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Older Lymphoma Classification and Typing Schemes

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The group of cancers under the general term Lymphoma is quite broad. The distinctions between types of lymphoma are based on the different characteristics of the cancerous cells. Over the years, different classification systems have been used to differentiate lymphomas:
  • Rappaport: Used until the 1970s, Rappaport was based on how the cells look in the microscope alone
  • Kiel: Used mainly in Europe, the Kiel system, is based on microscopic and immunologic (what types of proteins are produced by the cell) criteria, it was updated in 1992.
  • National Cancer Institute's Working Formulation (IWF): Ontroduced in 1982, the IWF divided lymphomas into low-grade, intermediate-grade and high-grade, with subgroups based on cell type and presentation
  • Revised European-American Classification of Lymphoid Neoplasms (REAL): Introduced in 1994, REAL describes the different types of lymphomas as entities, with each type classified according to cell origin, based on morphologic, immunologic and genetic criteria.
Historically, uniform treatment of patients with lymphoma has been hampered by the lack of uniformity in these classification systems. 

 

The following comparison of the Working formulation and the Rappaport system is shown in case older records use some of this terminology:

 

 
Working formulation                     Rappaport classification

Low-grade
A.small lymphocytic, consistent with    diffuse lymphocytic, well
   chronic lymphocytic leukemia (SL)       -differentiated (DLWD)
B.follicular, predominantly small       nodular lymphocytic poorly
   cleaved cell (FSC)                      differentiated (NLPD)
C.follicular, mixed small cleaved       nodular mixed, lymphocytic
    and large cell (FM)                     and histiocytic (NM)

Intermediate-grade
D.  follicular, predominantly large     nodular histiocytic(NH)
    cell (FL)                                   
E.  diffuse, small cleaved cell         diffuse lymphocytic poorly
    (DSC)                                  differentiated (DLDP)
F.  diffuse mixed, small and large      diffuse mixed, lymphocytic
    cell (DM)                              and histiocytic (DM)
G.  diffuse, large cell cleaved or      diffuse histiocytic (DH)
    noncleaved cell (DL)

High-grade
H.  immunoblastic, large cell (IBL)     diffuse histiocytic (DH)
I.  lymphoblastic, convoluted or        diffuse lymphoblastic (DL)
    nonconvoluted cell (LL)                 
J.  small noncleaved cell, Burkitt's    diffuse undifferentiated,
     or non-Burkitt's (SNC)               Burkitt's or non-Burkitt's (DU)

As the understanding of lymphoma has improved and as the histopathologic
diagnosis (the study of the cancerous cells themselves) of lymphoma has become
more sophisticated, a number of new pathologic entities were described. In
addition, the understanding and treatment of many of the previously described
pathologic subtypes changed. As a result, the Working Formulation had
become outdated and less useful to clinicians and pathologists. Thus, European
and American pathologists have proposed a new classification, the Revised
European American Lymphoma (REAL) Classification. 

The REAL Classification

B-cell neoplasms
 I. Precursor B-cell neoplasm: precursor B-lymphoblastic leukemia/lymphoma
II. Peripheral B-cell neoplasms
    A. B-cell chronic lymphocytic leukemia/prolymphocytic
       leukemia/small lymphocytic lymphoma
    B. Lymphoplasmacytoid lymphoma/immunocytoma
    C. Mantle cell lymphoma
    D. Follicle center cell lymphoma, follicular
       1. Provisional cytologic grades: 
          I small cell,
          II mixed small and large cell, 
          III large cell
       2. Provisional subtype: diffuse, predominantly
          small cell type
    E. Marginal zone B-cell lymphoma
       1. Extranodal (MALT-type +/- monocytoid B cells)
       2. Provisional subtype: nodal (+/- monocytoid B cells)
    F. Provisional entity: splenic marginal zone lymphoma
       (+/- villous lymphocytes)
    G. Hairy cell leukemia
    H. Plasmacytoma/plasma cell myeloma
    I. Diffuse large B-cell lymphoma
       1. Subtype: primary mediastinal (thymic) B-cell lymphoma
    J. Burkitt's lymphoma
    K. Provisional entity: high-grade B-cell lymphoma, Burkitt-like

T-cell and putative NK-cell neoplasms
 I. Precursor T-cell neoplasm: precursor T-lymphoblastic
    lymphoma/leukemia
II. Peripheral T-cell and NK-cell neoplasms
    A. T-cell chronic lymphocytic leukemia / prolymphocytic leukemia
    B. Large granular lymphocyte leukemia
       1. T-cell type
       2. NK-cell type
    C. Mycosis fungoides/Sezary's syndrome
    D. Peripheral T-cell lymphomas, unspecified
       1. Provisional cytologic categories: 
          medium-sized cell, mixed medium and 
          large cell, large cell, lymphoepithelioid cell
       2. Provisional subtype: hepatosplenic gamma/delta T-cell lymphoma
       3. Provisional subtype: subcutaneous panniculitic T-cell lymphoma
    E. Angioimmunoblastic T-cell lymphoma
    F. Angiocentric lymphoma
    G. Intestinal T-cell lymphoma (+/- enteropathy associated)
    H. Adult T-cell lymphoma/leukemia
    I. Anaplastic large cell lymphoma
       1. CD30+ -cell type
       2. T-cell type
       3. Null-cell types
    J. Provisional entity: anaplastic large cell lymphoma, Hodgkin's-like

Hodgkin's Disease (now known to be a B-cell neoplasm)
  I. Lymphocyte predominance
 II. Nodular sclerosis
III. Mixed cellularity
 IV. Lymphocyte depletion
  V. Provisional entity: lymphocyte-rich classical Hodgkin's disease

Back to Lymphomas - Current Classifications


For more information on Adult Non-Hodgkin's lymphoma, please see the
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Updated October 22, 2005