Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disease that often presents with nonspecific findings. A high index of suspicion is necessary to make a prompt diagnosis and prevent fatal disease. A 45-year-old man presented with fever, hypotension, abdominal pain, nausea, and vomiting. Imaging showed hepatosplenomegaly and laboratory tests revealed pancytopenia, increased ferritin, and a cholestatic pattern of injury with elevated alkaline phosphatase and total bilirubin. Due to a history of Crohn disease, systemic lupus erythematous, and rheumatoid arthritis, the patient was on immunosuppressants, including infliximab. After multiple negative cultures, persistent fever, and days of empiric broad spectrum antibiotics, our differential shifted to fever of unknown origin. A liver wedge biopsy revealed areas of sinusoidal dilatation with enlarged, activated macrophages containing erythrocytes and intracytoplasmic iron, consistent with hemophagocytosis due to HLH. The portal tracts showed mixed lymphoplasmacytic inflammation, a prominent bile ductular reaction, periportal fibrosis, and scattered large cells with occasional binucleation and prominent nucleoli. These cells stained positive for Epstein-Barr virus encoding region in situ hybridization, PAX5, CD15, and CD30, and hepatic involvement by classic Hodgkin lymphoma was diagnosed and determined to be the cause of the HLH and cholestatic pattern of injury. Simultaneously, a bone marrow biopsy showed diffuse involvement by Hodgkin lymphoma with a similar staining pattern. Aggressive treatment failed and the patient succumbed to multiorgan failure. HLH is a rare, potentially fatal disease, with nonspecific signs and symptoms, and should be considered in any patient presenting with fever and pancytopenia, especially if they are immune compromised

Abstract 1122‐000128: Imaging Follow‐Up in Carotid Webs: Is There Vascular Remodeling?

Introduction: Carotid web (CaW) is a shelf‐like fibrotic projection at the carotid bulb and constitutes an underrecognized cause of ischemic stroke. Atherosclerotic lesions are known to have dynamic remodeling with time however, little is known regarding the evolution of CaW over time. We aimed to better understand if CaW is a static or dynamic entity on delayed vascular imaging. Methods: This was a retrospective analysis of the CaW database at our comprehensive stroke center, including patients diagnosed with CaW between September 2014 through June 2021. Patients who had at least two good quality CT angiograms (CTAs) that were at least 6 months apart were included (CTAs with CaW and superimposed thrombus were excluded). CaW were quantified with 3‐D measurements using Horos software. This was done via volumetric analysis of free‐hand delineated CaW borders on thin cuts of axial CTA (Figure 1 Panel A). NASCET criteria was used to evaluate the degree of stenosis. Results: Sixteen CaW in 13 patients were identified and included. The median imaging follow‐up window was 16 months (IQR 12–22, range 6–29). Median patient age was 45.5 years‐old, 69% were women, 25% had hypertension, 38% hyperlipidemia, 25% diabetes mellitus, 0% atrial fibrillation, and 13% active smokers. 75% of the included CaW were symptomatic while 25% were asymptomatic. Median volume of CaW on initial CTA (8.52 mm3 [IQR 3.7‐13], range 2.2‐30.4) was comparable to median volume of CaW on most recent CTA (8.47 mm3 [IQR 4.0‐12.8], range 2.3‐29.4; p = <0.001 (Figure 1 Panel B). The CaW volumetric measurement correlation between the initial and most recent CTA was near perfect (rs = ‐0.99, p = <0.001). The median change in measured volume of CaW between first and last CTA was ‐0.19 mm3 [IQR ‐0.6‐0.4], range ‐1‐0.8. Median degree of stenosis was 8.1% [IQR 4.5‐17.1], range 0.4‐31.2. The duration of follow‐up imaging was not correlated with the change in CaW volume (Kendall tau‐b[τb] = ‐0.17, p = 0.93). The initial CaW volume was not found to be correlated to the degree of stenosis (τb = ‐0.08, p = 0.65). Conclusions: The volume of the CaW was not found to change over time, reinforcing the idea that this is a relatively static lesion. The CaW volume was not found to correlate with the degree of stenosis caused by it. Further longitudinal studies with longer follow‐up intervals are warranted

Abstract Number ‐ 10: Stroke Patients with Carotid Artery Web Have High RoPE Scores and Low Frequency of PFO

Introduction PFO‐associated stroke is more common in young patients (<60 years) with less vascular risk factors, and with an infarct pattern consistent with embolic phenomena. These features are included in the Risk of Paradoxical Embolization (RoPE) score in which a high score (≥ 7) indicates a high likelihood of a symptomatic PFO. However, carotid artery webs (CaW) have been reported in patients with the same profile in which a PFO might be detected. In this study, we calculated RoPE score for patients with symptomatic CaW related strokes to identify how many of these patients would have been potentially misclassified as having a PFO‐associated stroke. Methods Patients presenting with ESUS and ipsilateral symptomatic CaW were included. Stroke work up was completed including cervicocranial vascular imaging that was reviewed by a neuroradiologist and an interventional neurologist. Shunt study was done with a TTE, TEE, and/or TCD, all with a bubble study. RoPE score of ≥ 7 was considered high. Results A total of 75 patients fulfilled the inclusion criteria of having an ipsilateral symptomatic CaW as the etiology of ESUS with no competing etiologies aside from PFO. The baseline characteristics are described in the table. The rates of vascular risk factors were generally low which is reflected by a high median RoPE score of 7 [IQR 5‐8], with 52% (n = 39) of patients having a score of ≥ 7. Ten patients (13%) had a PFO, of which 3 had high‐risk features. There was no significant difference in median RoPE score between patients with and without PFO (8 [6‐8] vs 6 [5‐8], p = 0.238), nor in the rate of patients with high RoPE score (78% vs 44%, p = 0.06). Recurrence happened in 16% (n = 12) of the patients and was always ipsilateral to the symptomatic CaW. No significant difference was detected in the rates of recurrence between high vs low RoPE scores (20.5% vs 11.1%, p = 0.351). Patients with a PFO had higher rates of recurrence compared to those without a PFO (40%, n = 4 vs 12.3%, n = 8, p = 0.048); however, none of the PFO patients with a recurrent stroke had a high‐risk PFO. A superimposed thrombus was seen on the CaW in 12.2% (n = 9) and was more commonly seen in patients who had recurrence (36%, n = 4 vs 8%, n = 5, p = 0.024). Conclusions Patients with ESUS from a presumably symptomatic CaW‐related stroke have high RoPE scores. The recurrence rates were high in this population and were always ipsilateral to the side of the CaW including in the PFO population. The PFO is likely incidental in this population despite having a high RoPE score. Neurologists should carefully evaluate the cervical vasculature before concluding that a PFO is stroke‐related and committing patients to PFO treatment