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Lymphadenopathy in African Children 243

Other lymph node groups (e.g., cervical, intraabdominal) may occa- Age for lymph nodes
sionally be involved. Castleman disease is not always a benign disorder
3
in children, and associated systemic manifestations may or may not be
present. There are two clinical types, localised and multicentric, as well
as three histological variants (hyaline-vascular, plasma cell, and mixed
type). Of these, the plasma cell and mixed type appear to be more
aggressive than the hyaline-vascular type.
Although the aetiology of Castleman disease is uncertain,
multicentric Castleman disease (a lymphoproliferative disorder) and
Kaposi sarcoma have both been reported to have an increased
occurrence in patients with HIV infection. This is as a result of the
ability of the human herpes virus (HHV) 8 to persist in B-lymphoid

cells and endothelial cells, and the current HIV epidemic has raised a
new awareness of this association. Age for neoplastic nodes
Surgical excision is the treatment of choice for Castleman disease,
but it is not always possible to obtain a complete clearance.
Malignancy and Lymphadenopathy
Problems still exist in excluding malignancy in lymphadenopathy in child-
hood, which results in difficult clinical management decisions. All efforts
should be taken to achieve a definitive diagnosis. Repeated sampling of
deeper nodes may be indicated if difficulties persist, and results may be
further improved by sampling multiple lymph nodes and taking cultures.
The differential diagnosis of cervical lymphadenopathy includes
malignant tumours. The overall risk of malignancy is approximately
12% (1 in every 8 lymph nodes biopsied) in chronic cervical
lymphadenopathy. The similar incidence of reactive lymphadenopathy
and chronic granulomatous infections (of different kinds) in most
series suggests a similar incidence of lymph node malignancy in both
developed and developing countries.
1
In our own series, lymphomas were prominent in 70% of patients.
These may be divided into Hodgkin’s disease, non-Hodgkin’s Figure 37.4: Age range of 1,877 patients with biopsy of enlarged lymph nodes.

lymphomas, and Burkitt lymphoma in children. Each of the above Note the increased incidence of neoplastic nodes with increasing age (right)
groups represented approximately one-third of the total cases. when compared to the overall decrease in general (left).
Age
The mean age of patients with a malignancy was 8.5 years in our own Oncology patients

1
series (Figure 37.4), but some age differences are evident. Burkitt Oncology patients warrant special consideration as far as tuberculosis
lymphoma, for instance, usually presents almost exclusively in younger is concerned, and active TB should be excluded before commencing
children (mean age of 5 years), whereas Hodgkin’s and other lympho- chemotherapy in patients at risk. Further identification of latent TB
mas occur predominantly in the older age group. Nonlymphoma-related in oncology patients from endemic areas and the early introduction of
malignancies in the head and neck region have been reported to occur prophylactic anti-TB treatment during the early stage of chemotherapy
more frequently at the age of 5 years of less. In our series of 1,877 might be indicated in these patients. The importance of follow-up of
specimens, these included neuroblastomas (13) and rhabdomyosarco- patients with nonspecific reactive nodes is illustrated by the fact that 15
1
mas (4), as well as local spread from thyroid tumours (3) and nasopha- cases (3%) in our series were subsequently diagnosed with lymphoma,
ryngeal carcinoma (5). There were also a number of cervical metastases and patients identified as having an “atypical hyperplasia” should there-
from advanced tumours at distant sites (e.g., Wilm’s tumours, hepato- fore undergo repeat biopsy.
cellular carcinomas, sarcomas, malignant melanomas, teratomas, and Rarer Causes of Lymphadenopathy
ovarian tumours). Other less obvious causes of lymphadenopathy (e.g., CMV, toxoplas-
mosis, cat scratch disease) can be identified only with special tests.
Burkitt lymphoma
A special situation occurs with Burkitt lymphoma, a childhood cancer Lymphadenopathy and viruses
common in sub-Saharan Africa. Burkitt lymphoma has an extremely Many childhood viral diseases (e.g., measles, rubella, infected chicken
rapid growth and a 24-hour doubling time. It has a relationship to pox) are associated with lymphadenopathy but are not the primary
the Epstein-Barr virus (EBV, an important cause of a variety of viral presenting feature. CMV, EBV, and HHV, especially types 6, 7, and 8)
conditions as well as malignancy) and malaria, but its association with have been associated with lymphadenopathy.
HIV is not clear as yet. The HIV pandemic as well as the increase of Cytomegalovirus and lymphadenopathy
malaria in Africa have resulted in an increase of Burkitt lymphoma Most CMV infections are subclinical, including those acquired con-
in HIV-endemic areas, particularly in HIV-infected children. Recent genitally. The course of CMV mononucleosis infection is generally
studies suggest that EBV and malaria, and possibly HIV, act jointly in mild, lasting 2 to 3 weeks. The later stages may have lymphadenopathy
4
the pathogenesis of Burkitt lymphoma. Malaria prevention may thus as a feature, but that is usually atypical.
potentially decrease the risk of Burkitt lymphoma. As the diagnosis It is generally accepted that immunosuppressed or immunocompro-
and introduction of chemotherapy is urgent, there is an acute need for mised patients (especially those with HIV and bone marrow transplant
improved rapid diagnostic methods as well as early, appropriate onco- patients) may experience more severe human CMV (HCMV) infection
logic management. In addition, less toxic drug combinations need to be and disease. Control of HCMV infection and prevention of associated
utilised for HIV-infected patients. diseases in immunocompetent hosts are ensured mainly by CD8+ T cells.