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 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 Inherited 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%

CLL Blood Picture

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.

Sezary Cell

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