A 29-year-old woman presents to her doctor in the 30th week of a previously normal pregnancy. She has recently noticed some chest discomfort and a progressive feeling of swelling and tightness in her face. On examination she has obvious lymphadenopathy in the neck and signs of early superior vena cava obstruction (SVCO). A neck node is biopsied under local anaesthetic and proves to be a diffuse large B-cell non-Hodgkin lymphoma (DLBCL).
How does the pregnancy affect the approach to investigating and staging the disease?
What are the options for managing the patient’s lymphoma at 30 weeks of pregnancy?
What are her outcomes and what future therapy is available should she relapse?
Non-Hodgkin lymphoma (NHL) is a broad term encompassing many distinct lymphoid malignancies. The histological classification is complex, but clinically there remains a working distinction between aggressive and indolent forms of the disease. Diffuse large B-cell non-Hodgkin lymphoma represents about 30% of NHLs and is the commonest aggressive lymphoma. It classically affects lymph nodes, spleen, liver or bone marrow but not infrequently spreads to other viscera, skin or the central nervous system (CNS). Initial investigation requires a good-quality biopsy and detailed staging procedures, which always include clinical history and examination, blood tests, computed tomography scanning (chest, abdomen and pelvis) and examination of the bone marrow. The lactate dehydrogenase (LDH) level is a useful marker of DLBCL; a raised level has prognostic significance at diagnosis and can be a sign of relapse during follow-up. These elements of diagnosis and staging produce clinical and laboratory information that has been used to construct prognostic scores for DLBCL, the most validated of which is the International Prognostic Index (IPI).1 Depending on the number of risk factors the patient has at diagnosis, their anticipated 5-year survival ranges from 26% to 73%. The IPI risk factors, prognostic groups and outcomes are shown in Figure 29.1. It should be noted that age alone (below or above 60 years) is the most powerful single predictor.
The International Prognostic Index: risk factors, prognostic groups and survival (adapted with permission1).
First-line treatment is chemotherapy based although some elderly individuals with early stage disease may do well with local radiotherapy. In the UK, the standard of care is the administration of six to eight courses of CHOP (cyclophosphamide, adriamycin [doxorubicin], vincristine and prednisolone) chemotherapy at 21-day intervals with the addition of an infusion of the anti-CD20 monoclonal antibody rituximab with each of the courses. This combination is referred to as R-CHOP21.2 For early (localized) disease, the number of courses of R-CHOP21 may be reduced to three and radiation given to the area involved. This produces excellent results and minimizes toxicity. Attempts to intensify front-line chemotherapy have met with limited success and greater toxicity and CHOP has stood the test ...