A 43 year old man with a history of hairy cell leukemia in remission with resulting leukopenia presented with one day of fever, headache, photophobia, neck stiffness, and mild confusion. One week prior to presentation the patient had experienced a painful vesicular rash on the right side of his scalp. Vital signs were notable for a fever to 104.4 degrees Fahrenheit and a heart rate of 125 beats per minutes. Physical examination revealed a crusted rash over the right scalp with mild erythema. Laboratory studies were remarkable for a white blood cell count of 1.4 x 109/L, absolute neutrophil count of 1.2 x 109/L, and an absolute lymphocyte count of 0.2 x 109/L. Cerebrospinal fluid studies revealed colorless fluid, 32 x 106/L nucleated cells with 81% lymphocytes, glucose of 62 mg/dL, total protein of 36 mg/dL, negative culture, and positive polymerase chain reaction for varicella zoster virus DNA. Serum varicella polymerase chain reaction testing was negative. Brain magnetic resonance imaging revealed several regions of increased signal involving the white matter of the cerebral hemispheres. This patient’s clinical presentation of fever, head ache and mild confusion in the setting of recent zoster rash and cerebrospinal fluid containing varicella‐zoster DNA supports a diagnosis of varicella meningoencephalitis. The patient was treated with acyclovir for a total of ten days with marked improvement.
Patients with underlying neoplasms are known to experience herpes zoster at a higher incidence than the general population, 25% versus 1% respectively (1). Patients with leukemia or lymphoma have the highest rates of herpes zoster infection among adult cancer patients (2). In the general population infected with herpes zoster the incidence of zoster encephalitis was found to be 0.1‐0.2% (3). In the cancer population the most common neurologic complication of zoster, aside from pain, is meningoencephalitis occurring in almost 1% of cases (4). Patients with immunosuppression are at an increased rate of zoster dissemination and visceral organ involvement. (5) Intravenous acyclovir is the treatment of choice for varicella zoster in severely immunocompromised patients. Acyclovir halts disease progression, reduces the duration of viral replication, promotes faster disease resolution, and is effective at preventing dissemination (6). Acyclovir should be started early given that dissemination is associated with a high fatality rate (7).
This case demonstrates a condition which is relatively rare but occurs with much greater prevalence in an immunocompromised population, which is a patient population frequently encountered by hospitalists. Hospitalists should keep zoster infection in the differential for any patient with symptoms of meningitis, and should know that early acyclovir is important in preventing disseminated disease in the immunosuppressed patient.
To cite this abstract:Barron M, Czernik Z. In a Patient with Hairy Cell‐A, Should We Consider Varicella?. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 341. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/in-a-patient-with-hairy-cella-should-we-consider-varicella/. Accessed March 28, 2020.