Introduction

• Lymphoma is a malignancy of the lymphatic system.
• Categorized into Hodgkin Lymphoma and non-Hodgkin lymphoma.
• Include a wide variety of hematologic malignancies with varying clinical presentations.
• Some patients will present with symptoms secondary to extra-nodal involvement, which can include many common ED complaints such as GI complaints, headache, back pain etc.

Initial Presentation – Signs and Symptoms

Hodgkin Lymphoma – generally slow progression:

• Non-tender, firm lymph node (cervical/mediastinal more common).
• Constitutional symptoms: fever, night sweats, or unintended weight loss.
• Other symptoms: Pruritus, fatigue, alcohol-associated pain, splenomegaly, hepatomegaly.
• Incidental findings: Mediastinal Mass on CXR, Hypercalcemia, Anemia, Eosinophilia.

Non-Hodgkin Lymphoma – progression to extra nodal involvement can be rapid, wide variety of initial presentations:

• Most commonly: lymphadenopathy and constitutional symptoms similar to Hodgkin lymphoma.
• GI involvement: Abdominal mass/organomegaly, vomiting, early satiety, obstruction.
• CNS: headache, focal deficits, seizures, meningitis, back pain, cord compression.
• Tumor Lysis Syndrome: hyperkalemia, hyperphosphatemia, and hypocalcemia and associated symptoms.
• Other: Airway obstruction, pericardial tamponade, SVC syndrome.

Labs:

• CBC with differential
• CRP, ESR
• Extended electrolytes: Na, K, P, Mg, Ca
• LFTs & Kidney function
• LDH, uric acid
• HIV, Hep B & C

Imaging:

• CXR/Chest CT
• Further imaging depending on clinical presentation (eg. CT head, CT abdo-pelvis)

Ultimately patients will need lymph-node biopsy (excisional ideally), but this is not routinely done in the Emergency.

Patients will need to be referred to oncology for consideration for chemotherapy +/- radiation. Protocols vary based on classification of lymphoma.

Chemotherapy protocols and PPOs available from BC Cancer (link below).

When to use steroids:

• Patients with known lymphoma may present to ED with oncologic emergencies that may benefit from corticosteroids, 2 examples of this below.
• Generally pre-biopsy steroids are avoided if other treatments promptly available.
• Steroids can cause apoptosis in. lymphoma which has been reported in some cases to delay accurate histologic diagnosis.
• Spinal cord compression:
  • when signs and symptoms of cord compression (back pain, neurologic deficits) are present the entire spine should be evaluated with MRI ideally (CT if MRI not available).
  • Urgent neurosurgery/radiation oncology/medical oncology consultation.
  • If corticosteroids pursued, Dexamethasone (eg. 10-16mg IV, subsequently 16mg PO daily until definitive management).
• Hypercalcemia:
  • Hypercalcemia in most Hodgkin and up to a third of Non-Hodgkin lymphoma is due to increased calcitriol.
  • Hypercalcemia in these cases may respond well to corticosteroid treatment (eg. 20-40mg PO prednisone daily).
  • Symptomatic or severe hypercalcemia is also treated with IV hydration and calcitonin (4-8 IU/kg SC or IM q6-12 hrs) for acute management.
  • IV bisphosphonates (Zoledronic acid 4mg IV) and Denosumab (120mg SC) will reduce calcium over next few days. Generally, do not repeat dose within one week.

Complications secondary to treatment:

• Tumor lysis syndrome:
  • tumor lysis can release large amounts of potassium, phosphate, and uric acid.
  • Can be life threatening and can lead to AKI, arrhythmias, or seizures.
  • Cardiac monitoring and ICU admission likely needed.
  • Treatment involves IV fluids to maintain good urine output, treatment of hyperkalemia, phosphate binders can be considered to treat hyperphosphatemia, Rasburicase for hyperuricemia (0.2 mg/kg/day), and lastly calcium replacement once phosphate is corrected or if symptomatic (tetany/arrhythmias).
  • Dialysis is indicated if these measures do not fix above electrolyte abnormalities, or there is fluid overload/renal deterioration.
• Febrile neutropenia:
  • Treatment of febrile neutropenia involves recognition of fever or SIRS response in context of neutropenia and early initiation of empiric antibiotics.
  • Empiric treatment guidelines can be found at BC Cancer (link below).

• Chemotherapy Protocols BC Cancer
• Febrile Neutropenia Empiric treatment algorithm

1. Coiffier B, Altman A, Pui CH, Younes A, Cairo MS. Guidelines for the management of pediatric and adult tumor lysis syndrome: an evidence-based review. J Clin Oncol. 2008;26(16):2767-78.

   
   

2. Hingorjo MR, Syed S. Presentation, staging and diagnosis of lymphoma: a clinical perspective. J Ayub Med Coll Abbottabad. 2008;20(4):100-3.

   
   

3. Kumar A, Weber MH, Gokaslan Z, et al. Metastatic Spinal Cord Compression and Steroid Treatment: A Systematic Review. Clin Spine Surg. 2017;30(4):156-163.

   
   

4. Mirrakhimov AE. Hypercalcemia of Malignancy: An Update on Pathogenesis and Management. N Am J Med Sci. 2015;7(11):483-493.

   
   

5. 2019 surveillance of metastatic spinal cord compression in adults: risk assessment, diagnosis and management (NICE guideline CG75) [Internet]. National Institute for Health and Care Excellence. 2019 Feb 7.