3743s1 06 pittaluga

Document Sample
3743s1 06 pittaluga Powered By Docstoc
					Proposed WHO Classification of Lymphoid neoplasm

   B cell neoplasm

    Precursor B cell neoplasm
        Precursor B-lymphoblastic leukemia/lymphoma
        (precursor B-ALL)
   T cell neoplasm
    Precursor T-cell and NK-cell neoplasm

        Precursor T-lymphoblastic lymphoma/leukemia
        (precursor T-ALL)
Mature (peripheral) B cell neoplasm

B-cell chronic lymphocytic leukemia/SLL
Lymphoplasmacytic lymphoma

Splenic marginal zone B-cell lymphoma

Hairy cell leukemia

Plasma cell myeloma/plasmacytoma

Extra-nodal marginal zone B cell lymphoma of MALT type

Nodal marginal zone B cell lymphoma
Follicular lymphoma
Mantle cell lymphoma
Mature (peripheral) B cell neoplasm (cont’d)


       Diffuse large B cell lymphoma

              Mediastinal B cell lymphoma

              Primary effusion lymphoma



       Burkitt lymphoma/Burkitt cell leukemia
Mature (peripheral) T cell neoplasm

 T-cell prolymphocytic leukemia

 T-cell granular lymphocytic leukemia

 Aggressive NK-cell leukemia
 Adult T-cell leukemia/lymphoma (HTLV1)

 Extranodal NK/T cell lymphoma, nasal type

 Enteropathy type T cell lymphoma

 Hepatosplenic gamma delta T cell lymphoma
 Subcutaneous panniculitis-like T cell lymphoma
 Mycosis fungoides/Sezary syndrome
Mature (peripheral) T cell neoplasm (cont’d)

 Anaplastic large cell lymphoma, T/null cell, primary cutaneous

 Peripheral T cell lymphoma, NOS

 Angioimmunoblastic T cell lymphoma

 Anaplastic large cell lymphoma, T/null cell,
 primary systemic
  Hodgkin's lymphoma (Hodgkin's disease)

Nodular lymphocyte predominant Hodgkin's lymphoma

Classical Hodgkin's lymphoma

  Nodular sclerosis Hodgkin's lymphoma (grades 1 and 2)
  Lymphocyte-rich classical Hodgkin's lymphoma

  Mixed cellularity Hodgkin's lymphoma

  Lymphocyte depletion Hodgkin's lymphoma
             Pediatric Follicular Lymphoma
• Rare lymphoma subtype in children (1-2%)
• 60% present in head and neck
   – Tonsils, nasopharynx
   – Other sites: lymph nodes, GI tract, testis
Many differences with FL in adults
• Usually Grade II-III
• Bcl-2 usually negative (both protein & bcl-2R)
• Male: Female       ratio         3:1
• 85% present with Stage I or II disease
• 75% CR with low relapse rate
BL   LBCL      Children




BL      LBCL




                Adults
 Burkitt-like lymphoma should exist?

Burkitt-like lymphoma should be included in:


        Diffuse large B cell lymphoma

                     or

  Morphologic variant of Burkitt lymphoma
       Burkitt and Burkitt-like Lymphomas

May show follicular colonization
Cytologically tumor cells resemble the small blasts of the
dark zone of the GC

High rate of somatic mutations of Ig VH

BCL-6+, CD 10+

Evidence favors a germinal center derivation
                 Burkitt-like Lymphomas

• Similar to BL, but with greater variation in nuclear
  morphology

• MIB-1 100%, CD10 +, bcl-2 -

• Cytogenetics or molecular genetics desirable for diagnosis -
  should have a c-myc translocation

• This morphologic variant more common in immunodeficiency
  states
         In the WHO classification

The term “Burkitt-like lymphoma” is retained

Burkitt-like lymphoma will be considered a variant
of Burkitt lymphoma

                      Intermediate morphology

  Major criteria:     C-myc translocations

                      High proliferative fraction
           BURKITT LYMPHOMA

Morphologic Variants
     Classical Burkitt lymphoma
     Atypical Burkitt/ Burkitt- like
     With plasmacytoid differentiation (HIV -associated)

Subtypes, clinical genetic

      Endemic
      Sporadic
      Immunodeficiency-associated
          Anaplastic Large Cell Lymphoma


• Paradigm for process used to define disease entities
  (REAL)
• First recognized based on
   – Morphology - sinusoidal growth
   – Antigenic phenotype - CD30+
• Studies of molecular pathogenesis led to new diagnostic
  tools
   – RT-PCR and ALK-1 monoclonal antibody
• New diagnostic tools define the borderlands of the disease
      Anaplastic Large Cell Lymphoma
             Clinical Features

• Presents most commonly in lymph nodes
• Cutaneous involvement variable
• Most common in children, young adults
• Frequent presence of systemic symptoms
• “B” symptoms or high IPI less often predict poor
  outcome (in contrast to most other NHL)
• Aggressive natural history but good response to
  chemotherapy
DISTRIBUTION OF ANAPLASTIC LARGE CELL LYMPHOMA BY AGE
                      (267 cases)

                  120

                                                                                Sex Ratio= 1.34
                  100
Number of cases




                  80



                  60



                  40



                  20



                   0

                                                                                   > 59
                        0-9



                              10 - 19



                                        20 - 29



                                                  30 - 39



                                                            40 - 49



                                                                      50 - 59




                                                  Age
                 Survival of 154 patients with ALCL

           100
Survival




                                             ALK+ (n=132)
            75                               ALK - (n=22)
                                             p<0.001
            50


            25


             0         60     120     180     240      Months
        Clinical Significance of ALK +
     in Anaplastic Large Cell Lymphoma

• ALK+ cases are seen in younger age groups
   – Children and young adults; M>>F

• ALK+ cases have better prognostic than ALK-,
  irrespective of other clinical features; i.e. age, stage or
  IPI

Conclusion:
• ALK+ ALCL is probably a distinct disease entity,
  separate from ALK- ALCL

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:10
posted:6/22/2012
language:English
pages:18