I. Case report
A 67-year-old male HIV (human
immunodefficiency virus) negative ex-smoker patient was admitted with chief
complaint of vertigo, uncontrolled blood pressure, dyspnea, and bilateral decreased
hearing of few days duration. His past medical history included diabetes
mellitus (type II), hypertension, and ischemic heart disease. His daily
medication profile included: doxazocin 2 mg, losartan 50 mg, lansoprazole 30
mg, ticlopidine 250 mg, and chlorpropamide 125 mg combined with fenformin 30
mg.
The physical exam was significant for
elevated blood pressure (190/100 mmHg), slurred and slow speech and bilateral
markedly decreased hearing. There was no lymphadenopathy , hepatosplenomegaly ,
or neck stiffness. Laboratory studies showed thrombocytopenia (95,000/mm3;normal
150,000-400,000/mm3), elevated (white blood cells) WBC (13,750/ mm3
;normal 5,000-10,000/ mm3 ) , abnormal
peripheral smear (nucleated red blood cells, metamyelocytes), hyperuricemia (16
mg/dl, normal levels 2.4-7.5 mg/dl), increased lactate dehydrogenase (9,430
U/L; normal levels 50-240 U/L) , and elevated serum creatinine (1.9 mg/dl;
normal levels 0.8-1.2 mg/dl). CT–scan (computed tumography) and MRI
(magnetic resonance imaging) of the brain, chest x-ray and ultrasound of
abdomen were all normal. Echocardiography demonstrated mitral and aortic
regurgitation along with apical dyskinesia with a left ventricular ejection
fraction of 38%.
Two days after admission, the patientŐs blood pressure was controlled.
However, his hearing deteriorated dramatically and he became totally deaf.
Also, his platelet count decreased precipitously to 11,000/mm3.
Medical investigation was directed to explain the rapidly progressive changes
in clinical and laboratory findings. Initially, drug induced hearing damage was
suspected, but none of his medications was found to cause hearing loss. The
laboratory findings of low platelet count, increased LDH and abnormal
peripheral smear triggered his physician to recommend bone marrow aspirate and
biopsy to rule out a malignant process. Biopsy showed diffuse infiltration of
marrow spaces by monomorphous cell population with one or two conspicious
nucleoli and deeply basophilic cytoplasm with abundant vacuolization; mitosis
was frequent. Morphology and immunohistochemical staining were consistent with
BurkittŐs lymphoma. The diagnosis of BurkittŐs lymphoma with CNS (particularly
vestibulo-cochlear cranial nerve) involvement was suspected. However, no lumbar
puncture was done since the patient had severe thrombocytopenia and could
develop epidural hemorhage. The patient was provided with supportive care and
flew back home for further management of his malignant hematologic disorder.
Two weeks later the patient passed away, after receiving an unknown
chemotherapy.
II. Discussion
In 1958, Burkitt described a
mandibular malignancy in African children that later proved to be non-cleaved B cell lymphoma
(Magrath, 1990). The increasing frequency of AIDS and immunosuppressive therapy
has lead to increase incedence of nonendemic BurkittŐs lymphoma (Ziemler et al,
1982). In such cases, extranodal involvement with ultimate CNS involvement is
common (Bomfim da paz and Kolmel, 1992).
Literature review showed that many
cases of BurkittŐs lymphoma with optic nerve involvement were reported. In such
cases, diplopia was found to be the initial manifestation (Grassi and Lee,
2002). In some cases, patients presented with multiple cranial nerve palsies
(Pal et al, 1995). Bone marrow involvement, initial CNS manifestation and older
age at diagnosis all speak for a poor prognosis (Michael et al, 1990). All
these factors were present in our patient. Our case illustrates that abrupt
change in cranial nerve function and other neurologic findings indicate the
need for vigorous investigation.
To our knowledge, this is the first
case of BurkittŐs lymphoma to be reported with suspected eighth cranial nerve
involvement. The early diagnosis of BurkittŐs lymphoma is crucial since it is
the most rapidly progressive human tumor and any delay in initiating therapy
can adversely affect the patientŐs outcome.
References
Bomfim da paz R, Kolmel HW. (1992)
Meningitis with Burkitt like B-cell lymphoma in HIV infection. J Neuroncol 13, 73-79.
Grassi MA, Lee AG (2002)
Lymphomatous meningitis of Burkitt
type presenting with multiple cranial neuropathies. Am J Ophthalmol 133, 424-425.
Magrath I. (1990) The
pathogenesis of BurkittŐs lymphoma. Adv
Cancer Res 55, 132-270.
Michael T,Grgory BK, Robert SH, Faramarz N (1980) BurkittŐs lymphoma with cranial nerve involvement. Arch Ophthalmol 98, 2015-2017.
Pal L,Valli ER, Santosh V, Menon A,Veerendrakumar M, Nagaraja D, Das S,
Shankar SK. (1995) Disseminated
BurkittŐs lymphoma presenting as multiple cranial nerve palsies. Indian J Cancer 32,116-120.
Ziegler JL, Miner RC, Rosenbaum E, et al (1982) Outbreak of
BurkittŐs like lymphoma in homosexual men. Lancet 2, 631-633.
Dr.
Ismail Zaidan