EBV Associated Hemophagocytic Syndrome Accompanied by Central Pontine Myelinolysis

The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitte...

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Bibliographic Details
Published in:Leukemia & lymphoma 2002-10, Vol.43 (10), p.2047-2050
Main Authors: Uehara, Eisuke, Tasaka, Taizo, Matsuhashi, Yoshiko, Fujita, Mitsuhiro, Tamura, Takahiro, Kuwajima, Minoru, Nagai, Masami, Ishida, Toshihiko
Format: Article
Language:English
Subjects:
Aged
Antineoplastic Combined Chemotherapy Protocols - administration & dosage
Bone Marrow Examination
Central Pontine Myelinolysis
Cytokines
Epstein-Barr Virus
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - diagnosis
Epstein-Barr Virus Infections - drug therapy
Fatal Outcome
Hemophagocytic Syndrome
Histiocytosis, Non-Langerhans-Cell - complications
Histiocytosis, Non-Langerhans-Cell - diagnosis
Histiocytosis, Non-Langerhans-Cell - drug therapy
Humans
Magnetic Resonance Imaging
Male
Myelinolysis, Central Pontine - complications
Myelinolysis, Central Pontine - diagnosis
Myelinolysis, Central Pontine - drug therapy
Myelinolysis, Central Pontine - etiology
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Summary:The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. Physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. Magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. Bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methyl-predonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.
ISSN:1042-8194
1029-2403
DOI:10.1080/1042819021000016032