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Lymphoblastic Lymphoma; ALL (null; non-B, non-T or common ALL) (Table 26–14) is the related leukemia.
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Relatively homogeneous population of lymphoblasts with numerous mitoses.
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Most often presents as leukemia; less often presents as a lymphoblastic lymphoma. Common ALL has best prognosis; then null; T cell ALL least favorable.
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Neoplastic cells lack the usual markers (hence non-B, non-T, or null cell). Some cases express common ALL antigen (CALLA). Show Ig gene rearrangement.
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B cell
|
 |
B, follicular center cell (FCC), small noncleaved, and Burkitt's lymphoma (Figure 29–2). ALL B cell type is related leukemia, equivalent to B lymphoblastic lymphoma (Figure 29–1).
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Cells resemble small transformed lymphocytes or lymphoblasts. Nuclei round but variable in size (by definition do not exceed size of histiocyte nucleus). Nuclear chromatin finely dispersed. One to three small nucleoli. Moderate amount of basophilic cytoplasm. Starry sky reactive histiocytes common. Generally diffuse, obilterating lymph node architecture (Figure 29–2).
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Abdominal presentation characteristic in U.S. cases, often in children. May become manifest as leukemia (then equals L3 subtype of ALL). Rapidly growing tumor. Includes classic Burkitt's lymphoma of Africa that typically presents in the jaw and is associated with EBV.
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Monoclonal surface Ig:IgM, CD20, and other B cell markers t (8:14),
myc;
(See Chapter 18: Neoplasia: II. Mechanisms & Causes of Neoplasia.)
|
B cell
|
 |
Small lymphocytic lymphoma/leukemia; B cell; CLL B-cell type is the related leukemia.
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Diffuse population of small lymphocytes. Uniform, round nuclei with basophilic compact chromatin and inconspicuous nucleoli. Narrow rim of pale cytoplasm. Large transformed lymphocytes and mitoses rare.
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Typically prolonged benign course in elderly. Merges with spectrum of CLL. Rarely transforms to large cell (immunoblastic sarcoma) with rapid turnover (Richter's syndrome).
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Most show monoclonal surface Ig pattern, usually IgM or IgD. CD20 and other B cell markers.
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B cell
|
 |
Intermediate and mantle zone lymphoma.
3
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Related to FCC lymphomas. Follicular or diffuse arrangement of intermediate-sized cells with irregular nuclei. Thought to be derived from B cells of the mantle zone that surrounds the follicle.
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Elderly patients, often with wide-spread disease. Low-grade.
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Monoclonal Ig. CD20 and other B cell markers. Paradoxically, often CD5-positive. Most show t (11:14)
bcl-1
rearrangement.
|
B cell
|
 |
Monocytoid B cell lymphoma.
3
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Normal B cell counterpart not identified. Diffuse or mantle zone pattern. Larger than normal B cell, with more cytoplasm. May occupy sinuses of node.
|
Elderly patients, 80% with localized disease; 15% extranodal. Low-grade.
|
Monoclonal Ig. CD20 and other B cell markers. May show CD11 (monocytic).
|
B cell
|
 |
FCC; small cleaved lymphoma (Figure 29–3).
|
Wide range of cell sizes, but small cells predominate. Nuclei have basophilic compact chromatin, and may show deep cleavage planes. Nucleoli are inconspicuous, andcytoplasm is indistinct or scanty. Mitoses rare. 75% are follicular, remainder diffuse (Figure 29–3).
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Asymptomatic presentation typical Low cell turnover rate. Marrow involved at presentation in over 70% of cases. Paradox of widespread distribution but prolonged median survival. Occasionally leukemic, resembling CLL, but cleaved nuclear morphology.
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Most show monoclonal surface Ig pattern; usually IgM or IgG. CD75, CD20, and other B cell markers. Most show t (14:18)
bcl-2
rearrangement.
|
B cell
|
 |
FCC, large cleaved lymphoma.
|
Nuclei larger than nuclei of reactive histiocytes. Prominent nuclear irregularity with exaggerated cleavage planes. Cytoplasm moderate. Small cleaved and non-cleaved cells generally present in small numbers.
|
Commonly present in mesenteric, retroperitoneal, or inguinal nodes. Also often extranodal.
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Typically monoclonal surfact Ig:IgM, IgG, IgA. CD20, CD75, and other B cell markers; t (14:18),
bcl-2.
|
B cell
|
 |
FCC; large noncleaved lymphoma.
|
Similar to small noncleaved (or lymphoblastic), but cells and nuclei larger. Mitoses numerous. Follicular in approximately 10% of cases.
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Aggressive neoplasm with high turnover rate. Rapid dissemination.
|
Typically shows monoclonal surface Ig:Igm, or IgG. CD20, CD75, and other B cell markers t (14:18)
bcl-2
.
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B cell
|
 |
Immunoblastic sarcoma; B cell (Figure 29–4).
|
Immunoblasts resemble large non-cleaved FCC, but more deeply staining basophilic cytoplasm. Often plasmacytoid features. Nucleoli often central and prominent; nucleus appears vesicular owing to margination of chromatin (Figure 29–4).
|
Abnormal immune states (immunosuppression, alpha chain disease, systemic lupus erythematosus, drug hypersensitivity, immunoblastic lymphadenopathy) frequently precede development of lymphoma. Rapidly progressive neoplasm.
|
Majority monoclonal for surface Ig or cytoplasmic Ig:IgG, IgA, or IgM. CD20 and other B cell markers.
|
B cell
|
 |
Plasmacytoid lymphocytic lymphoma.
|
Similar to small lymphocytic lymphoma but has abnormal plasmacytoid cell component of variable prominence. These cells possess cytoplasm resembling plasma cell, but nucleus more like lymphocyte. Some cells have PAS-positive intranuclear structures (Dutcher bodies).
|
Monoclonal serum spike present. High level of IgM in serum may give hyperviscosity syndrome (Waldenström's macroglobulinemia).
|
Usually monoclonal IgM, at times IgG. Surface Ig type same as serum spike. CD20 and other B cell markers.
|
B cell
|
 |
Hairy cell leukemia (leukemic reticuloendotheliosis).
|
In tissue sections, cells appear medium-sized with abundant pale cytoplasm and round to oval nuclei.
|
Manifestations of pancytopenia and splenomegaly. Benign course, particularly following splenectomy. Cytotoxic therapy may hasten demise.
|
Tartrate-resistant acid phosphatase in cytoplasm. Monoclonal surface Ig:Ig synthesis and Ig gene rearrangement reported. CD20 and other B cell markers.
|
B cell
|
 |
Lymphoblastic (convoluted T cell lymphoma); ALL T cell type is related leukemia.
|
Diffuse proliferation of primitive cells. Nuclear chromatin finely stippled, and nucleolus inconspicuous. Mitoses numerous. Some cells have convoluted or complexly folded nucleus.
|
Primary lymphoma-leukemia of children and young adults but may occur at any age. Male predominance. Presentation usually in lymph nodes or mediastinum. Response to therapy poor. If present in blood or marrow, termed T ALL. Prognosis worse than that of non-B, non-T ALL.
|
Diagnostic cells mark with anti-T cell antibodies.
|
T cell
|
 |
Small lymphocytic lymphoma, T cells; CLL T cell type is related leukemia.
|
Cells resemble small lymphocytes. Nuclei with compact chromatin. Rim of pale cytoplasm. Nuclei occasionally irregular in form.
|
May be leukemic resembling B cell CLL. Variant is common in Japan and is caused by HTLV-I, a retrovirus probably transmitted in milk. The latent period is 40 years or more.
|
Diagnostic cells mark with anti-T cell antibodies. Often helper phenotype.
|
T cell
|
 |
Immunoblastic sarcoma; T cell.
|
Admixture of small and transformed lymphocytes. Latter predominate. In sections, they have pale, water-clear cytoplasm.
|
Less common than B cell type from which it may be distinguished immunologically. Prognosis poor.
|
Lymphoma cells mark with anti-T cell antibodies.
|
T cell
|
 |
Mycosis fungoides and Sézary cell (Figure 29–5).
|
Cells have compact chromatin and few mitoses resembling normal small lymphocytes except that they are larger and the nuclei often show complex folding (Figure 29–5).
|
Mycosis fungoides and Sézary syndrome closely related. Affinity of cells for skin consistent with affinity of T cell for skin. Mycosis fungoides may progress to involvement of nodes, spleen, and blood. Sézary syndrome involves blood from the outset.
|
Cells mark with anti-T cell antibodies. Frequently helper phenotype.
|
T cell
|
 |
Lymphoepithelioid cell lymphoma.
|
Neoplastic T lymphocytes are admixed with reactive histiocytes. Sometimes confused with Hodgkin's disease.
|
Relatively rare. Intermediate grade of malignancy.
|
Lymphoid component marks as T cells.
|
T cell
|
 |
True histiocytic lymphoma.
4
|
Large cells with extensive cytoplasm. Diffuse or sinusoidal pattern. May show phagocytosis.
|
Rare—less than 5% of all lymphomas. Aggressive.
|
Usually CD68-positive. May lack usual monocyte markers. No B or T cell makers.
|
Histiocytic cell
|