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Unusual Presentations of Hematologic Malignancies

CASE 3. Primary Central Nervous System B-Cell Lymphoma of Marginal Zone B-Cell Type in a Neurosyphilis Patient

The Brooklyn Hospital Center of Cornell University, Brooklyn, NY

A 63-year-old male resident of a nursing home was sent to our hospital for evaluation of lethargy, decreased responsiveness, and high-grade temperature. Medical history was significant for Parkinson's disease, depression, and neurosyphilis (treated in the past as per nursing home records). Computed tomography (CT) scan of the head without contrast was done on admission, revealing a high-density left frontal subdural mass with an irregular inner table. Septic work-up was negative. Lumbar puncture showed lymphocyte pleocytosis, high protein, and low glucose. CSF cultures and cytology studies were negative (Venereal Disease Research Laboratory test was not done). Broad-spectrum antibiotics were empirically given for possible meningitis. Patient had an episode of seizure during hospital admission and antiepileptic therapy was recommended. Serum rapid plasma reagin was found to be positive (titer, 1:16), with positive fluorescent treponemal antibody. HIV testing was nonreactive. Broad-spectrum antibiotics were discontinued, and benzathine penicillin (2.4 million units) was given once a week for 3 weeks. The patient's general condition and mental status improved considerably with this management. Repeat CT scan of the head with contrast showed destructive mass in the left side with epidural extension, 7 x 5 x 2.5 cm in size (Fig 1). Bone scan showed patchy uptake in the left parietal/temporal region of the skull. Surgical intervention was indicated, and craniotomy through the left frontal/temporal region and excision of an intradural and extradural brain tumor were performed. Immunophenotypic and microscopic studies were consistent with low-grade B-cell lymphoma. Presence of CD20 and bcl-2 were strongly positive, and CD5 and CD10 were negative (Fig 2A, B, C, and D), with perifollicular distribution and follicular colonization. These findings support the diagnosis of marginal-zone B-cell lymphoma. A CD5 negative mantle-cell lymphoma would be very unlikely. No other evidence of lymphoma was detected on CT studies of the chest, abdomen, and pelvis; Gallium Scan; or bilateral bone marrow biopsy.

View larger version (85K): [in this window] [in a new window]   Fig 1.

View larger version (147K): [in this window] [in a new window]   Fig 2.

CNS involvement occurs in up to 40% of syphilis patients secondary to treponemal seeding during spirochetemia. Characteristic findings in neurosyphillis, including CNS pleocytosis, elevated CSF protein, and decreased glucose levels, were present in this patient's work-up. We believe that the clinical and serological data presented and the patient's response to antibiotic therapy make the diagnosis of neurosyphilis highly likely.

Certain infectious agents such as Helicobacter pylori, hepatitis C,^5,6 Borrelia burgdorferi,⁷ and chronic malaria⁸ have shown to have a strong association with low-grade lymphoma. On literature search, we found only one case of neurosyphilis in a patient with low-grade lymphoma.⁹ As such, we suspect other infectious agents may be linked to low-grade lymphomas. Chronic antigenic stimulation may lead to proliferation of malignant clones of B lymphocytes, resulting in development of low-grade lymphomas. It is unclear whether treatment of these infectious diseases can change the course of low-grade lymphoma, as has been shown in cases of H. pylori and mucosa-associated lymphoid tissue lymphomas.

Authors' Disclosures of Potential Conflicts of Interest

The authors indicated no potential conflicts of interest.

REFERENCES

1. Altundag KM, Ozisik Y, Yalcin S, et al: Primary low-grade B-cell lymphoma of the dura in an immunocompromised patient. J Exp Clin Cancer Res 19:249-251, 2000[Medline]

2. Goetz P, Lafuente J, Revesz T et al: Primary low grade B-cell lymphoma of mucosa-associated lymphoid tissue of the dura mimicking the presentation of an acute subdural hematoma. J Neurosurg 96:611-614, 2002[Medline]

3. Norman L, Lehman MD, Dikran S, et al: Dura marginal zone lymphoma with massive amyloid deposition: Rare low-grade primary central nervous system B-cell lymphoma. J Neurosurg 96:368-372, 2002[Medline]

4. Kumar S, Kumar D, Kaldjian EP, et al: The clinicopathological findings in five patients with primary low grade B-cell lymphoma of the intracranial dura are described. Am J Surg Pathol 21:81-87, 1997[CrossRef][Medline]

5. Hermine O, Lefrere F, Bronowicki J, et al: Regression of splenic lymphoma with villous lymphocytes after treatment of Hepatitis C virus infection. N Engl J Med 347:89-94, 2002[Abstract/Free Full Text]

6. Gasparotto D, De Re V, Boiocchi M: Hepatitis C virus, B-cell proliferation and lymphomas. Leuk Lymphoma 43:747-751, 2002[CrossRef][Medline]

7. Roggero E, Zucca E, Mainetti C, et al: Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin. Hum Pathol 31:263-268, 2000[CrossRef][Medline]

8. Bates I, Bedu-Addo G: Chronic malaria and splenic lymphoma: Clues to understanding lymphoma evolution. Leukemia 11:2162-2167, 1997[Medline]

9. Mantadakis E, Simonis G. Case of neurosyphilis diagnosed in a patient with primary CNS low-grade follicular small-cleaved cell lymphoma. Infection 30:43-45, 2002[Medline]

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Haque, B. Articles by Patel, A. Search for Related Content PubMed PubMed Citation Articles by Haque, B. Articles by Patel, A. Social Bookmarking                            What's this?