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MLAB 1415- Hematology Keri Brophy-Martinez. Chapter 26: Lymphoid Malignancies: Chronic Lymphoid Leukemias Lymphomas Plasma Cell Neoplasms. Introduction. Divided into four categories based on maturity of neoplastic cells and distribution of disease Acute lymphoblastic leukemia
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MLAB 1415- HematologyKeri Brophy-Martinez Chapter 26: Lymphoid Malignancies: Chronic Lymphoid Leukemias Lymphomas Plasma Cell Neoplasms
Introduction • Divided into four categories based on maturity of neoplastic cells and distribution of disease • Acute lymphoblastic leukemia • Proliferation of blasts belonging to lymphoid lineage • Chronic leukemic lymphoid malignancies • Mature lymphocytes, insidious onset, indolent course • Malignant lymphoma • Tumor masses involving lymphoid organs • Plasma cell neoplasms • Involvement of immunoglobulin-secreting cells
Pathogenesis • Acquired genetic factors • Proto-oncogenes • Tumor suppressor genes • Inherited genetic factors • Wiskott Aldrich • Ataxia telangiectasia • Environmental factors • EBV infection • Helicobacter pylori
Chronic Lymphocytic Leukemia • General requirements for diagnosis • Peripheral blood (>5000 ALC) and bone marrow lymphocytosis (>30%) • Lymphocytes are small to slightly larger than normal with mature appearance • Nucleus is round, with block-type chromatin • Cytoplasm scarce • Smudge cells (bare nuclei) are common • Occur due to the cell’s fragility in making a smear • Prolymphocyte < 10%
Chronic Lymphocytic Leukemia • Clinical features • Occurs in persons >50 years old • Men are affected more than women 2:1 • Chronic fatigue, infection • Result of bone marrow replacement of normal cells with lymphocytes. • Skin and organ infiltration and enlargement • Median survival is 4-5 years, with 30% of patients surviving 10 years
Chronic Lymphocytic Leukemia • Treatment • Usually treatment is not required until lymphocytosis causes other cells to be crowded out resulting in infections. • Treatment depends on the stage at which the disease is diagnosed and is usually for the symptoms, not the disease. • Radiation of localized infiltration • Chemotherapy given according to stage of disease • IV gamma globulin for prevention of infection • Bone marrow transplant done on aggressive cases
Hairy cell leukemia (HCL) • Presents in middle age • Affects males7:1 over females • Pancytopenia common • Increases opportunity for infections • Bone marrow aspirate can result in a “dry tap” due to marrow fibrosis. • Hairy cell leukemia can be treated with a one-time chemotherapy regimen with a good prognosis of long-term survival.
Hairy cell TRAP stain
T cell Neoplasms • Large Granular Lymphocyte Leukemia • Sezary’s Syndrome
Large Granular Lymphocyte Leukemia (LGLL) • Moderate lymphocytosis • Abundant pale blue cytoplasm • Azurophilic granules • Clumped chromatin in nuclei • Anemia • Neutropenia • Thrombocytopenia
Sezary’s syndrome • Leukemic phase of the most common cutaneous T-cell lymphoma, mycosis fungoides. • Diagnosis is confirmed by skin biopsy and presence of Sézary cells.
References • McKenzie, S. B., & Williams, J. L. (2010). Clinical Laboratory Hematology . Upper Saddle River: Pearson Education, Inc. • http://www.med-ed.virginia.edu/courses/path/innes/wcd/lympleuk.cfm