Malignant Leukocyte Disorders

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Author:
ncrook
ID:
210581
Filename:
Malignant Leukocyte Disorders
Updated:
2013-05-01 22:28:11
Tags:
Other Lymphoid Malignancies Acute Myloproliferative Disorders Chronic Myelodysplastic Syndromes
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Description:
Other Lymphoid Malignancies, Acute Myloproliferative Disorders, Chronic Myloproliferative Disorders, Myelodysplastic Syndromes
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  1. What condition:
    Causes B cell production of excessive IgG (most common) or IgA, with decreased production of the other immunoglobulins
    Serum protein electrophoresis by an "M" spike in the gamma globulin region
    Bone marrow: Plasma cells >30%
    Marked rouleaux
    Increased ESR
    Plasma cells and lymphs on blood smear
    Bence Jones proteins (free light chains - kappa or lambda) found in urine
    Causes: Multiple skeletal system tumors of plasma cells which cause lytic bone lesions and hypercalcemia, Increased blood viscosity due to excessive IgG or IgA, Prolonged bleeding, Kidney damage due to Bence Jones protein
    Monoclonal gammopathy in Multiple Myeloma
  2. What condition:
    Causes B cell production of excessive IgM (macroglobulin) and decreased production of the other immunoglobulins
    Lymphadenopathy
    Hepatosplenomegaly
    Increased blood viscosity due to IgM production
    Possible interference with platelet function, fibrin polymerization, and function of other coag proteins
    Serum electrophoresis by an "M" spike in the gamma globulin region
    Marked rouleaux
    Increased ESR
    Plasmacytoid lymphocytes
    Plasma cells and Lymps on blood smear
    Monoclonal gammopathy in Waldenstrom macroglobulinemia
  3. What condition:
    Causes a proliferation of malignant cells in solid lymphatic tissue, lymphadenopathy, and intially localized but may spread to bone marrow and blood
    Diagnosed: Tissue biopsy, CD surface markers, Cytogenetics, DNA analysis/PCR
    Lymphoma
  4. What are the groups of lymphoma, according to WHO?
    • Hodgkin B cell
    • T/NK cell (non-Hodgkin) neoplasms
  5. What condition:
    Has Reed-Sternberg (RS) cell found in lymph node biopsy are large, multi-nucleated cells each with prominent large nucleoli (B cell lineage)
    Mild anemia
    Eosinophilia
    Monocytosis
    Increased LAP score
    Increase ESR during active disease state
    Causes: Enlarged lymph nodes, Gastrointestinal tumors, B cell neoplasms (more common)
    Hodgkin lymphoma
  6. What are the subtypes of Hodgkin lymphoma, according to WHO?
    • Nodular sclerosis - 70% are this subtype; lowest EBV association
    • Mixed cellularity - 20% are this subtype; highest EBV association
    • Lymphocyte rich
    • Lymphocyte depleted - uncommon
    • All subtypes are associated with RS cells
  7. What does Mycosis funoides (cutaneous T cell lymphoma) cause?
    • Cutaneous lymphoma causes skin itching, leading to ulcerative tumors
    • Sezary syndrome: a variant of mycosis fungoides, presents as disseminated disease with widespread skin involvement and circulating lymphoma cells
  8. How does WHO classify Mycosis fungoides (cutaneous T cell lymphoma)?
    T/NK cell neoplasm
  9. What cell markers are present in Mycosis fungoides / T/NK cell neoplasm?
    • CD2
    • CD3
    • CD4
  10. What condition:
    Neutropenia
    Anemia
    Thrombocytopenia
    Variable WBC count
    Hypercellular marrow with bone marrow blasts >/=20% (WHO) or >30% (FAB)
    Acute Myeloproliferative Disorder
  11. What FAB classification is consistent with the findings of:
    Blasts exhibit myeloid markers CD13, CD33, CD34
    Stain negatively with the usual cytochemical stains (MPO and Sudan black B (SBB))
    FAB M0
  12. What FAB classification is consistent with the findings of:
    90% or more marrow myeloblasts
    May have Auer rods
    SBB, MPO, and specific esterase positive
    FAB M1
  13. What FAB classification is consistent with the findings of:
    <90% marrow myeloblasts
    May have Auer rods
    Chromosome abnormality t(8;21)
    SBB, MPO, and specific esterase positive
    CD13, CD33 Positive
    FAB M2 (AML with maturation)
  14. What disease is consistent with the findings:
    >30% marrow promyelocytes with bundles of Auer rods (faggot cells)
    Heavy azurophilic granulation
    SBB, MPO, and specific esterase positive
    CD13, CD33 positive
    Chromosome abnormality t(15;17)
    • Acute promyelocytic leukemia (APL)
    • or FAB M3
  15. What disease is consistent with the findings:
    >/=20% marrow myeloblasts (WHO) or >30% (FAB), may have auer rods
    Proliferation of unipotential stem cell CFU-GM that gives rise to granulocytes and monocytes
    Increased urine/serum lysozyme
    SBB, MPO, specific and nonspecific esterase positive
    CD13, CD33, and CD14 positive
    • Acute myelomonocytic leukemia (AMML)
    • or FAB M4
  16. What disease is consistent with the findings:
    >/=20% or >30% marrow monoblasts
    Nonspecific esterase positive
    CD14 positive
    • Acute monocytic leukemia (AMoL)
    • or FAB M5
  17. What disease is consistent with the findings:
    >/=20% or 30% marrow myeloblasts
    >50% dysplastic marrow normoblasts
    Malignant normoblasts are
    - PAS positive
    - CD45, CD71
    Myeloblasts are
    - SBB and MPO positive
    - CD13, CD15, CD33
    • Acute erythroleukemia (AEL)/Di Guglielmo syndrome
    • or FAB M6
  18. What disease is consistent with the findings:
    Proliferation of megakaryocytes and atypical megakaryocytes in the bone marrow
    Blasts may have cytoplasmic blebs
    Marrow aspiration is a dry tap
    Blood shows pancytopenia
    CD41, CD42, CD61
    • Acute megakaryocytic leukemia (AMegL)
    • or FAB M7
  19. What is the term for containing 2 cell populations that expresses
    Myeloid antigens
    Lymphoid antigens
    Bilineage leukemias
  20. What is the term for occuring when myeloid and lymphoid antigens are expressed on the same cell?
    Biphenotypic leukemia
  21. What oncogene is implicated in polycythemia vera, chronic idiopathic myelofibrosis, and essential thrombocythemia?
    JAK2 oncogene
  22. What oncogene is associated in chronic myelogenous leukemia?
    BCR/ABL
  23. What disease is consistent with the findings:
    Proliferation of granulocytes
    Increased M:E ratio
    WBC between 50-500, with all stages of granulocyte production
    Myelocytes dominate, may have few circulating blasts
    LAP score is low
    Chronic myelogenous leukemia (CML)
  24. What disease is consistent with the findings:
    Chromosome translocation t(9;22)
    All cell lines affected, except lymphs
    Philadelphia chromosome
  25. What disease is consistent with the findings:
    Proliferation of megakaryocytes
    Platelets >1000
    Platelet function abnormalities
    Leukocytosis
    Essential thrombocythemia (ET)
  26. What disease is consistent with the findings:
    Malignant hyperplasia of mulipotential myeloid stem cell - causing an increase in all cell lines (polycythemia)
    Erythrocytes most greatly increased despite decreased erythropoietin (EPO)
    High blood viscosity
    RBC in between 7-10
    Hgb >20 g/dL
    HCT >60%
    Polycythemia vera (PV)
  27. What disease is consistent with the findings:
    Myeloid stem cell disorder characterized by proliferation of erythroid, granulocytic, and megakaryocytic precursors in marrow with dyspoiesis
    Progressive marrow fibrosis
    Poikilocytosis with tear drop cells
    Leukoerythroblastic anemia (immature neutrophils and NRBCs)
    Abnormal morphology in all cell lines
    Chronic idiopathic myelofibrosis
  28. What disease is consistent with the findings:
    Anemia that is refractory (not responsive) to therapy
    Oval macrocytes
    Reticulocytopenia
    dyserythropoiesis
    Bone marrow blasts <5%
    Peripheral blood blasts <1%
    Refractory anemia (RA)

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