Proposed WHO Classification of Lymphoid neoplasm

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Proposed WHO Classification of Lymphoid neoplasm
B cell neoplasm
Precursor B cell neoplasm
Precursor B-lymphoblastic leukemia/lymphoma (precu
rsor B-ALL)
T cell neoplasm
Precursor T-cell and NK-cell neoplasm
Precursor T-lymphoblastic lymphoma/leukemia
(precursor T-ALL)
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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
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Mature (peripheral) B cell neoplasm (contd)
Diffuse large B cell lymphoma
Mediastinal B cell lymphoma
Primary effusion lymphoma
Burkitt lymphoma/Burkitt cell leukemia
4
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
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Mature (peripheral) T cell neoplasm (contd)
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
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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
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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 31
• 85 present with Stage I or II disease
• 75 CR with low relapse rate

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BL
Children
LBCL
BL
Adults
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Burkitt-like lymphoma should exist?
Burkitt-like lymphoma should be included in
Diffuse large B cell lymphoma
or
Morphologic variant of Burkitt lymphoma
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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
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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

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In the WHO classification
The term Burkitt-like lymphoma is retained
Burkitt-like lymphoma will be considered a
variant of Burkitt lymphoma
Major criteria
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BURKITT LYMPHOMA
Morphologic Variants
Classical Burkitt lymphoma
Subtypes, clinical genetic
Endemic
Sporadic
Immunodeficiency-associated
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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

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Anaplastic Large Cell LymphomaClinical 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

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DISTRIBUTION OF ANAPLASTIC LARGE CELL LYMPHOMA BY
AGE (267 cases)
Sex Ratio 1.34
Number of cases
Age
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• Survival of 154 patients with ALCL

100
ALK (n132)
Survival
ALK - (n22)
75
plt0.001
50
25
0
120
180
240
60
Months
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Clinical Significance of ALK in Anaplastic
Large Cell Lymphoma

• ALK cases are seen in younger age groups
• Children and young adults MgtgtF
• 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