and Mhamed Harif2
(1)
South African Medical Research Council, Cape Town, South Africa
(2)
Université Mohammed VI des Sciences de la Santé Cheikh Khalifa Hospital, Casablanca, Morocco
Keywords
Cancer of lymph nodeLymph node biopsiesA and B symptomsReed-Sternberg cellsBimodal distributionLymph node enlargementClassical nodular sclerosisMixed cellularityLymphocyte depletedLymphocyte predominantAnn ArborCase Presentation
A 14-year-old girl presented to the hospital with fever, sweating, loss of weight for the last 6 months and lower back pain for a week. On physical examination the patient was not acutely ill, had moderate pallor and lymphadenopathy along the left jugular chain, small, not tender and mobile palpable lymph nodes. Two firm tender lymph nodes (5 × 2 cm diameter) in the right inguinal area were noted. The lower lumbar spine was tender to palpation but no deformity noted. Rest of the exam was unremarkable (Figs. 14.1 and 14.2).
Fig. 14.1
Lymphadenopathy
Fig. 14.2
Lymphadenopathy
What Is the Differential Diagnosis?
Differential diagnosis.
Infections : EBV, HIV, tuberculosis, toxoplasmosis, cat scratch disease, histoplasmosis.
Lymphomas: NHL, Hodgkin lymphoma.
Metastatic adenopathy from solid tumors.
Autoimmune disorders : lupus, autoimmune lymphoproliferative disorder, Rosai Dorfman disease.
What Investigations Would You Like to Request?
CBC, ESR, CRP, peripheral smear, LDH, biochemistry
Chest X-ray PA view and lateral view and spine
CT scan of the neck, chest, abdomen, and pelvis
Lymph node biopsy
HIV, EBV, CMV
Herein listed are available results for your case scenario:
WBC | HB | MCV | PLT | N | L | Na+ | K+ | Ur | Cr | Ca++ | Mg++ | PO4 | UA | HIV |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
6.3 | 9.9 | 83 | 219 | 3.5 | 1.7 | 136 | 3.6 | 2.7 | 46 | 2.2 | 0.7 | 0.9 | 0.35 | Neg |
Peripheral blood film: No abnormal cells (Figs. 14.3 and 14.4).
Fig. 14.3
Chest X-ray (a and b)
Fig. 14.4
Confirmation of diagnosis by biopsy with the presence of Reed-Sternberg cells
Epidemiology and Etiology
Hodgkin lymphoma amou nts to around 10 % of all childhood cancers and roughly 17 % of all cancers in adolescents.
This disease is predominantly present in boys (ratio M/F = 3:4) and primarily affects the lower socioeconomic class.
This is the most common childhood cancer in 15–19-year-olds and has a bimodal distribution with peaks in the late teens and then later above age 55 years.
In young children in Westernized populations this disease is rare, but in developing countries it is more prevalent among these children.
The lymph nodes are mainly affected and are characterized by the presence of Reed-Sternberg (RS) cells in the tumor tissue.
The Epstein–Barr virus (EBV) and Hodgkin Lymphoma often coexist but causality is not well understood. The EBV genome is frequently found (up to 50 %) in tumor tissue. This association is more common in young children and in the mixed cellularity type which is more commonly found in developing countries (up to 90 %). However, its exact role in the carcinogenesis of the disease is not established.
The hypothesis of an abnormal immune response was also raised. The higher incidence in patients with acquired immune deficiency or constitutional (ataxia telangiectasia syndrome Purtillo) argues in favor of this hypothesis. Other risk factors are a family history and late exposure to oral pathogens.
The prognosis has been radically transformed by combining chemotherapy and radiation treatments that allow a survival rate of 90 %. New therapeutic approaches aim at maintaining high survival rates with minimal sequelae.
In Africa
The real incidence of HL on the African continent is not known because of lack of data and registries. A study looking at the patterns of distribution of childhood cancer showed that HL represented 9 % of the total childhood cancers in Senegal, 8.4 % in Mali, and 7.6 % in Kenya. Other studies reported an incidence of 6.8 % in Benin and Nigeria, and 7 % in Morocco and Namibia.
Clinical Presentation
Hodgkin lymphoma is characterized by progressive lymph node enlargement . More than 95 % of cases present at a site above the diaphragm.
This disease is unicentric in origin and begins in one node or group of nodes. Subsequently, it spreads in a predictable way to contiguous nodes which is more likely associated with the nodular sclerosing subtype. Whereas noncontiguous spread is more likely involved with mixed cellularity and lymphocyte depletion.
The disease can present with general signs: pruritus, fever, anorexia, weight loss, and/or night sweats.
Taking down history is important and involves looking for the symptoms that would classify the disease as A (patients without symptoms) or B (patients with symptoms). The presence of symptoms of heavy night sweating (drenching), fever (>38 °C for 3 days or more), and weight loss of >10 % in the preceding 6 months are specifically important aspects to ask about.
Other history is the presence of nodes, which is important if these have been present for a long time and may have increased or decreased in size over time.
The lymph nodes, especially in the neck are the most affected and represent the usual mode of revelation of the disease. The lymph nodes are usually painless, mobile, and in groups.
Of special interest is the presentation of the patients with signs and symptoms suggestive of tuberculosis. The misdiagnosis, in these cases, can lead to a delay in the diagnosis and treatment of the lymphoma. In countries with a high incidence of tuberculosis, the coexistence of the two diseases may present a challenge for the administration of the therapy and the management of added side effects.
Deep mediastinal lymphadenopathy can be present and is initially asymptomatic. These lymph nodes can transform in the rapidly evolving compressive forms which will then cause coughing or true compression: the superior vena cava syndrome.
In the mediastinal location, the bulky form is defined by a mass exceeding 33 % of the diameter of the thorax through D5–D6 or more than 10 cm maximum diameter.
Other locations are less frequent.
Pulmonary involvement is often associated with mediastinal lymph node localization, and is best demonstrated by a CT scan, which should always be recommended (if available) to clarify the subsequent radiation fields. This may be through an extension to the adjacent lymph nodes or rarely because of hematogenous dissemination.
Neurological involvement is usually late in the disease progression, and may also be related to a particular compression locoregional if paravertebral lymph nodes are involved. This may then give rise to epidural spinal cord compression, and less frequently involve hematogenous spread.
Intracerebral damage , described in the literature, can be characterized by cerebral seizures or neurological deficits, though this is not a common finding.
Bone involvement is usually demonstrated as bone pain and is rarely isolated.
Involvement of the spleen and/or liver is frequently found. Symptoms of bleeding and jaundice may indicate the presence of immune hemolytic anemia and thrombocytopenia.
Involvement of the bone marrow can be suspected in the clinical signs of the step by bone marrow failure. This disease is often subclinical and affects the blood count.
In exceptional cases, the disease can be revealed and accompanied by autoimmune thrombocytopenic purpura or autoimmune hemolytic anemia.
In Africa
Children present with advanced disease and associated comorbidities. Tuberculosis and HIV may coexist and do not exclude the presence of the lymphoma.
Bulky disease is often encountered and confirmed by chest X-rays (Fig. 14.5). Superior vena cava syndrome if present should be treated immediately.
Fig. 14.5
Bulky disease confirmed by chest X-rays
Diagnosis of HL and Histology
The diagnosis of Hodgkin lymphoma is based on the histopathology of a biopsy of a lymph node or default of another organ or tissue.
FNA (fine needle aspiration ) can diagnose Classical Hodgkin lymphoma together with immunostains but cannot identify subtypes, which are not needed for treatment.
Description of Pathology
Diagnosis is made by the presence of RS cells in the context of inflammatory granuloma resulting from lymphocytes, granulocytes, and plasma cells and eosinophils. The RS cells are large cells usually having two lobes with prominent nucleoli (Fig. 14.6).
Fig. 14.6
Reed-Sternberg cells
The WHO recognizes two major subtypes of HL: the classical and nodular lymphocyte predominant HL (Fig. 14.7a, b below—pathology classification).
Fig. 14.7
Pathology classification of Hodgkin lymphoma
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