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Chapter 105. Malignancies of Lymphoid Cells (Part 11)

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. Patients typically present with signs of bone marrow failure such as pallor, fatigue, bleeding, fever, and infection related to peripheral blood cytopenias. Peripheral blood counts regularly show anemia and thrombocytopenia but might show leukopenia, a normal leukocyte. | Chapter 105. Malignancies of Lymphoid Cells Part 11 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. Patients typically present with signs of bone marrow failure such as pallor fatigue bleeding fever and infection related to peripheral blood cytopenias. Peripheral blood counts regularly show anemia and thrombocytopenia but might show leukopenia a normal leukocyte count or leukocytosis based largely on the number of circulating malignant cells Fig. 105-5 . Extramedullary sites of disease are frequently involved in patients who present with leukemia including lymphadenopathy hepato- or splenomegaly CNS disease testicular enlargement and or cutaneous infiltration. Figure 105-5 Hire Fauci Mipi Du er u ld t 51 Longa PL J rr icm JI. Lotulto Ji HirrisM r Prifcopfas fttiwi PtojKfnt 17th Edibani http vwM oc -isrTi d cm .ppm Copyright Th omponlllj Ina All right riarir-.nd. Acute lymphoblastic leukemia. The cells are heterogeneous in size have round or convoluted nuclei high nuclear cytoplasmic ratio and absence of cytoplasmic granules. The diagnosis is usually made by bone marrow biopsy which shows infiltration by malignant lymphoblasts. Demonstration of a pre-B cell immunophenotype Fig. 105-2 and often characteristic cytogenetic abnormalities Table 105-6 confirm the diagnosis. An adverse prognosis in patients with precursor B cell ALL is predicted by a very high white cell count the presence of symptomatic CNS disease and unfavorable cytogenetic abnormalities. For example t 9 22 frequently found in adults with B cell ALL has been associated with a very poor outlook. The bcr abl kinase .