What is the typical translocation of ALCL?
t(2;5)(p23:q35)
Leads to constitutional activation of ALK
Name 4 common sites of extranodal disease in ALCL
Skin, soft tissue, bone, lung
What are hallmark cells?
Typical cells of ALCL: horseshoe-shaped, eccentric nucleus
Name the 3 subtypes of Burkitt lymphoma; state how often it is associated with EBV positivity
Sporadic (10-15%)
Endemic (95%)
Related to immunodeficiency (40%)
Name the typical translocations associated with Burkitt
t(2;8) - Ig K chains
t(8;14) - IgH
t(8;22) - Ig light chains
What is the immunophenotype associated with mature B cell lymphoma (Burkitt, DLBCL)?
(+)ve: CD19, C20, CD22, CD79a, sIg
(-)ve: CD10, tdT, CD5, CD23
What is the outcome of Burkitt lymphoma, both 1) localized and 2) disseminated?
1) EFS: 90%, OS 95%
2) EFS: 80%
Name 2 novel prognostic risk factors in ALCL
1) Anti-ALK antibodies in blood at diagnosis (protector effect)
2) Copy number of NPM-ALK variants in bone marrow at diagnosis (associated with higher relapse rates)
Name the 3 histologic variants of DLBCL
Centroblastic; immunoblastic; anaplastic
Define Group A for localized mature B-cell neoplasm
As per the current standard of care, what is the treatment (group A)?
Fully resected Stage I or stage II disease
COPAD * 2
When do relapses occur in ALCL?
Within 2 years of initial treatment
What are the late effects of therapy of NHL treatment?
Very rare compared to other cancers
Cardiotoxicity
HCV infections from transfusion (old studies)
Second malignancies (5% at 20y)
Risk factors for PTLD in solid organ transplants
- Type of transplant (worse with heart, lung, GI, compared with kidney or liver)
- EBV negativity in recipient
- CMV negativity (less consistently reported than EBV)
- Degree of immunosuppression
Risk factors for PTLD in HSCT
- T-cell depleted stem cell source
- Conditioning regimen including antithymocyte globulin
Late effects of HD attributable to radiation therapy (7)
- Growth impairment
- Cardio-vascular disease (coronary artery disease, valvular disease, pericardial disorders)
- Stroke
- Infections (splenic radiation)
- Thyroid dysfunction
- Infertility
- Lung fibrosis
- Second malignancies
Late effects of HD attributable to chemotherapy (4)
- Cardiomyopathy (anthracyclines)
- Lung dysfunction (bleomycin)
- Male infertility (alkylating agents, procarbazine)
- Hematologic malignancies (alkylating agents, topoisomerase II inhibitors)
Risk factors for anthracycline-related toxicity (4)
- Younger age
- Female sex
- Cumulative doses of anthracyclines
- Combination of mediastinal radiation
Risk factors for lung toxicity (3)
- Cumulative doses of toxic agents: bleomycin, BCNU, CCNU
- Combination of chemotherapy and lung radiation
- Younger age
Name 6 prognostic factors in ALCL
Age
Histologic subtype
High MDD
High MRD
Increased copy number variants of ALK in bone marrow at diagnosis
Absence of anti-ALK Ig in peripheral blood at diagnosis