Malignancies of lymphoid cells

Non-Hodgkin lymphoma is a malignant disease of lymphoid tissue (lymph nodes, lymphoid organs such as nasopharynx, tonsils, digestive tract, spleen, thymus, bone marrow, etc) which originates from many types of lymphocytes such as progenitor B cell, progenitor T cell, mature B cell or mature T cell. To evaluate the characteristics of children with non-Hodgkin lymphomas at Children’s Hospital 1.

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The diagnosis of Hodgkin's disease is established by review of an adequate biopsy specimen by an expert hematopathologist. In the United States, most patients have nodular sclerosing Hodgkin's disease, with a minority of patients having mixed-cellularity Hodgkin's disease. Lymphocyte-predominant and lymphocyte-depleted Hodgkin's disease are rare. Mixed-cellularity Hodgkin's disease or lymphocyte-depletion Hodgkin's disease are seen more frequently in patients infected by HIV (Fig. 105-11).

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A number of other late side effects from the treatment of Hodgkin's disease are well known. Patients who receive thoracic radiotherapy are at very high risk for the eventual development of hypothyroidism and should be observed for this complication; intermittent measurement of thyrotropin should be made to identify the condition before it becomes symptomatic. Lhermitte's syndrome occurs in ~15% of patients who receive thoracic radiotherapy. This syndrome is manifested by an "electric shock" sensation into the lower extremities on flexion of the neck.

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Rare patients with localized early stage mycosis fungoides can be cured with radiotherapy, often total-skin electron beam irradiation. More advanced disease has been treated with topical glucocorticoids, topical nitrogen mustard, phototherapy, psoralen with ultraviolet A (PUVA), electron beam radiation, interferon, antibodies, fusion toxins, and systemic cytotoxic therapy. Unfortunately, these treatments are palliative. Adult T Cell Lymphoma/Leukemia Adult T cell lymphoma/leukemia is one manifestation of infection by the HTLV-I retrovirus.

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Anaplastic Large T/Null Cell Lymphoma: Treatment Treatment regimens appropriate for other aggressive lymphomas, such as diffuse large B cell lymphoma, should be utilized in patients with anaplastic large T/null cell lymphoma, with the exception that the B cell–specific antibody, rituximab, is omitted. Surprisingly, given the anaplastic appearance, this disorder has the best survival rate of any aggressive lymphoma. The 5-year survival is 75%.

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Other B Cell Lymphoid Malignancies B cell prolymphocytic leukemia involves blood and marrow infiltration by large lymphocytes with prominent nucleoli. Patients typically have a high white cell count, splenomegaly, and minimal lymphadenopathy. The chances for a complete response to therapy are poor. Hairy cell leukemia is a rare disease that presents predominantly in older males. Typical presentation involves pancytopenia, although occasional patients will have a leukemic presentation. Splenomegaly is usual.

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Because a number of patients with diffuse large B cell lymphoma are either initially refractory to therapy or relapse after apparently effective chemotherapy, 30–40% of patients will be candidates for salvage treatment at some point. Alternative combination chemotherapy regimens can induce complete remission in as many as 50% of these patients, but long-term disease-free survival is seen in ≤10%.

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Evaluation of an adequate biopsy by an expert hematopathologist is sufficient to make a diagnosis of follicular lymphoma. The tumor is composed of small cleaved and large cells in varying proportions organized in a follicular pattern of growth (Fig. 105-7). Confirmation of B cell immunophenotype and the existence of the t(14;18) and abnormal expression of BCL-2 protein are confirmatory. The major differential diagnosis is between lymphoma and reactive follicular hyperplasia. The coexistence of diffuse large B cell lymphoma must be considered.

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Patients with follicular lymphoma have a high rate of histologic transformation to diffuse large B cell lymphoma (5–7% per year). This is recognized ~40% of the time during the course of the illness by repeat biopsy and is present in almost all patients at autopsy. This transformation is usually heralded by rapid growth of lymph nodes—often localized—and the development of systemic symptoms such as fevers, sweats, and weight loss.

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B Cell Chronic Lymphoid Leukemia/Small Lymphocytic Lymphoma: Treatment Patients whose presentation is typical B cell CLL with no manifestations of the disease other than bone marrow involvement and lymphocytosis (i.e., Rai stage O and Binet stage A; Table 105-7) can be followed without specific therapy for their malignancy. These patients have a median survival 10 years, and some will never require therapy for this disorder.

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Evaluation of patients with MALT lymphoma follows the pattern (Table 105-11) for staging a patient with non-Hodgkin's lymphoma. In particular, patients with gastric lymphoma need to have studies performed to document the presence or absence of H. pylori infection. Endoscopic studies including ultrasound can help define the extent of gastric involvement. Most patients with MALT lymphoma have a good prognosis, with a 5-year survival of ~75%. In patients with a low IPI score, the 5-year survival is ~90%, while it drops to ~40% in patients with a high IPI score.

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Table 105-10 Clinical Characteristics of Patients with Common Types of Non-Hodgkin's Lymphomas (NHL) Dis ease edian Age, years M Fre quency in tage S B one B Gastro intestinal Survi Children Male I/II vs Sympt Marro Tract III/IV, oms, % % w Involvement, ving 5 years Involve % ment, % B 6 Rar 9 3 7 3 cell chronic lymphocyt ic leukemia/ small lymphocyt ic lymphoma 5 e 3 vs 91 3 2 1 Ma ntle cell 3 6 e Rar 4 2 0 vs 80 8 2 4 6 9 7 lymphoma Ext ranodal marginal zone cell lymphoma of MALT B 0 6 e Rar 8 6 7 vs 33 9 1 4 1 50 4 type Fol licular lymphoma 9 ...

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Chronic lymphocytic leukemia. The peripheral white blood cell count is high due to increased numbers of small, well-differentiated, normal-appearing lymphocytes. The leukemia lymphocytes are fragile, and substantial numbers of broken, smudged cells are usually also present on the blood smear. If the primary presentation is lymphadenopathy and a lymph node biopsy is performed, pathologists usually have little difficulty in making the diagnosis of small lymphocytic lymphoma based on morphologic findings and immunophenotype.

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Table 105-9 International Prognostic Index for NHL Five clinical risk factors: Age ≥60 years Serum lactate dehydrogenase levels elevated Performance status ≥2 (ECOG) or ≤70 (Karnofsky) Ann Arbor stage III or IV 1 site of extranodal involvement Patients are assigned a number for each risk factor they have Patients are grouped differently based upon the type of lymphoma For diffuse large B cell lymphoma: 0, 1 factor = low risk: 73% 35% of cases; 5-year survival, 2 factors = low-intermediate risk: 51% 27% of cases; 5-year survival, 3 factors = high-intermediate risk: 43% 22% ...

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Clinical Features, Treatment, and Prognosis of Specific Lymphoid Malignancies Precursor Cell B Cell Neoplasms Precursor B Cell Lymphoblastic Leukemia/Lymphoma The most common cancer in childhood is B cell ALL. Although this disorder can also present as a lymphoma in either adults or children, presentation as lymphoma is rare. The malignant cells in patients with precursor B cell lymphoblastic leukemia are most commonly of pre-B cell origin.

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Two other features may be used to assess prognosis in B cell CLL, but neither has yet been incorporated into a staging classification. At least two subsets of CLL have been identified based on the cytoplasmic expression of ZAP-70; expression of this protein, which is usually expressed in T cells, identifies a subgroup with poorer prognosis. A less powerful subsetting tool is CD38 expression. CD38+ tumors tend to have a poorer prognosis than CD38– tumors. The initial evaluation of a patient with Hodgkin's disease or non-Hodgkin's lymphoma is similar.

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Table 105-6 presents the best documented translocations and associated oncogenes for various subtypes of lymphoid malignancies. In some cases, such as the association of the t(14;18) in follicular lymphoma, the t(2;5) in anaplastic large T/null cell lymphoma, the t(8;14) in Burkitt's lymphoma, and the t(11;14) in mantle cell lymphoma, the great majority of tumors in patients with these diagnoses display these abnormalities. In other types of lymphoma where a minority of the patients have tumors expressing specific genetic abnormalities, the defects may have prognostic significance.

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Chapter 105. Malignancies of Lymphoid Cells (Part 8) Approach to the Patient: Lymphoid Cell Malignancies Regardless of the type of lymphoid malignancy, the initial evaluation of the patient should include performance of a careful history and physical examination. These will help confirm the diagnosis, identify those manifestations of the disease that might require prompt attention, and aid in the selection of further studies to optimally characterize the patient's status to allow the best choice of therapy.

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General Aspects of Lymphoid Malignancies Etiology and Epidemiology The relative frequency of the various lymphoid malignancies is shown in Fig. 105-1. Chronic lymphoid leukemia (CLL) is the most prevalent form of leukemia in western countries. It occurs most frequently in older adults and is exceedingly rare in children. In 2007, 15,340 new cases were diagnosed in the United States, but because of the prolonged survival associated with this disorder, the total prevalence is many times higher. CLL is more common in men than in women and more common in whites than in blacks.

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The incidence of non-Hodgkin's lymphomas and the patterns of expression of the various subtypes differ geographically. T cell lymphomas are more common in Asia than in western countries, while certain subtypes of B cell lymphomas such as follicular lymphoma are more common in western countries. A specific subtype of non-Hodgkin's lymphoma known as the angiocentric nasal T/natural killer (NK) cell lymphoma has a striking geographic occurrence, being most frequent in Southern Asia and parts of Latin America.

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