Mantle Cell Lymphoma : A Clinicopathologic View

الملخص EN

Mantle cell lymphoma (MCL) is a recently recognized subtype of non-Hodgkin’s lymphoma (NHL) by the REAL classification.

It is characterized by chromosomal translocation t (11 ;14) (q13 ; 32) resulting in overexpression of Bcl-1 (cycling D1), the cell regulator protein.

Clinically, the disease presents as a generalized form with frequent treatment failures and poor prognosis.

MCL has not been documented as a specific disease entity in Egyptian material prior to its precise identification.

Owing to its important clinic-pathologic implications, retrospective analysis of 712 cases of NHL treated at the NCI during the years 1990-1995 was performed.

Re-classification of 63 MCL cases (8.8 %) was established with a male: female ratio of 3:1 and a median age of 55 years (range 16-65 years).

Histologic variants were predominantly diffuse, mantle zone or vaguely nodular.

The cytological variants were small, pleomorphic or blasted.

Molecular and immunohistochemical markers of this group showed the following profile : Bcl-1 +ve in 89 % of cases, multidrug resistance (MDR) p-glycoprotein +ve in half of the cases, and p53 occasionally +ve in the blasted variant.

Response to anthracyclin-based chemotherapy was evaluated in 20 out of the 63 cases.

Complete remission was achieved in 15 patients (75 %), with a median disease-free survival time of 12 + months.

These data confirm that MCL represents a distinct clinic pathologic entity and urge for including the REAL classification in the diagnosis of malignant lymphoma.