SHM Converge Scientific Abstract ePoster Gallery-Hemophagocytic Lymphohistiocytosis

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This case describes a rare postpartum presentation of hemophagocytic lymphohistiocytosis (HLH), a severe and often fatal immune activation syndrome that requires prompt recognition and treatment. In adults, HLH is usually secondary to an underlying trigger such as infection, autoimmune disease, or malignancy, but in this patient no secondary cause was identified aside from the postpartum state.<br /><br />A 27-year-old woman with no significant past medical history developed acute kidney injury, metabolic acidosis, worsening liver enzymes, coagulopathy, anemia, thrombocytopenia, and undifferentiated shock requiring vasopressors on postpartum day 6. Peripheral blood smear findings suggested a strong inflammatory process. Laboratory studies showed markedly elevated ferritin, peaking at 46,812 ng/mL, as well as elevated soluble IL-2 receptor and triglycerides. Bone marrow biopsy demonstrated numerous histiocytes with frequent hemophagocytosis, confirming the diagnosis of HLH.<br /><br />She was started on the HLH-2004 treatment protocol with etoposide, cyclosporine, and dexamethasone. Shortly after treatment began, she developed encephalopathy and generalized seizures requiring mechanical ventilation. Brain MRI showed changes consistent with posterior reversible encephalopathy syndrome (PRES). The cause of PRES was uncertain, but cyclosporine was temporarily held because of concern it may have contributed.<br /><br />Extensive evaluation for infectious, rheumatologic, malignant, and familial causes was unrevealing. Despite her severe presentation, her condition improved rapidly with treatment. She was extubated after 7 days, discharged on treatment day 21, and continued outpatient therapy with ongoing clinical and laboratory improvement.<br /><br />The case highlights that postpartum HLH, though rare, should be considered in patients with unexplained multi-organ failure and marked hyperinflammation after delivery. Early diagnosis is especially important because central nervous system involvement worsens prognosis, and timely treatment can be lifesaving.

Asset Subtitle

Daniela Urueta Portillo

Meta Tag

Author List Daniela Urueta Portillo, Ian Mines, Miguel Ocampo, Zohra Nooruddin

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept HLH-04 criteria

Concept Diagnostic Criteria

Concept Postpartum Hemophagocytic Lymphohistiocytosis

Concept Ferritin

Distinguished Non-Finalist

Presenter Organization UT Health SA

Presenting Author Daniela Urueta Portillo

Track Adult

Keywords

postpartum HLH

hemophagocytic lymphohistiocytosis

hyperinflammation

ferritin

hemophagocytosis

posterior reversible encephalopathy syndrome

PRES

bone marrow biopsy

multi-organ failure

etoposide cyclosporine dexamethasone

Hemophagocytic Lymphohistiocytosis

HLH-04 criteria

Diagnostic Criteria

Postpartum Hemophagocytic Lymphohistiocytosis

Ferritin