mantle cell lymphoma

mantle cell lymphoma

a clinically and biologically distinct B-cell neoplasm with a recurring acquired genetic abnormality, the t(11;14) translocation, and a heterogeneous histologic appearance that may lead to confusion with reactive or other neoplastic lymphoproliferative disorders.

mantle cell lymphoma

An uncommon low-to-intermediate-grade non-Hodgkin lymphoma (NHL), which is a malignancy of naive B cells. It comprises ± 6% of NHL cases in the US, slightly higher in Europe. It is hard to treat and harder to cure. It is more common in men ≥ age 55.
 
Clinical findings
Massive splenomegaly, generalised lymphadenopathy, 1/3 present with “B” symptoms—i.e., fever, weight loss, night sweats.

Sites
Nodes, spleen, bone marrow, Waldeyer's ring, GI (lymphomatous polyposis), gallbladder. 

DiffDx
• Small lymphocytic lymphoma/CLL
• MALT lymphoma
• Follicular lymphoma

Prognosis MCLs are often aggressive and involve the spleen, liver, bone marrow; they are regarded as incurable. Median 2- to 5-year survival. Cases with mutations in the immunoglobulin gene have better prognosis—40% achieve complete remission with chemotherapy.

man·tle cell lym·pho·ma

(man'tĕl sel lim-fō'mă)

A clinically and biologically distinct B-cell neoplasm with a recurring acquired genetic abnormality, the t(11;14) translocation, and a heterogeneous histologic appearance that may lead to confusion with reactive or other neoplastic lymphoproliferative disorders.