Hematopathology Case Studies


CASE 5: Hodgkin disease (Hodgkin lymphoma), nodular sclerosis type


Clinical History:

A 27-year-old woman presents to her physician with a month-long history of dull chest pain. She has no dyspnea and reports no hemoptysis. On physical examination, her lungs are clear to auscultation. She has no lymphadenopathy or hepatosplenomegaly. She does have plethora of the head and neck region. A CBC shows Hgb 13.1 g/dL, Hct 39.5%, MCV 91 fL, platelet count 215,000/microliter, and a WBC count of 6350/microliter. Resection of a lesion is performed.
  1. What would a chest radiograph reveal?

    2. There is a middle to anterior mediastinal mass. A mass in this location may have produced some superior vena cava obstruction to produce the plethora noted on physical examination. At thoracotomy a 5 x 8 cm mass was resected. At this location it is probably arising in lymph nodes.

  2. What histologic features are noted at low power?

    3. Under low power, the lymph node capsule is markedly thickened and the lymph node parenchyma is traversed by bands of collagen, dividing the node into nodules. The normal nodal architecture is obliterated by this process.

  3. What type of cells are present in the nodules? What surrounds the nodules?

    4. At higher power, you can see that small lymphocytes are mixed with some large cells with lobulated nuclei, or with multinucleated cells that have prominent nucleoli (Reed-Sternberg cells). Some of these large cells appear to be lying in a space (lacunar or variant Reed-Sternberg cells). Cytokines such as IL-5, IL-6, IL-13, tumor necrosis factor (TNF), and GM-CSF promote formation of the reactive infiltrate of lymphocytes, macrophages, and fibroblasts which form the bulk of the mass.

  4. What is your diagnosis in this case?

    5. The diagnosis is: Hodgkin disease (Hodgkin lymphoma), nodular sclerosis type.

  5. What is the prognosis in this case?

    6. Nodular sclerosis is the most common type of Hodgkin disease (the other types being lymphocyte depletion, mixed cellularity, and lymphocyte predominance). Nodular sclerosing Hodgkin disease (NSHD) often involves the mediastinum and it is generally associated with a good prognosis. More than 80% of patients with Hodgkin disease can be cured, particularly the younger patients, those with the lymphocyte predominance and nodular sclerosis types, and those with low stage disease. Additional radiologic imaging of the abdomen and a bone marrow biopsy are done to help define the stage of disease. Long-term survival following chemotherapy and radiotherapy increases the risk for development of a second malignancy such as acute myelogenous leukemia, lung cancer, and breast cancer.