Hey Everyone! Crazy oncology report today with Andrew and Josh presenting the case of a middle-aged man with shortness of breath and fevers, found to have endobronchial B-cell lymphoma almost completely obstructing the entire airway! Eek! We watched an awesome video of the interventional pulmonologists freezing and removing the endobronchial masses. Pearls below:
- DLBCL is the most common non-Hodgkin lymphoma.
- Lymphoma causing oncologic emergency (e.g. airway compromise) should be treated promptly with steroids with monitoring and prophylaxis for tumor lysis syndrome.
- Hodgkin and many non-Hodgkin lymphomas are curable in the majority of patients. Mantle cell lymphoma portends the worst prognosis of the group.
For those who want more info:
Lymphomas can be divided into Hodgkin and non-Hodgkin lymphoma. The most common non-Hodgkin lymphomas are: DLBCL (25%), mantle cell, Burkitt, CLL/SLL, follicular, lymphoplasmacytic, and marginal zone.
Lymphoid neoplasm classification was updated in 2016, including a lot of genetic markers to help with subclassification:
A brief primer on the five major categories of mature lymphoid neoplasms:
Hodgkin lymphoma is its own group including classical and nodular Hodgkin lymphoma.
Mature B-cell neoplasms include CLL, marginal zone lymphoma, hairy cell leukemia, myeloma, MALT lymphoma, follicular lymphoma, mantle cell lymphoma, DLBCL, Burkitt lymphoma, and many others.
Mature T and NK neoplasms include T cell leukemia/lymphoma, mycosis fungoides, Sezary syndrome, cutaneous T cell lymphoma, anaplastic large cell lymphoma, and others.
Post-transplant lymphoproliferative disorder (PTLD) is its own group.
Histiocytic and dendritic cell neoplasms include Langerhans cell histiocytosis, Erdheim-Chester disease, and others.
*Pearl: There is an “unclassifiable” B-cell lymphoma with features intermediate between DLBCL and classical Hodgkin, so not all tumors may fit perfectly into a clear category.
Treatment of Hodgkin lymphoma = ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine)
Treatment of most non-Hodgkin lymphoma = R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
What to do overnight for an oncologic emergency due to lymphoma (e.g. airway obstruction)?
- Check tumor lysis labs and LDH and start prophylaxis.
- Some chemo (cytoxan and rituximab) may be started as path is pending (call oncology!)
- Call relevant consultants (ENT for airway, neurosurg for spine, etc).
*Pearl: There are case reports of steroids causing tumor lysis syndrome in high risk patients! Start TLS prophylaxis even if you are just treating with steroids!
*Pearl: All DLBCL is at least intermediate risk for tumor lysis syndrome and should receive allopurinol (in addition to hydration and monitoring). Bulky DLBCL or LDH >2x ULN (and all Burkitt lymphoma) is high risk and merits rasburicase!
There are different prognostic scoring systems for each of the different lymphomas.
As an example, for DLBCL, risk factors for poor prognosis are older age, elevated LDH, poor performance status, advanced stage disease, and number of extra nodal disease sites. But the risk factors may differ slightly for each type of lymphoma.
Hodgkin: 5 year survival 98% for low risk, 67% for high risk.
DLBCL: 5 year survival 90% for low risk, 50% for high risk.
Burkitt: 5 year survival 90% in younger patients, 54% overall.
Follicular: 5 year survival 91% for low risk, 52% for high risk.
Marginal zone: 5 year survival 83% for low risk, 56% for high risk.
Mantle cell: 5 year survival 60% for low risk, 20% for high risk (worst prognosis).
Have a great day everyone!