TAILIEUCHUNG - Chapter 105. Malignancies of Lymphoid Cells (Part 20)

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. The malignant cells appear to have "hairy" projections on light and electron microscopy and show a characteristic staining pattern with tartrate-resistant acid phosphatase. . | Chapter 105. Malignancies of Lymphoid Cells Part 20 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. The malignant cells appear to have hairy projections on light and electron microscopy and show a characteristic staining pattern with tartrate-resistant acid phosphatase. Bone marrow is typically not able to be aspirated and biopsy shows a pattern of fibrosis with diffuse infiltration by the malignant cells. Patients with this disorder are prone to unusual infections including infection by Mycobacterium avium intracellulare and to vasculitic syndromes. Hairy cell leukemia is responsive to chemotherapy with interferon a pentostatin or cladribine with the latter being the usually preferred treatment. Clinical complete remissions with cladribine occur in the majority of patients and long-term disease-free survival is frequent. Splenic marginal zone lymphoma involves infiltration of the splenic white pulp by small monoclonal B cells. This is a rare disorder that can present as leukemia as well as lymphoma. Definitive diagnosis is often made at splenectomy which is also an effective therapy. This is an extremely indolent disorder but when chemotherapy is required the most usual treatment has been chlorambucil. Lymphoplasmacytic lymphoma is the tissue manifestation of Waldenstrom s macroglobulinemia Chap. 106 . This type of lymphoma has been associated with chronic hepatitis C virus infection and an etiologic association has been proposed. Patients typically present with lymphadenopathy splenomegaly bone marrow .

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