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1636 Part XI: Malignant Lymphoid Diseases Chapter 98: Diffuse Large B-Cell Lymphoma and Related Diseases 1637

Therapy and Prognosis of adenopathy, and the liver, spleen, bone and gastrointestinal tract
When treated with CHOP-like regimens, most series suggest that the out- being the most common sites of extranodal involvement. 159,160
come for these patients is similar to patients with typical DLBCL, 149,151–154
with CR rates of approximately 60 percent, and 3-year and 5-year OS rates Laboratory Features
of 50 to 64 percent and 45 to 58 percent, respectively. Two case-control The immunoblastic cells of anaplastic DLBCL typically have a large cen-
analyses have been performed comparing T-cell–histiocyte-rich large tral nucleolus and may exhibit maturation to plasmablastic cells. The
B-cell lymphoma and DLBCL with no differences in OS observed. 149,150 lymphoma cells stain for the ALK protein by immunohistocytochem-
Based on these data, this subtype of large B-cell lymphoma should be istry, usually with a granular cytoplasmic appearance though nuclear
treated in the same fashion as traditional DLBCL. Six cycles of R-CHOP staining may also occur. These cells are usually negative for CD3, CD20,
for advanced disease is a reasonable initial approach to therapy. CD30, and CD79a. MUC1 mucin, a high-molecular-weight transmem-
brane glycoprotein, also known as epithelial membrane antigen and
PRIMARY CUTANEOUS DIFFUSE LARGE B-CELL CD138 are usually strongly expressed by these cells. Monoclonal (light-
chain restricted) IgA or IgG is generally present in the cytoplasm of the
LYMPHOMA, LEG TYPE malignant cells. Occasional cases possess a t(2;17)(p23;q23) transloca-
Definition tion that results in a clathrin-ALK fusion protein.
This lymphoma is a primary cutaneous lymphoma composed solely of large
transformed B cells that exhibits a predilection for the skin of the leg. 2 Therapy and Prognosis
The clinical course of ALK-positive large B-cell lymphoma is aggres-
Epidemiology sive with a median survival time of 24 months. Because the tumors
Primary cutaneous DLBCLs of the leg type constitute approximately are usually negative for CD20, the utility of rituximab is dubious for
4 percent of all primary cutaneous B-cell lymphomas. 2,155 The median this entity. Anthracycline-based chemotherapy is often inadequate and
age at the time of presentation is 60 to 70 years. more intensive therapies should be considered. 159,160
Clinical Features HUMAN IMMUNODEFICIENCY RELATED
These lymphomatous tumors affect the skin of the legs in most cases,
but approximately 10 percent arise at other sites. 156–158 Multiple tumors, DIFFUSE LARGE B-CELL LYMPHOMA VARIANTS
sometimes ulcerating, are associated with poorer prognosis. There are Primary effusion lymphoma, plasmablastic lymphoma, and large B-cell
frequent relapses and extracutaneous dissemination may occur. lymphoma arising in human herpesvirus-8–associated multicentric
Castleman disease usually develop in the setting of acquired immuno-
Laboratory Features deficiency induced by HIV and are discussed in Chap. 81.
The malignant B cells in this entity usually express CD20, BCL2, and
FOX-P1. FISH of the lymphoma cells often detects translocations
involving MYC, BCL6, or IGH genes. Amplification of the BCL2 gene REFERENCES
is usually responsible for the high frequency of BCL2 overexpression.
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10. Ye BH, Chaganti S, Chang CC, et al: Chromosomal translocations cause deregulated
Definition BCL6 expression by promoter substitution in B cell lymphoma. EMBO J 14:24, 1995.
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nal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell
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