Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ CLASSIFICATION ++ The lymphomas of mature T cells and natural killer (NK) cells are shown in Table 67–1. The cutaneous forms are discussed in Chap. 66. ++Table Graphic Jump LocationTABLE 67-1WORLD HEALTH ORGANIZATION CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER CELL NEOPLASMSView Table||Download (.pdf) TABLE 67-1 WORLD HEALTH ORGANIZATION CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER CELL NEOPLASMS Leukemic neoplasms: T-cell prolymphocytic leukemia T-cell large granular lymphocytic leukemia Aggressive NK-cell leukemia Nodal neoplasms: Adult T-cell leukemia/lymphoma Anaplastic large cell lymphoma Peripheral T-cell lymphoma, unspecified Angioimmunoblastic T-cell lymphoma Extranodal neoplasms: Hepatosplenic T-cell lymphoma Nasal NK/T-cell lymphoma Enteropathy-type T-cell lymphoma Subcutaneous panniculitis T-cell lymphoma Cutaneous neoplasms Mycosis fungoides Sézary syndrome Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders CD, cluster of differentiation; NK, natural killer. Source: Williams Hematology, 8th ed, Chap.106, Table 106–1, p. 1609. +++ T-CELL LEUKEMIAS +++ T-Cell Prolymphocytic Leukemia ++ Prolymphocytes (large [T] lymphocytes usually with prominent nucleoli) represent at least 50 percent of lymphocytes in the blood. They often have nuclear convolutions. T-prolymphocytes characteristically express pan-T-cell markers: CD2, CD3, CD5, and CD7. In the majority of cases, prolymphocytes express CD4 but not CD8 (i.e., malignant cell of T-helper cell origin). In occasional morphologically identical cases, CD4 may be weakly expressed or absent and CD8 may be weakly positive. CD52 is highly expressed and can be a target of therapy. Inversion of chromosome 14 is the most common cytogenetic abnormality and is found in 80 percent of patients. The t(14;14) occurs in 10 percent of patients. Associated abnormalities of chromosome 8 (e.g., t(8;8)) are common as well. Several other chromosomes (e.g., 6, 11, 17) may also be abnormal. Represents 20 percent of prolymphocytic leukemias; the remainder have a B-cell phenotype. The male:female ratio is 1.5:1. The tissue (e.g., lymphatic tissue) involvement and blood involvement coexist and are striking in both locations. Most patients have generalized lymphadenopathy and hepatosplenomegaly with marrow replacement and lymphoma cells dominating the white cell count (usually >100 × 109/L). Anemia and thrombocytopenia are nearly always evident at diagnosis. Skin involvement occurs in approximately 20 percent of patients. Serology for the human lymphocytotropic virus-1 is negative. The clinical course is usually rapid. Median survival is less than 1 year. No standard chemotherapeutic treatment program has been developed. For example, cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine (Oncovin), prednisone (CHOP) therapy does not produced long-term remissions. Pentostatin and cladrabine can induce responses in approximately 40 percent of patients; but, complete remissions are infrequent (~10%) and long-term responses (>1 year) are uncommon. Alemtuzamab, a humanized monoclonal antibody against CD52, given three times weekly, has induced good responses in over 50 percent of patients. Allogeneic hematopoietic stem cell transplantation can induce a long-term remission of the disease. Occasional patients may have a chronic course of a few years but then evolve to a rapidly progressive phase. +++ T-Cell Large Granular Lymphocytic ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.