Atlas of pathophysiology, 2 Edition

Part II - Disorders

Hematologic Disorders

Hodgkin's disease

Hodgkin's disease is a neoplastic disease characterized by painless, progressive enlargement of lymph nodes, spleen, and other lymphoid tissue resulting from proliferation of lymphocytes, histiocytes, eosinophils, and Reed-Sternberg giant cells. Untreated, Hodgkin's disease follows a variable but relentlessly progressive and, ultimately, fatal course. With appropriate treatment, more than 80% of patients with stage I or II disease survive at least 10 years.

Causes

Unknown; however, viral etiology is suspected (Epstein-Barr virus is a leading candidate)

Age Alert

Hodgkin's disease is most common in young adults, and more common in males than in females. Incidence peaks in two age-groups: ages 15 to 38 and after age 50—except in Japan, where it occurs exclusively among people over age 50.

Pathophysiology

Hodgkin's disease is characterized by proliferation of a tumor in which only a small proportion of the cells are malignant and most are normal lymphocytes. The characteristic malignant cells—called Reed-Sternberg cells—are most likely multinucleated, giant-cell mutations of the T-lymphocyte. Infiltration of the nodes with eosinophils and plasma cells is associated with lymph node necrosis and fibrosis.

Signs and symptoms

· Painless swelling in one of lymph nodes (usually the cervical region) with a history of upper respiratory infection

· Persistent fever

· Night sweats

· Fatigue

· Weight loss

· Malaise

· Pruritus

· Extremity pain

· Nerve irritation

· Absence of pulse due to rapid enlargement of lymph nodes

· Pericardial friction rub

· Pericardial effusion

· Neck vein engorgement

· Enlargement of retroperitoneal nodes, spleen, and liver

Diagnostic test results

· Lymph node biopsy confirms presence of Reed-Sternberg cells, nodular fibrosis, and necrosis.

· Bone marrow, liver, mediastinal, lymph node, and spleen biopsies reveal histologic presence of cells.

· Chest X-ray, abdominal computed tomography scan, lung scan, bone scan, and lymphangiography detect lymph and organ involvement.

· Hematologic tests show:

§ mild to severe normocytic anemia

§ normochromic anemia

§ elevated, normal, or reduced white blood cell count

§ differential with any combination of neutrophilia, lymphocytopenia, monocytosis, and eosinophilia.

· Elevated serum alkaline phosphatase indicates bone or liver involvement.

Treatment

· Chemotherapy, radiation, or both, appropriate to stage of the disease—based on histologic interpretation and clinical staging

· Concomitant antiemetics, sedatives, or antidiarrheals to combat adverse GI effects

· Autologous bone marrow transplant

· Autologous peripheral blood sternal transfusions

· Immunotherapy

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ANN ARBOR STAGING SYSTEM FOR HODGKIN'S DISEASE