Clinical presentation

Etiology

History

HL is named after Thomas Hodgkin, a British pathologist, who in 1832 described the disease. 150 years later, with the advent of microscopic histology, Sternberg (1898) and Reed (1902) described the distinctive multinucleated giant cell with the prominent nucleoli that are characteristic of Hodgkin’s disease.

The exact etiology of HL is unknown; however, an infectious cause has been suggested. It is likely associated with the Epstein-Barr virus (EBV). This concept is supported by the presence of EBV in serologic studies and biopsy material from Hodgkin's lymphoma patients.

Immune system dysfunction is hypothesized to be one of the primary causes for HL.

HL must be considered in any child with lymphadenopathy. Involved nodes are described as firm, nodular, and painless (Image 9.23). Children and adolescents most frequently present with cervical and or supraclavicular lymphadenopathy (80%). Patients presenting primarily with enlarged axillary nodes (25% of all cases) or inguinal nodes (5%) are far less common. Associated mediastinal disease is found in up to 75% of adolescents and 33% of children.

The presence of facial swelling or distended cervical veins alerts the physician to mediastinal disease and the possibility of airway obstruction. Axillary and inguinal lymph nodes can be involved, but primary axillary or inguinal tumors are uncommon.

Image 9.23 Hodgkin's lymphoma. The most common childhood presentation of HL is painless cervical or supraclavicular adenopathy.

Systemic symptoms (B symptoms) occur in approximately one-third of children at the time of diagnosis and indicate advanced disease. Systemic symptoms include:

· recent (<6 months) loss of greater than 10% body weight,

· drenching night sweats, and

· fever exceeding 38°C.

These symptoms are not specific to HL and can occur in non-Hodgkin lymphoma. The presence or absence of B symptoms, which occur in up to a third of children, has prognostic significance and is reflected in the staging of HL.

Pruritis, lethargy and anorexia are no longer considered prognostic symptoms. Splenomegaly and/or hepatomegaly indicate more advanced disease.

Depending on the primary site of the disease and its extent, patients may present with airway obstruction, pleural or pericardial effusion, hepatocellular dysfunction, anemia, neutropenia, thrombocytopenia and/or nephrotic syndrome. Respiratory distress due to mediastinal lymph node enlargement is much less common in children with HL than in children with NHL but it should always be considered prior to general anesthesia and biopsy.

Дата добавления: 2014-10-15