Age: 18.0
Sex: M
Vaccine: PPV
Symptoms: Papilloedema, Red blood cells CSF positive, Retinal haemorrhage, Sinusitis
Clinical narrative: It was reported in a published literature article, titled as stated above that an 18 year old male with a history of acute myeloid leukaemia was vaccinated on an unspecified date with PNEUMOVAX 23 (manufacturer unknown). It was noted that this 18-year-old man with a history of acute myelogenous leukemia presented for neurological consultation with a 1-month history of headaches. The headaches were described had increased in frequency and severity during the 10 days before presentation, and were associated with episodes of transient, self-limited, binocular loss of vision. The headaches did not correlate with time of day, did not awaken from sleep, and were unaffected by changes in body position. He denied photophobia, phonophobia, nausea, vomiting, or scintillating auras. The pain improved slightly with acetaminophen. A thorough review of the signs were remarkable only for mild neck pain, intermittent lightheadedness, and dizziness. His past medical history was remarkable for acute myelogenous leukemia in the background of monosomy 7-associated myelodysplastic syndrome. His initial presentation at age 15 years was accompanied by syncope and pancytopenia (white blood cells, 2,900/cc3; hemoglobin, 5 g/dL; platelets, 15,000/cc3). Analysis of his cerebrospinal fluid at the time of his original diagnosis produced normal results. He was treated initially with systemic cytarabine, daunorubicin, etoposide, and intrathecal infusions of Methotrexate and cytarabine. He then underwent a 6/6 matched, unrelated-donor bone marrow transplant, after receiving total body radiation (total dose, 1,440 cGy) and cyclophosphamide as pretransplant conditioning. He engrafted without difficulty, and exhibited only mild, self-limited, oral chronic graft-versus-host disease that did not require systemic therapy. Three years after transplantation, during an evaluation for causes of recurrent sinusitis, mild immunodeficiency was diagnosed, based on a poor specific antibody response to PNEUMOVAX 23. As a result, the patient had been receiving monthly intravenous gamma globulin. He had also experienced episodes of lightheadedness, 2 years before the current presentation, at which time he underwent a neurologic examination, magnetic resonance imaging of the brain, lumbar puncture, and electroencephalogram, all of which produced normal results. He had mild adjustment issues related to his underlying illness. The patient denied the use of caffeine, illicit drugs, tobacco, or alcohol. Medications included nasal fluticasone, topical tretinoin, and acetaminophen. His family was remarkable for a distant history of migraines and several relatives with solid-organ cancer. On examination, the patient was afebrile, with normal blood pressure. He was in no distress, without neck tenderness or meningismus. A general examination was notable only for mild, healing acne vulgaris. On neurologic assessment, his mental status was entirely intact. The visual fields in each eye exhibited full to double simultaneous quadratic confrontation. His pupils were equal, round, and reactive to both light and dark. His visual acuity was 20/20 in both eyes. There was no relative efferent papillary defect. A fundoscopic examination demonstrated bilateral papilledema with associated retinal hemorrhage. There were no extudes, and the retinal vasculature appeared normal. Venous pulsations were not observed. A slit-lamp examination of the anterior and poster chambers and intraocular pressure produced normal results. Sensory, motor, and cerebellar examinations produced normal results. His deep-tendon reflexes were symmetric and normal, without spread or clonus. His plantar responses were flexor bilaterally. The initial evaluation included normal results of magnetic resonance imaging, a magnetic resonance angiogram, and a magnetic resonance venogram of the brain, performed without the use of intravenous contrast. A lumbar puncture performed in the lateral decubitus position, with the legs extended demonstrated an opening pressure of 53 cm H2O. The cerebrospinal fluid profile revealed 108/cc3 white blood cells, with 38% myeloblasts, and 1,000 red blood cells/cc3. His total protein was normal, at 25.6 mg/dL, and his glucose was slightly elevated, at 74 mg/dL. Cerebrospinal fluid cytology confirmed abundant large blasts with irregular nuclei, fine chromatin, prominent nucleoli, abundant pale-blue cytoplasm, and occasional cytoplasmic granules. Auer rods were not detected. Flow cytometry demonstrated a population of immature cells positive for cluster of differentiation (CD) 45 (dim), CD34, human leukocyte antigen-DR, myeloid markers CD13, CD33, CD117, and CD15, monocytic marker CD11b, and aberrantly expressing CD7 (dim) in a subset of cells, but negative for other monocytic, B, and T lymphoid markers. The cerebrospinal fluid blasts indicated monosomy 7 by fluorescent in situ hybridization. A bone marrow aspirate and biopsy demonstrated slightly hypocellular marrow, without histologic signs of acute myelogenous leukemia. Cytogenetic and fluorescent in situ hybridization analyses performed on the bone marrow were negative for monosomy 7, and chimerism studies demonstrated 100% donor hematopoiesis. An ophthalmologic evaluation demonstrated papilledema with normal Goldman visual fields. A diagnosis was rendered of meningeal leukemia representing an isolated central nervous system relapse of acute myelogneous leukemia. Oral acetazolamide therapy was initiated. The patient then began treatment with intrathecal cytarabine, methotrexate, and hydrocortisone. Topical tretinoin was discontinued. Cerebrospinal fluid blasts persisted after 9 doses of intrathecal cytarabine, given in most cases with intrathecal methotrexate and hydrocortisone. Blasts finally cleared after 2 additional doses of liposomal cytarabine, and as of this writing, his cytology remains negative for malignant cells after a total of 6 doses of liposomal cytarabine. Despite the disappearance of detectable malignant cells from the cerebrospinal fluid and the use of acetazolamide, his intracranial pressure remains elevated at 40 cm water on latest assessment, 7 months after presentation. His headache resolved, and there is minimal visual-field constriction in one eye, with persistent papilledema. Upon internal review acute myelogenous leukemia was considered an other important medical event. A copy of the published article is attached as further documentation of the patient's experience.