Objective:

To discuss a rare case of central pontine myelinolysis (CPM) in a patient with hepatosplenic T cell lymphoma (HSTL) and hemophagocytic lymphohistiocytosis (HLH).

Background:

HSTL is a rare disease characterized by infiltration of mature T cells within the sinusoids of the spleen, liver, and bone marrow. Hyponatremia prior to orthotopic liver transplant for liver failure has previously been identified as a risk factor for the development of CPM due to possible disruption of astrocyte metabolism and impaired capacity to regulate osmotic changes leading to secondary dysfunction of the blood brain barrier. CPM has also previously been discovered in EBV positive HLH.

Design/Methods:

We conducted a case report and literature review with PubMed search terms: 1) “central pontine myelinolysis,” “hyponatremia,” and “liver” 2) “central pontine myelinolysis,” “hemophagocytic lymphohistiocytosis,” and “hemophagocytic syndrome” 3) “central pontine myelinolysis” and “hepatosplenic T cell lymphoma.” 

Results:

A 20-year-old EBV negative male presented with fever, splenomegaly, and blurry vision with bilateral retinal hemorrhages. Workup was significant for pancytopenia, mild hypovolemic hyponatremia secondary to emesis (initially 132 mmol/L), triglycerides 377, INR 1.2, ALT 55, and ALP 402. HSTL and HLH were confirmed on bone marrow biopsy. He was found to have subclinical CPM on MRI brain without contrast which resolved on 2 month follow-up imaging after 3 rounds of ifosfamide, carboplatin, and etoposide (ICE) intrathecal chemotherapy. We found no prior literature describing HSTL and CPM, 22 articles regarding CPM and liver transplant, and 1 article describing CPM and HLH.

Conclusions:

CPM should be considered as a neurologic complication in patients with HSTL and HLH. We report such a case, despite only mild levels of hyponatremia, the absence of liver failure, and negative EBV status. Future research should explore the connection between these factors to further our understanding of osmotic regulation of the blood brain barrier leading to CPM.