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White Blood Cell Disorders. Jonathan Ben-Ezra, M.D. Professor of Pathology MCV Campus of VCU. White Blood Cells. Infection fighting cells of blood Excess causes tissue infiltration/leukostasis 4.4- 5.9 x 10 6 /L (M) 3.8 -5.2 x 10 6 /L (F) Several Types of Cells Granulocytes Lymphocytes

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White blood cell disorders l.jpg

White Blood Cell Disorders

Jonathan Ben-Ezra, M.D.

Professor of Pathology

MCV Campus of VCU


White blood cells l.jpg
White Blood Cells

  • Infection fighting cells of blood

  • Excess causes tissue infiltration/leukostasis

  • 4.4- 5.9 x 106/L (M)

  • 3.8 -5.2 x 106/L (F)

  • Several Types of Cells

    • Granulocytes

    • Lymphocytes

    • Monocytes


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Granulocytes

  • Produced in the Bone Marrow

  • Neutrophils

    • Life span of 8 hours

    • Bacteriocidal

  • Eosinophils

  • Basophils

  • Approximately 2/3 of WBCs in Blood


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Granulocytes

  • Increased in Bacterial Infection

    • May see early forms (left shift)

  • Increased in Myeloid Leukemia


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Lymphocytes

  • B cells

    • Antibody production

  • T cells

    • Fight viral infection

  • Morphologically can’t distinguish between the two

  • Approximately 30% of WBCs in PB

  • Increased in viral infections and lymphoid leukemia


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Monocytes

  • Become tissue macrophages

  • Involved in phagocytosis

  • Approximately 15% of WBCs in PB



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Leukemia

  • Neoplastic Proliferations of WBCs in Blood and Bone Marrow

    • Anemia, infection, bleeding

  • Acute Leukemias

    • Blast (precursor) cells

    • Rapidly fatal if not treated

  • Chronic Leukemias

    • More mature cells

    • Longer life expectancy



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Acute Lymphoblastic Leukemia

  • Proliferation of lymphoblasts

    • anemia, thrombocytopenia, increased WBC

    • lymphadenopathy/splenomegaly

  • B- or T-cell

    • flow cytometry

  • TdT+

  • Most common leukemia of childhood



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Acute Myelogenous Leukemia

  • Proliferation of myeloblasts

    • anemia, thrombocytopenia, increased WBC

  • Myeloid, monocytic, RBC, or megakaryocytic

    • flow cytometry

    • myeloperoxidase +, TdT-

  • Auer rod

  • Over age of 20



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Chronic Myelogenous Leukemia

  • 1 of myeloproliferative diseases (PV, ET)

  • Proliferation of more mature granulocytes

    • normal to increased platelet count

    • anemia

  • Splenomegaly

  • t(9;22) (bcr-abl) (Philadelphia chromosome)


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Chronic Myelogenous Leukemia

  • Long chronic phase

  • Blast crisis

  • Hydroxyurea, interferons

  • Bone marrow transplantation



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Myelofibrosis

  • Marrow becomes fibrotic

    • extramedullary hematopoiesis

    • dry tap

  • Teardrop RBC

  • Myeloproliferative, toxin, infection


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Chronic Lymphocytic Leukemia

  • Proliferation of small mature B-lymphocytes

    • flow cytometry (monoclonal Kappa or lambda)

  • Lymphadenopathy

    • relationship to small lymphocytic lymphoma

  • May have Ab production and AIHA

  • 50% 5-year survival



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Multiple Myeloma

  • Neoplasm of plasma cells

    • monoclonal protein in serum (SPEP)

    • Proteinuria (Bence-Jones) (UPEP)

  • Lytic lesions in bones

    • fractures

  • Anemia, increased globulin

    • Rouleaux formation

  • Renal failure/ amyloidosis



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Infectious Mononucleosis

  • Acute infection with EBV

  • Adolescent/Young Adult

  • Fever, sore throat, splenomegaly, fatigue

  • Heterophile antibodies

  • Usually self limited


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Lymphomas

  • Nodal based malignant proliferations of lymphoid cells

  • Hodgkin’s disease

  • Non-Hodgkin’s lymphoma

    • follicular vs. diffuse

    • small vs. large cell

  • Staging



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Low grade NHL

  • Small lymphocytic lymphoma

    • nodal counterpart for CLL

  • Follicular lymphomas

    • B-cell

    • t(14;18)

    • failure of apoptosis





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High Grade NHL

  • May be nodal or extranodal

  • May be T- or B-cell

  • Adults or pediatric

  • Large cell lymphoma

  • Burkitt’s lymphoma

    • t(8;14)

    • related to EBV

  • Lymphoblastic lymphoma





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Extranodal Marginal Zone B-cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT)

  • Proliferation of mucosal associated lymphoid tissue

  • Small lymphoid cells, with/out abundant cytoplasm

  • May transform to large cell lymphoma

  • Median survival of 8 years


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Extranodal Marginal Zone B-cell Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT)


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Hodgkin Lymphoma Mucosa-Associated Lymphoid Tissue (MALT

  • Bimodal age distribution

  • Related to EBV

  • Reed Sternberg cell

    • other background inflammatory cells

  • Radiation, chemotherapy, or BMT


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Hodgkin Lymphoma Mucosa-Associated Lymphoid Tissue (MALT

  • Nodular Lymphocyte Predominant

  • Nodular Sclerosis

  • Mixed Cellularity

  • Lymphocyte Depleted


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Hodgkin Lymphoma Mucosa-Associated Lymphoid Tissue (MALT