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Abstract Details

Neurologic Presentation of Hemophagocytic Lymphohistiocytosis
General Neurology
S32 - General Neurology: Advances in Neurology: From the Clinic to the Bench (3:30 PM-3:41 PM)
001
HLH is a rare but serious multisystem condition. HLH can affect the nervous system and neurologic manifestations can rarely predominate. Little is known about the clinical and radiographic characteristics of adult HLH patients with neurologic involvement.
Characterize neurologic involvement in adult hemophagocytic lymphohistiocytosis (HLH).
Using the Mayo Clinic Rochester, Minnesota database, patients seen with HLH between February 1996 and December 2014 were retrospectively reviewed. Demographic and clinical data were assessed.

Out of 99 total HLH patients, fourteen (median age 48 years, range 21-71) were identified as having neurologic symptoms attributable to HLH.  Eight (57%) had neurologic symptoms at disease onset. The majority (64%) presented with encephalopathy but one presented with hemiatixia and one with diplopia. Three patients (21%) had prominent peripheral nervous system involvement. Clinical course was complicated by seizures in three patients, and stroke in two (one ischemic, one hemorrhagic). In most patients, HLH was associated with either malignancy (36%), or infection (36%). Three (21.5%) had no identifiable associated systemic condition.  Of the 9/14 patients who underwent lumbar puncture, the majority (89%) had elevated protein but only three (33%) had pleocytosis. Brain MRI was done in twelve patients with a wide variety of findings. Seven patients were treated with etoposide and dexamethasone, and in six, treatment was directed at the underlying condition. Only two patients were alive at the time of our review.

 

In this large, single-center retrospective series, neurologic manifestations of HLH occurred in 14% of patients. Neurologic symptoms were often present at disease onset, and peripheral nerve involvement, which has only rarely been reported, occurred in 21%. Diagnosis can be delayed due to patients often having multiple medical comorbidities. In addition to standard hematologic workup, MRI and lumbar puncture are important pieces of the evaluation. Patients may improve with treatment, but overall prognosis is poor.
Authors/Disclosures
Micah D. Yost, DO
PRESENTER
No disclosure on file
Derek W. Stitt, MD (Mayo Clinic) Dr. Stitt has nothing to disclose.
Michel Toledano, MD (Mayo Clinic) Dr. Toledano has nothing to disclose.
No disclosure on file
Neeraj Kumar, MD (Mayo Clinic, Dept of Neurology) Dr. Kumar has nothing to disclose.