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1610 Part XI: Malignant Lymphoid Diseases Chapter 97: Hodgkin Lymphoma 1611

TABLE 97–2. Staging System for Hodgkin Lymphoma increased levels of 1,25-dihydroxyvitamin D by Hodgkin lymphoma
cells. A variety of other abnormalities have been reported, including
120
Stage hypoglycemia 121,122 resulting from autoantibodies to insulin receptors
I. Involvement of a single lymph node region (I) or a single extra- and hyponatremia resulting from inappropriate secretion of antidiuretic
lymphatic organ or site (I ) hormone. 123
E
II. Involvement of two or more lymph node regions on the same Anemia, granulocytosis, lymphopenia, and low serum albumin con-
side of the diaphragm alone (II) or with involvement of limited, stitute four of seven adverse prognostic factors identified in advanced
contiguous extralymphatic organ or tissue (II ) Hodgkin lymphoma by an international consortium. Similar to the
124
E
III. Involvement of lymph node regions on both sides of the dia- non-Hodgkin lymphomas, serum β -microglobulin levels correlate
2
125
phragm (III), which may include the spleen (III ) or limited, con- with tumor burden and prognosis in Hodgkin lymphoma. Serum lev-
S
tiguous extralymphatic organ or site (III ) or both (III ) els of cytokines including soluble CD30, IL-6, IL-10, and the IL-2 recep-
E ES
IV. Multiple or disseminated foci of involvement of one or more tor, have been reported to correlate with constitutional symptoms and
extralymphatic organs or tissues, with or without associated advanced disease. 126–129 Examination of pleural fluid in Hodgkin lym-
lymph node involvement phoma may reveal transudative, exudative or chylous characteristics.
Modifying Features Because cytology rarely yields diagnostic Hodgkin and Reed-Sternberg
A. Asymptomatic cells, the etiology is most often considered to be one of central lymphatic
B. Drenching night sweats; fever >38°C; loss of more than 10% obstruction. Laboratory abnormalities, including abnormal liver func-
tion tests associated with marked enlargement of porta hepatis nodes
body weight in 6 months and biliary obstruction or intrahepatic cholestasis, may be prominent
C. Involvement of a single, contiguous or proximal extranodal site in rare presentations of Hodgkin lymphoma. Similarly, the nephrotic
130
Bulky disease is defined as a mass >10 cm or a mediastinal mass syndrome, characterized by edema, azotemia, hypoalbuminemia, and
ration >0.33. The mediastinal mass ratio is the ratio of the maximal hyperlipidemia, may rarely be present at the time of diagnosis of Hodg-
width of a mediastinal mass relative to the maximal width of the kin lymphoma. 131
mediastinum, as measured by CT imaging.
DIFFERENTIAL DIAGNOSIS
principal treatment for all but stage IV disease. Recommended staging Clinically enlarged lymph nodes may be associated with a variety
procedures for untreated patients have evolved with changes in therapy. of infectious, inflammatory, autoimmune, and neoplastic disorders.
Exploratory laparotomy and splenectomy, which historically advanced Biopsy of unexplained, persistent, or recurrent adenopathy should be
about one-third of clinical stages I and II patients to pathologic stages reviewed by an experienced hematopathologist. Distinction of cHL
132
III and IV, is no longer performed. CT of the chest, abdomen, and pelvis from primary mediastinal B-cell lymphoma may be difficult based on
and FDG-PET imaging provide sensitive delineation of involved sites, both clinical and histologic features. Gene expression profiling studies
and the use of chemotherapy in all stages of disease has reduced the suggest that these disorders are closely related pathogenetically. 132–135
critical nature of detecting subclinical disease. Marrow involvement Mixed cellularity Hodgkin lymphoma may demonstrate varied cellular
occurs in approximately 12 percent of new patients and is more com- and stromal compositions and must be distinguished from peripheral
mon in patients of older age, advanced stage, less-favorable histology, T-cell lymphoma and T-cell–rich, B-cell lymphoma, which can also be
or those with constitutional symptoms or immunodeficiency. Because difficult to distinguish from NLPHL. Immune markers of Hodgkin
136
the marrow is almost never involved in young, asymptomatic patients and Reed-Sternberg cells, such as CD30, CD20, and CD15, are invalu-
with favorable clinical stage I or II presentations, marrow biopsy may be able for differential diagnosis (see Chap. 96, Figs. 96–36 and 96–41).
omitted in staging such patients. Nonneoplastic conditions that simulate Hodgkin lymphoma include
viral infections, particularly infectious mononucleosis. Depleted
nodes of any histology, including the depleted phase of lymph nodes
LABORATORY FEATURES from HIV-infected patients, may resemble the diffuse fibrosis variant
of lymphocyte-depleted Hodgkin lymphoma. Histologic assessment of
There are no diagnostic laboratory features of cHL. A complete blood extranodal sites depends upon the organ involved and whether there
count may reveal granulocytosis, eosinophilia, lymphocytopenia, throm- is a known diagnosis of cHL. Identification of typical Hodgkin and
bocytosis, or anemia. Anemia is usually the result of “chronic disease,” Reed-Sternberg cells in needle biopsies of liver and marrow biopsies
but rarely may be caused by hemolysis associated with a positive direct are not required because the specimens for evaluation are typically very
antiglobulin (Coombs) test. Thrombocytopenia may occur as a result small.
of marrow involvement, hypersplenism or an immune mechanism.
Immune neutropenia can occur in cHL. Cytopenias are particularly
common in advanced-stage disease and the lymphocyte-depleted sub- THERAPY
type. Elevation of the erythrocyte sedimentation rate (ESR) is most
common in advanced disease and correlates with constitutional symp- HISTORICAL PERSPECTIVE
toms. 115,116 The degree of sedimentation rate elevation correlates with Hodgkin lymphoma first became a curable neoplasm through the sys-
prognosis, particularly in limited-stage disease. Although nonspe- tematic study of the spread of the disease and the use of higher dose,
117
cific, it may be a useful harbinger of recurrent disease if serially moni- extended field radiotherapy delivered with supervoltage techniques.
137
tored. Serum lactate dehydrogenase levels are elevated in 35 percent of When used alone, radiotherapy doses to involved fields usually ranged
patients at diagnosis. 118,119 The alkaline phosphatase may be elevated in from 3500 to 4400 cGy with prophylactic doses of 3000 to 3500 cGy
cHL, nonspecifically in limited disease, or in association with involve- to uninvolved tissues. The mantle, paraaortic region, and pelvis con-
ment of liver, bone, or marrow in advanced disease. Hypercalcemia is stitute the classic radiotherapy regions. With increased recognition of
unusual in cHL and when present may be secondary to synthesis of late effects, radiation therapy has been modified to reduce field size to

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