Lymphoma
Key points
Hodgkin lymphoma is most common in children
Non-Hodgkin lymphoma (NHL) increases with age
Epstein-Barr Virus (EBV) is an important risk factor for Hodgkin lymphoma and Burkitt lymphoma in Africa
Immunocompromise and HIV are associated with mature B cell NHL
Hodgkin lymphoma is classified into:
Nodular lymphocyte predominant Hodgkin lymphoma: typically affects younger individuals, no B symptoms
Classical Hodgkin lymphoma: divided into four subtypes—nodular sclerosing (most common in children), mixed cellularity, lymphocyte rich, and lymphocyte depleted
Mediastinal lymphoma is most likely to be HL
Liver is the most common site of spread, followed by bone marrow
CNS involvement is unlikely in Hodgkin lymphoma
Common NHL subtypes include:
Burkitt lymphoma: most common NHL subtype, can present with intussusception and CNS disease.
Diffuse large B cell lymphoma and primary mediastinal B cell lymphoma: mature B cell subtypes
T lymphoblastic lymphoma: most likely NHL subtype to cause a mediastinal mass
Abdominal lymphoma is most likely to be a NHL
Arises in Peyers patches in ileocaecal region
Can mimic appendicitis
Burkitts lymphoma - most common cause of intussusception in children >4 years (Pathology review article)
'Sandwich sign' on CT if mesenteric vessels encased
Dawson criteria for primary GI lymphoma:
No palpable superficial lymphadenopathy
No involvement of mediastinal lymph nodes
White cell count within normal limits
No involvement of abdominal lymph nodes, except for those directly adjacent to the mass
No involvement of the liver or spleen
Post-transplant lymphoproliferative disease (PTLD) involves B-lymphocyte proliferation, almost always associated with EBV
Treatment for PTLD includes reduction or elimination of immunosuppressive medication.
If aggressive, treatment involves rituximab (anti-CD20) or conventional lymphoma chemotherapy
Investigations
Biopsy - lymph node excision if suitable palpable node
Radiology guided core biopsy if not
Bone marrow aspirate is required for all NHL cases and stage 3/4 Hodgkin lymphoma.
Lumbar puncture is required for NHL
CT CAP
18F-FDG PET
Lymphoma staging is via the Lugano staging system (modified from Ann Arbor)
Stages 1-2: Disease on one side of the diaprhagm
Stage 3-4: Advnaced disease on both sides of the diaphragm
Additional Designations:
A/B: Systemic symptoms (e.g. weight loss, night sweats) absent (A) or present (B) (note this one is only used in HL)
E: Extranodal spread within a radiation field.
Bulky: Large nodal mass >10 cm or >1/3 transthoracic diameter.
Histology
Reed-Sternberg cell - B cells with 'Owl eye appearance' - classical of HL
Burkitt lymphoma - CD10, CD19 and CD20 markers - numerous macrophages = 'starry sky appearance'
Diffuse large B cell lymphoma - centroblastic or immunoblastic differentiation - express CD19, CD20, CD79a+
Management
HL: ICE (ifosfamide, carboplatin, etoposide) + Radiotherapy
NHL: R CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride (hydroxydaunorubicin), vincristine sulfate (Oncovin), and prednisone) - not likely to need radiotherapy
Rituximab - targets CD20 on B cells
Outcome
70-95% Survival
Advanced stage - worse survival
Younger patients tend to have better outcomes than older patients, particularly for Burkitt’s lymphoma
lymphoblastic lymphoma and is associated with a poorer prognosis
An elevated lDH is also associated with poorer survival in Burkitt’s lymphoma
Standard scenario
Patient with cervical and mediastinal lymphadenopathy
Concern:
1. Airway compromise under general anaesthetic
2. Suitable location for biopsy
History
Onset of symptoms
Dysphagia, dyspnoea
Orthopnoea
Examination
Palpation of all lymph node zones for one suitable for excision biopsy + abdomen
Investigations
CXR then CT chest to look for airway compromise
If potential airway compromise -
Discuss with anaesthetist
Options -
Procedure under local anaesthetic
Sitting position + sedation with ketamine
Last resort is steroids + empirical treatment (Steroid treatment causes rapid regression in most cases which can mean that it is difficult to find suitable nodes for biopsy. In addition the histology may be compromised by steroid treatment, risking a delayed diagnosis)
Is central access required at the same time as biopsy?
If so - check that the vessels are not compressed by the disease
Special scenario - Abdominal lymphoma
Lymphoma found incidentally at operation e.g. Appendicectomy or as lead point in intussusception
Management
Descrub, discuss with oncologist if available and get parental consent - laparotomy and resect completely along with affected nodes - if possible - if not, biopsy and stoma
(2011 Retrospective study Kim et al - diffuse B cell lymphoma - surgery + chemotherapy superior survival vs chemotherapy alone)
For primary GI lymphoma - laparoscopic/open resection may be only method of obtaining tissue diagnosis
References
Jamil A, Mukkamalla SKR. Lymphoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560826/
Cheng J, Campos A, Weerakkody Y, et al. Lugano staging classification. Reference article, Radiopaedia.org (Accessed on 13 Aug 2024) https://doi.org/10.53347/rID-63811
Kim SJ et al. Comparison of treatment strategies for patients with intestinal diffuse large B-cell lymphoma: surgical resection followed by chemotherapy versus chemotherapy alone. Blood. 2011 Feb 10;117(6):1958-65. doi: 10.1182/blood-2010-06-288480. Epub 2010 Dec 9. PMID: 21148334.