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

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Stage I

·   Involvement of single lymph node region or

·   Involvement of single extralymphatic site (stage IE)

Stage II

·   Involvement of 2 or more lymph node regions on same side of diaphragm

·   May include localized extralymphatic involvement on same side of diaphragm (stage IIE)

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Stage III

·   Involvement of lymph node regions on both sides of diaphragm

·   May include involvement of spleen (stage IIIS) or localized extranodal disease (stage IIIE)

·   Hodgkin's disease Stage III1: disease limited to upper abdomen — spleen, splenic hilar, celiac, or portohepatic nodes

·   Hodgkin's disease Stage III2: disease limited to lower abdomen — periaortic, pelvic, or inguinal nodes

Stage IV

·   Diffuse extralymphatic disease (for example, in liver, bone marrow, lung, skin)