Mature B-Cell Lymphoma And Leukemia Study III

1. This study will perform analysis of newly diagnosed mature B-cell lymphomas (e.g.
Burkitt lymphoma/leukemia, DLBCL, and MLBCL) obtained from participants in different
parts of the world.

2. This study will describe the types and frequency of mutations in the ARF-HDM2-TP53
pathway, in B-cell lymphomas in the United States and that found in selected geographic
regions of the world.

3. This study will describe the expression of ARF-HDM2-TP53 and PUMA-associated pathways in
B-cell lymphomas in the United States and that found in B-cell lymphomas of other
selected geographic regions of the world.

4. This study will describe the pattern and frequency of XLP gene mutations presenting with
B-cell lymphomas in the United States and selected geographic regions.

5. This study will describe the frequency of EBV-positive B-cell lymphomas in the United
States and selected geographic regions of the world: and will describe the pattern of
EBV protein and gene expression (e.g., EBNA 3) in EBV-positive lymphomas and the study
will compare patterns of EBV protein and gene expression with clinical, laboratory and
outcome data.

Secondary Objective:

To estimate the complete response rate, event-free survival, and overall survival rates in
patients with Burkitt lymphoma (BL), Burkitt leukemia/B-cell acute leukemia (B-ALL) and
diffuse large B-cell lymphoma (DLBCL) treated with a stage-adapted regimen based on the St.
Jude B-cell II protocol.

Inclusion Criteria:

St. Jude participants and collaborating sites participating in therapeutic and biological
objectives:

1. Participant must have a histologic diagnosis of a mature B cell lymphoma (e.g.,
Burkitt lymphoma/leukemia, atypical Burkitt lymphoma, diffuse large B-cell lymphoma,
mediastinal large B-cell lymphoma, mature B-cell lymphoma NOS) as defined in the WHO
classification.

2. Participant must be previously untreated, (no more than 72 hours of steroids, one
intrathecal chemotherapy treatment, and/or emergency radiation)

3. Participant must be < 22 years of age at the time of diagnosis

4. For selected higher-risk Group B participants receiving rituximab only (e.g., those
with MLBLC, Stage III with LDH ≥ 2 times upper limit of normal (ULN), and/or bone
marrow/CNS involvement. All participants who receive rituximab must have hepatitis
screening prior to enrollment. Participants whose results indicate that they are
carrier of hepatitis B can still be treated per Group B but will NOT receive
rituximab. This screening must be done for eligibility BUT the results are not needed
prior to enrollment:

- Hepatitis B immunization status (vaccination Yes or No)

- HBsAg

- Anti-HBs antibody

- Anti-HBc antibody.

5. HIV test has been obtained within 42 days. Participants who test positive for HIV
cannot be enrolled on therapeutic part of study, but are still eligible for biology
studies.

6. Informed consent must be obtained according to St. Jude guidelines before enrollment
into study

Participants from Collaborating Sites Participating in Biological Objectives Only:

1. Participant must have a histologic diagnosis of a mature B cell lymphoma (e.g.,
Burkitt lymphoma/leukemia, atypical Burkitt lymphoma, diffuse large B-cell lymphoma,
mediastinal large B-cell lymphoma, mature B-cell lymphoma NOS) as defined in the WHO
classification

2. Participant must be < 22 years of age at the time of diagnosis

3. Participant must be previously untreated (no more than 72 hours of steroids, one
intrathecal chemotherapy treatment and/or no emergency radiation)

4. Informed consent must be obtained by local PI or his/her designee according to
ICH/Good Clinical Practice and local guidelines before enrollment into study

Exclusion Criteria:

Participants from Collaborating Sites Participating in Therapeutic and Biological
Objectives:

1. Participants known to be HIV positive (for therapeutic part of protocol, HIV
participants are eligible for biology studies).

2. Participants who are pregnant or lactating

3. Inability or unwillingness of research participant or legal guardian to consent

Participants from Collaborating Sites Participating in Biological Objectives Only:

1. Inability or unwillingness of research participant or legal guardian to consent

2. Histologic diagnosis other than a mature B-cell lymphoma as defined in the WHO
classification