Intravascular Lymphoma in the CNS: Options for Treatment

Nizamutdinov, Damir; Patel, Nitesh P.; Huang, Jason H.; Fonkem, Ekokobe

doi:10.1007/s11940-017-0471-4

Cited by 29 publications

(18 citation statements)

References 41 publications

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“…The most effective treatment of IVLBCL involving the CNS is a combination of R-CHOP and intrathecal methotrexate, which could even be administered in prophylaxis of CNS involvement [22]. Etoposide has been administered in relapse protocol in some patients, but in this case, the choice was driven by the association with HPS [23].…”

Section: Discussionmentioning

confidence: 99%

Pituitary Gland and Neurological Involvement in a Case of Hemophagocytic Syndrome Revealing an Intravascular Large B-Cell Lymphoma

Njonnou

Couturier

Gombeir

et al. 2019

Case Reports in Hematology

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Intravascular large B-cell lymphoma is a rare entity characterized by the proliferation of neoplastic lymphocytes in the lumen of small blood vessels and high mortality. Diagnosis of intravascular lymphoma is often delayed or established postmortem. Here, we report the case of a 48-year-old woman presenting hemophagocytic syndrome, with pituitary gland and neurological involvement. Diagnosis of intravascular large B-cell lymphoma was made on perisplenic vessels, while liver and bone marrow biopsy was noncontributive. This case demonstrates the importance of thorough histopathologic investigations in the setting of high suspicion.

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Section: Discussionmentioning

confidence: 99%

Pituitary Gland and Neurological Involvement in a Case of Hemophagocytic Syndrome Revealing an Intravascular Large B-Cell Lymphoma

Njonnou

Couturier

Gombeir

et al. 2019

Case Reports in Hematology

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“…CNS presentation and involvement represents a specific challenge, as there are no clear pathognomonic radiographic findings associated with IVLBCL[7]. In our case, we note the combination of mental status changes and MRI abnormalities including the “boomerang” sign noted above.…”

Section: Discussionmentioning

confidence: 60%

Intravascular large B-cell lymphoma presenting with altered mental status: A case report

D'Angelo¹,

Ku²,

Gulliver³

et al. 2019

WJCO

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BACKGROUNDIntravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of non-Hodgkin lymphoma with a varied presentation and no pathognomonic findings. Early diagnosis is critical to altering the disease course as early treatment with chemoimmunotherapy is required to prevent a rapidly fatal outcome. Strategies including improved awareness of this clinical entity through publication of cases with unique presentations are essential to prompt consideration of IVLBCL early in the disease workup. Here, we present a case of IVLBCL presenting with altered mental status and systemic organ dysfunction.CASE SUMMARYA 61-year-old male patient presented with flu-like symptoms and a high fever. He experienced rapid clinical deterioration with liver, kidney failure, and shock despite rapid antibiotic administration and supportive care. A broad infectious workup was negative. Intracranial imaging revealed nonspecific changes to the corpus callosum suspicious for vasculitis. Renal biopsy was non-diagnostic. After further progression of his symptoms, the family elected to withdraw care and the patient died shortly thereafter. Post-mortem analysis revealed clear multi-organ involvement by IVLBCL, prompting re-examination of the ante-mortem renal biopsy that also identified IVLBCL involvement.CONCLUSIONIVLBCL is a rare disease. Communication with specialties and early biopsy is critical to establishing the diagnosis and initiating therapy.

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“…The mainstay of treatment is with combination chemotherapy. Anthracycline-based chemotherapy with rituximab (eg, R-CHOP) is the most commonly use regimen and is associated with 80% complete response rate and 2-year overall survival higher than 60%, with consideration of CNS penetrating drugs, including intrathecal methotrexate if patients are fit enough to tolerate such regimens 9. Median survival for intravascular large B cell lymphoma where CNS is the primary site of disease is 14.0 months (0.1–84.0) though this has improved from 6 months in cases reported between 1960 and 1989 to 18 months for cases reported in 2000s 2.…”

Section: Discussionmentioning

confidence: 99%