Lymphomas are the third most common childhood cancers of which 40% are Hodgkin's disease.

Q. What is hodgkin's disease?

A. It is a lymphocytic cancer originating from B or T lymphocytes. It is characterized by pleomorphic lymphocytic infiltrate with malignant multinucleated giant cells Reed-Sternberg cells).

Q. What are its clinical features and how does it present?

A. Hodgkin's disease has a bimodal peak with an early peak in late 20s and a second peak in late adulthood. In developing countries, the early peak occurs before adolescence. Hodgkin's disease is rare before 5 years of age. In children < 10 years, the incidence is more in boys. In developing countries, it has been associated with the presence of Ebstein Barr virus in the Reed-Sternberg.

Clinically, the patients present with painless enlargement of the lymphnodes, commonly in the supraclavicular and cervical region. The enlarged nodes are firm and rubbery. Sometimes the nodes may fluctuate in size. Almost 60% of the patients may have mediastinal disease at diagnosis. Patients may also have systemic symptoms such as fever, night sweats and weight loss most probably due to release of lymphokines by the Hodgkin's cells. The disease spreads contiguously via the lymphatics from one node to another; in later stages it may disseminate to organs

Q. How is Hodgkin's disease diagnosed ?

A. Any peripheral lymph node > 1 cm in size that does not regress after 6 weeks of observation should be biopsied. (Care should be taken not to biopsy the edge of the lymph node as it as it may appear as "reactive" on histopathology, but central nodes should be biopsied.) Once, the diagnosis is confirmed, it is necessary to determine the stage of the disease for prognosis and treatment evaluation. Staging is based on a combination of history, physical examination, and x-rays (including CT Scan of chest, abdomen and pelvis), gallium scan, bone marrow biopsy and laboratory biochemical profile. In patients with mediastinal mass, if pleural effusion is present, a thoracocentesis should be done as cytological diagnosis is possible. Mediastinoscopy, anterior mediastinotomy and thoracoscopy are procedures of choice when other methods fail to establish the diagnosis.

Pathologic staging requires laparotomy with splenectomy, retroperitoneal lymph node biopsy and liver biopsy. However in children, chemotherapy alone, has been used increasingly for treatment. Hence, staging laparotomy is rarely required.

Q. How do you stage Hodgkin's lymphoma ?

A. Hodgkin's disease is staged by the Ann Arbor staging system. It is divided into 4 stages and sub classified into A & B categories with a special subcategory E.

A:- Patients are asymptomatic

B:- Patients with following symptoms -

• Unexplained loss of weight > 10% in last 6 months.
• Unexplained fever with temperature > 38 degree Celsius for more than 3 days.
• Drenching night sweats.

E :- Minimal extralymphatic disease from direct extension. It denotes limited involvement of a single extranodal site.

Stage I - Involvement of a single lymph node region (I) or single extralymphatic site (IE).

Stage II - Involvement of 2 or more lymph node regions on same side of diaphragm (II) or extension from these lymph nodes to extralymphatic adjacent organ (IIE).

Stage III - Involvement of lymph nodes on both sides of diaphragm (III); with extension to an adjacent extralymphatic organ (IIIE) or involvement of the spleen (IIIS+) or both (IIIE+S).

Stage IV - Disseminated disease with or without associated lymph node disease. (Occurs due to spread through the blood stream as opposed by lymphatics).