months of shortness of breath, weight loss, fever, hypotension, and tachycardia. Her labs showed severe anemia, thrombocytopenia, and markedly elevated ferritin, raising concern for hemophagocytic lymphohistiocytosis (HLH). Her H-score was 187, supporting a high probability of HLH.<br /><br />HLH is a rare, life-threatening hyperinflammatory syndrome caused by excessive immune activation and can progress to multiorgan failure. Diagnosis is typically made when 5 of 8 HLH criteria are met, including fever, splenomegaly, cytopenias, hypertriglyceridemia or hypofibrinogenemia, hemophagocytosis, low NK-cell activity, hyperferritinemia, and elevated soluble IL-2 receptor. In this case, persistent fever, very high ferritin, liver dysfunction, cytopenias, triglyceride elevation, and elevated soluble IL-2 receptor supported the diagnosis.<br /><br />Dexamethasone was started according to the HLH-2004 protocol, but the patient rapidly worsened, developing respiratory failure and shock requiring intubation and vasopressors. CT imaging showed extensive lymphadenopathy, and biopsy of a left supraclavicular node confirmed lymphocyte-depleted classical Hodgkin lymphoma (LDCHL). Bone marrow biopsy showed no lymphoma involvement.<br /><br />The case highlights that HLH may be triggered by an underlying malignancy, especially lymphoma, and that patients with HLH should be evaluated promptly for cancer. LDCHL is a rare and aggressive subtype of Hodgkin lymphoma associated with poor outcomes. Management is challenging because both the inflammatory syndrome and the cancer must be addressed, often requiring a multidisciplinary approach.
Asset Subtitle
Jessica Igiede
Meta Tag
Author List
Jessica Igiede, Melissa Gunchenko, Michelle Sweet
Category
Clinical Vignettes
Concept
Secondary hemophagocytic lymphohistiocytosis
Concept
HLH-04 criteria
Concept
Cytopenia
Concept
Lymphoma
Concept
Malignancy-Associated Hemophagocytic Lymphohistiocytosis
Distinguished
Non-Finalist
Presenter Organization
Rush Medical College
Presenting Author
Jessica Igiede
Track
Adult
Keywords
hemophagocytic lymphohistiocytosis
HLH
H-score
lymphocyte-depleted classical Hodgkin lymphoma
LDCHL
hyperferritinemia
cytopenias
dexamethasone
lymphadenopathy
multiorgan failure
Secondary hemophagocytic lymphohistiocytosis
HLH-04 criteria
Cytopenia
Lymphoma
Malignancy-Associated Hemophagocytic Lymphohistiocytosis
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