Primary Cutaneous Blastoid Mantle Cell Lymphoma-Case Report

Estrozi, Bruna; Sanches, José Antônio; Varela, Paulo C S; Bacchi, Carlos E.

doi:10.1097/dad.0b013e31819d845a

Cited by 31 publications

(33 citation statements)

References 12 publications

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“…2018;7(1):38-42 toid and pleomorphic variants have been generally associated with morbid prognosis and incurability [7]. The blastoid variant is found in 10-30% of those diagnosed with MCL and has a shorter median survival time than classical type (15 vs. 45 months) [5,8,9]. The skin involvement, even if rare, has been more frequently reported in the blastoid variant of MCL [2].…”

Section: Discussionmentioning

confidence: 99%

“…Similar to translocation (11:14), cyclin D1 is characteristic to MCL and is expressed in more than 60-70% of cases of MCL but also can be positive in 4% of B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL) [11]. The lymphoma is usually positive for B-cell markers, like CD79a, CD19, CD20, CD22 and CD5 as well as usually negative for CD10, CD23 and BCL-6 [9]. CD5 immunoreactivity helps differentiate MCL from other B-cell lymphomas such as follicular or marginal zone lymphomas but it can be absent in 10-30% of all MCL cases.…”

Section: Discussionmentioning

confidence: 99%

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Mantle Cell Lymphoma Relapsing as Disease of Skin, Orbit and CNS: An Extremely Rare Presentation and a Review of Literature

Shaikh¹,

Jani²,

Shah³

et al. 2018

J Hematol

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Mantle cell lymphoma (MCL) is a form of non-Hodgkin's lymphoma originating from mature B cells. The hallmark gene translocation (11:14) results in overexpression of cyclin D1. Affected extranodal sites include bone marrow and gastrointestinal tract, but skin, orbit or CNS are rarely involved. Twenty-four cases have reported involvement of skin by MCL, while orbital MCL is equally rare. Our case is the first to report relapsed MCL with involvement of the skin and orbit simultaneously without disease in the lymphatic system or the bone marrow. A 53-year-old female with stage IV MCL initially presented with pancytopenia, adenopathy and splenomegaly. She achieved complete remission after six cycles of rituximab and bendamustine. Within 4 weeks of treatment, she developed diplopia and a rash of the left breast. Skin biopsy showed lymphoma infiltrates with B-cell markers for MCL. MRI of the orbits and brain suggested orbital lymphoma. CSF cytology further confirmed MCL cells. At time of relapse, she continued to be in hematologic remission. She initiated intrathecal cytarabine and methotrexate along with ibrutinib. R-CHOP was then added to the regimen. Within 2 weeks of starting treatment, her skin disease resolved and she had improvement in vision. MCL commonly presents as a disseminated disease, resulting in high mortality. Involvement of the skin or orbit has been sparingly reported and always suggests aggressive disease. It thus poses a challenge to diagnose and treat the condition as evidenced by resolution of adenopathy and bone marrow disease. Due to the overall poor prognosis of MCL and its unique presentations, as demonstrated by our case, early detection and prompt treatment are crucial to survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Mantle Cell Lymphoma Relapsing as Disease of Skin, Orbit and CNS: An Extremely Rare Presentation and a Review of Literature

Shaikh¹,

Jani²,

Shah³

et al. 2018

J Hematol

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“…The most frequent site is represented by the gastrointestinal tract 5 , with the characteristic clinico-pathological presentation of a multiple lymphomatous polyposis. Other primary MCLs have been reported in the skin 3,6 , the oral cavity 7 and the conjunctiva 8 , but they can be considered extremely rare.…”

Section: Dear Editormentioning

confidence: 99%