A Rare Manifestation of a Rare Disease: Mantle Cell Lymphoma | Figure 1

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A Rare Manifestation of a Rare Disease: Mantle Cell Lymphoma Presenting With Aseptic Meningitis

Mantle cell lymphoma (MCL) is a rare form of non-Hodgkin lymphoma characterized by clonal proliferation of follicular mantle zone B lymphocytes. It is caused by abnormal chromosomal translocation t(11;14) resulting in aberrant expression of cyclin D1. This leads to activation of anti-apoptotic pathways and abnormal proliferation of MCL cells. Patients can present with an indolent course or a fulminant disease with short overall survival. The disease frequently involves extranodal organs, but rarely manifests with neurological symptoms. We report a rare case of aberrant CD5-negative MCL presenting with aseptic meningitis.

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Case Information

  1. Image:

    • Description: Coronal (left) and axial (right) magnetic resonance images demonstrating a 2.3 × 1.0 × 2.7 cm dural-based mass (yellow arrow) in the left frontoparietal region with associated thickening and enhancement of the adjacent dura. There is an extracranial component to the mass in the adjacent soft tissues without visible abnormalities of the bone. In addition, there are linear extension into the gyri of the adjacent brain parenchyma with an associated focal area of subcortical edema in the lateral aspect of the postcentral gyrus.
    • Case Description: Follicular lymphoma (FL) usually has an indolent course and presents with painless, waxing and waning lymphadenopathy in the absence of systemic symptoms. It is uncommon for FL to present outside of lymph nodes, although it can develop in the gastrointestinal tract, skin, thyroid, and testes. Central nervous system (CNS) involvement in FL is rare. Most CNS lymphomas are diffuse large B-cell lymphoma, although Burkitt lymphoma, lymphoblastic lymphoma, and peripheral T-cell lymphoma are also observed. These tumors usually involve white matter but may also involve gray matter. Lymphomas of the dura are very uncommon and are usually mucosa-associated lymphoid tissue lymphomas. Here, we present a case of FL of the dura arising in a 62-year-old woman that was responsive to chemotherapy. According to a literature review, there have been 15 previously reported cases of FL of the dura. Dural FL has been most frequently treated with radiation and chemotherapy. Patients were still alive in all cases in which follow-up was reported. Although the sample size is small, these data suggest that dural FL, like other forms of FL, is an indolent disease that is associated with prolonged survival despite usually being incurable.

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    • Description: Plasmablastic lymphoma (PBL) is a very rare disease and it is usually considered a human immunodeficiency virus (HIV)–related B-cell lymphoma that carries a poor prognosis. It mostly involves the oral cavity, lungs, nasal cavity, gastrointestinal tract, lymph node, and skin. Therapeutic regimens like dose-adjusted etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone (DA-EPOCH) have shown better results in these aggressive lymphomas. We report a rare case of PBL in an HIV-negative patient who presented to the clinic with a complaint of left testicular swelling for 3 months.

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    • Description: Axial T2 weighted (image 1 and 2), T1-weighted (image 3), diffusion weighted (image 4), and T1-weighted postcontrast (image 5) MRI shows a large mass arising within the left liver. After biopsy, the mass was confirmed to represent diffuse large B-cell lymphoma.

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    • Description: This is a mature B cell #Leukemia. Morphologically, neoplastic cells have too much cytoplasm to be blasts/immature. On the #Flow-cytometry you can see the abnormal population is 72% of WBCs and expresses CD5, CD19, CD20, and surface kappa light chain. That tells us it's a B cell leukemia/lymphoma (CD19 & 20) and the differential includes #Mantle-cell-lymphoma and transformed #Chronic-lymphocytic-leukemia as well as leukemic #Diffuse-large-B-cell-lymphoma. #Mantle-cell-lymphoma was ruled out by cytogenetics for t(11;14).

  5. Image:

    • Description: A 52-year-old man presented with a 20-minute history of chest pain radiating to the left shoulder and neck, associated with dyspnea and diaphoresis.

  6. Image:

    • Description: A 28-year-old woman presented with a 3-day history of intense pruritus over the lower back. Physical examination revealed multiple small vesicles.

  7. Image:

    • Description: A 4-month-old male infant presented with skin lesions localized to the chin for the past 3 days.

  8. Image:

    • Description: patient in late teens presenting after a collapse, no chest pain, no previous cardiac history.