Treatment of Chronic Urticaria Associated with Lymphoma
Patients with chronic urticaria associated with lymphoma should be treated with cimetidine, carbamazepine, gabapentin, or mirtazapine as first-line pharmacotherapy, with oral corticosteroids reserved for incurable cases and phototherapy as adjunctive treatment. 1
Primary Treatment Options
First-line Medications
- Antihistamine-resistant cases:
- Cimetidine
- Carbamazepine
- Gabapentin
- Mirtazapine
These medications have demonstrated efficacy in resolving itch associated with lymphoma-related urticaria, though evidence is primarily based on case reports and small case series 1.
For Incurable Lymphoma
- Oral corticosteroids are recommended for palliative itch relief in patients with incurable lymphoma 1
- Typically initiated at doses equivalent to prednisone 40 mg daily
- Aim to reach an effective low, alternate-day dose followed by discontinuation when possible
Phototherapy Options
- For Hodgkin lymphoma: Broadband UVB (BB-UVB) phototherapy provides temporary itch relief 1
- For Non-Hodgkin lymphoma: Narrowband UVB (NB-UVB) phototherapy is recommended 1
Treatment Algorithm
Definitive treatment: Curative treatment of the underlying lymphoma, which invariably resolves associated pruritus 1
While awaiting definitive treatment:
- Start with one of the recommended medications: cimetidine, carbamazepine, gabapentin, or mirtazapine
- Consider adding appropriate phototherapy based on lymphoma type
- For severe, refractory symptoms or incurable cases, add oral corticosteroids
For persistent symptoms despite above measures:
- Consider immunomodulating therapies such as ciclosporin, which has shown efficacy in severe autoimmune urticaria unresponsive to antihistamines 1
Diagnostic Considerations
Chronic urticaria may sometimes be the presenting symptom of lymphoma. Key diagnostic clues include:
- Intense itching at night associated with weight loss, fevers, and night sweats 1
- Enlarged lymph nodes or masses requiring excision or ultrasound-assisted core biopsy 1
- Occasionally, normal-appearing skin that reveals skin lymphoma on biopsy 1
Monitoring and Follow-up
- Regular monitoring of blood counts, lactate dehydrogenase, and ESR
- Skin biopsy may be necessary in persistent, unexplained pruritus with normal-appearing skin 1
- Periodic reassessment of treatment efficacy and disease status
Important Caveats
- While traditional antihistamines are first-line for most urticaria cases, they are often ineffective in lymphoma-associated urticaria, necessitating the specialized approaches outlined above
- Sedating antihistamines should be avoided long-term except in palliative care settings due to potential adverse effects 1
- The prognosis for resolution of urticaria is directly tied to successful treatment of the underlying lymphoma
- In rare cases where chronic urticaria is the initial presentation of lymphoma, failure to respond to conventional urticaria treatments should prompt investigation for underlying malignancy 2
By following this treatment approach, most patients with lymphoma-associated urticaria can achieve significant symptom relief while undergoing definitive treatment for their underlying malignancy.