Malignant Leukocyte Disorders

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Malignant Leukocyte Disorders
2013-06-04 21:01:41
Review Acute Chronic Leukemias Cytochemical Stains Lymphoproliferative Disorders Immunophenotyping

Review, Acute and Chronic Leukemias, Cytochemical Stains, Acute Lymphoproliferative Disorders, Immunophenotyping, Chronic Lymphoproliferative Disorders
Show Answers:

  1. What disorder originates in the bone marrow and is initially systemic?
  2. What disorder originates in the lymphoid tissues and is initially localized?
  3. Name some of the theories behind oncogenic activation
    • Viral - viruses can suppress immune function or activate oncogenes (HTLV-I, II, V) and HIV-I
    • Bone marrow damage
    • Chromosome defects
    • Genetic factors
    • Immune dysfunction
  4. What test is diagnostic for acute promyelocytic leukemia?
  5. How are malignant disorders classified and name some
    • Classified by stem cell cell involved and length of clinical duration
    • Lymphoproliferative disorders: actue or chronic
    • Myeloproliferative disorders: acute or chronic
  6. What specimens are used to aid in diagnosis is malignant disorders?
    • Bone marrow exam - aspirate and biospy
    • Peripheral blood exam - unexplained cytopenias
    • Ongoing monitoring of response to therapy in patients with malignancy
    • Assesses for cellularity, M:E ratio, megakaryocyte evaluation, iron stores, differential
    • Flow cytomety, cytogenetics, molecular, and microbiology
  7. What sites are used for bone marrow biopsies?
    • Posterior superior iliac crest - most common
    • Anterior iliac crest
    • Sternum
  8. How long is the duration for acute and chronic leukemias?
    • Acute: weeks to months without treatment, death is due to infection and bleeding
    • Chronic: Years without treatment
  9. What is the predominant cell type in Acute and Chronic leukemias?
    • Acute: Immature/blasts cells predominant
    • AML: myeloblasts
    • ALL: lymphoblasts
    • Chronic: Maturing or mature cells predominate
    • CML: granulocytes
    • CLL: lymphocytes
  10. What is the clinical and laboratory findings associated with acute leukemia?
    • Sudden onset: affecting all ages
    • Weakness and fatigue due to anemia
    • petechiae and bruising due to thrombocytopenia
    • Fever and infection due to neutropenia
    • Variable leukocyte count:
    • - WHO: Marrow blasts >/=20%
    • - FAB - Marrow blasts >30%
    • - with cellularity >70%
  11. What is the clinical and laboratory findings associated with chronic leukemia?
    • Frequently asymptomatic initially: affects adults
    • Anemia: mild or absent
    • Platelet: normal to slightly increased
    • WBC: high
    • Marrow cellularity is >70%
  12. What type of treatment is used for acute and chronic leukemias?
    • Chemotherapy used is dependent on type of leukemia - proper diagnosis is crucial
    • Radiation
    • Bone marrow/stem cell transplant
    • Supportive with transfusions of RBCs and platelets, antibiotics, and growth factors
  13. What is myeloperoxidase (MPO) stain used for?
    • Auer rods stain positive
    • Lymphocytic cells are negative for this stain
    • Used to differentiate blasts of acute myelogenous leukemias (AMLs) from acute lymphoblastic leukemias (ALLs)
  14. What is Sudan Black B stain used for?
    • Stains phopholipids and lipoproteins
    • Granulocytic cells and auer rods stain positive
    • Lymphocytic cells are negative
    • Used to differentiate blasts of AML from ALL
  15. What is specific esterase stain (naphthol AS-D choloracetate esterase) used for?
    • Detects esterase enzyme present in primary granules of granulocytic cells
    • Monocytic cells negative for this stain
  16. What is the nonspecific stain (alpha napthyl acetate and alpha napthyl butyrate) used for?
    • Detects esterase enzyme present in monocytic cells
    • Granulocytic cells are negative for this stain
  17. What are the esterase stains useful in distinguishing?
    Acute leukemias that are of myeloid origin from those leukemias that are of monocytic origin
  18. What is Periodic acid-Schiff (PAS) stain used for?
    • Stains intracellular glycogen bright pink
    • Immature lymphoid cells, malignant erythroblasts, and megakaryocytic cells stain positive
    • Myeloblasts and normal erythrocytic cells are negative
    • Useful in diagnosis of erythroleukemia and acute lympoblastic leukemia
  19. What is Leukocyte alkaline phosphatase (LAP) stain used for?
    • Detects alkaline phosphatase enzyme activity in primary granules of neutrophils
    • Use to differentiate chronic myelogenous leukemia (CML) from neutrophilic leukemoid reaction (NLR)
  20. Describe the LAP (leukocyte alkaline phosphatase) score and the reference range
    • 100 neutrophils are graded on a scale of 0-4+ based on stain intensity and size of granules
    • Results are added together
    • Reference range is 13-130
  21. What is the clinical significance of a decreased LAP score?
    • CML
    • Paryoxysmal nocturnal hemoglobinuria
  22. What is the clinical significance of an increased LAP score?
    • Neutrophilic leukemoid reaction
    • Polycythemia vera
    • CML in blast crisis
    • Late trimester pregnancy
  23. What is the tartrate-resistant acid phophatase stain (TRAP) used for?
    • Only hairy cells from hairy cell leukemia are resistant to inhibitiion with tartrate and continue to stain positive
    • All other cells negative
  24. What is Perl's Prussian blue stain used for?
    Free iron precipitates, cells called siderocytes (pappenheimer bodies with Wright's stain)
  25. What is associated with increased percentage of siderocytes?
    • Severe hemolytic anemias
    • Iron overload
    • Sideroblastic anemia
    • Post splenectomy
    • Ringed sideroblasts seen in bone marrow of myelodysplastic syndrome
  26. What condition:
    Symptoms: Fever, Bone/joint pain, Bleeding, Hepatosplenomegaly
    Variable WBC count
    hypercellular marrow with bone marrow blasts >/=20%
    Lymphoblasts stain PAS POS
    Acute Lympoproliferative Disorders
  27. What FAB classification has the following findings:
    Most common childhood leukemia
    Small lymphoblasts, homogeneous appearance
    Best prognosis
    Most T cell ALLs
    FAB L1
  28. What FAB classification has the following findings:
    Most common in adults
    Large lymphoblasts, heterogeneous appearance
    FAB L2
  29. What FAB classification has the following findings:
    Leukemic phase of Burkitt lymphoma
    Seen in both adults and children
    Lymphoblasts are large and uniform with prominent nucleoli, cytoplasm stains deeply basophilic and may show vacuoles
    Poor prognosis
    B Cell lineage
    FAB L3
  30. What acute lymphoproliferative disorder has the following findings:
    High-grade non-Hodgkin lymphoma phase of FAB L3 leukemia
    Endemic in East Africa with high association with Epstein-Barr virus
    Children present with jaw/facial bone tumors
    US variant seen in children and young adults, present with abdominal mass 
    Burkitt lymphoma
  31. What are the CD markers of progenitor B cells?
    • CD19
    • CD34
    • TdT
  32. What are the most common subtype and early pre-B cells CD markers?
    • CD10
    • CD19
    • CD34
    • TdT
  33. What are the CD markers of Pre-B cells and the 2nd most common subtype?
    • CD10
    • CD19
    • CD20
    • TdT
  34. What are the CD markers of B cells?
    • CD19
    • CD20
  35. What CD marker is present in all stages of B cells?
  36. What genetic translocation is associated with FAB L3/Burkitt Lymphoma?
    t(8;14) with rearrangement of the MYC oncogene
  37. What genetic translocation is associated with Pre-B cell ALL?
  38. What genetic translocation is associated with B cell ALL?
  39. What genetic translocation is associated with T cell ALL?
  40. What condition:
    Bone marrow: hypercellular
    Blood: absolute lymphocytosis of >5, homogeneous, small, hyperclumped lymphocytes, and smudge cells
    Chronic Lymphocytic Leukemia (CLL)
  41. What condition: 
    B cell malignancy (CD19, CD20 POS)
    Massive splenomegaly, extensive bone marrow involvement results in dry tap on bone marrow aspiration
    Cytoplasm of lymphocytes shows hair-like projections
    Tartrate resistant acid phosphatase (TRAP) stain positive
    Hairy cell leukemia (HCL)
  42. What condition:
    B cell or T cell malignancy
    Marked splenomegaly
    Lymphocytosis >100 with many prolymphs
    Prolymphocytic leukemia (PLL)
  43. Name the following cells identified with the following CD Markers:
    CD2, CD3
    CD11c, CD14
    CD19, CD20
    CD16, CD56
  44. Name what the following cytochemical stains identify:
    Sudan black B
    Specific esterase stain
    Non-Specific esterase stain
    PAS (Periodic Acid-Schiff)
    TRAP (Tartrate resistant acid phosphatase)