HematologyTest2NonNeoplasticLeukocyteDisorders

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victimsofadown
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HematologyTest2NonNeoplasticLeukocyteDisorders
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2014-04-24 16:15:34
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HematologyTest2NonNeoplasticLeukocyteDisorders
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HematologyTest2NonNeoplasticLeukocyteDisorders
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  1. Define non-neoplastic, leukocytosis, leukopenia, leukomoid reaction, granulocytosis, neutrophilia, neutropenia
    • Non-neoplastic: regarding a tumor (cancerous)
    • leukocytosis: absolute WBC >11,000/μL
    • leukopenia: absolute WBC < 4,300/μL 
    • leukomoid reaction: WBC 10,000-20,000/μL
    • caused by infection, inflammation, toxin
    • transitory
    • most cells are neutrophilic myelocytes, metamyelocytes, bands and segs
    • granulocytosis: absolute increase in granulocytes
    • neutrophilia: absolute increase in neutrophils
    • neutropenia: absolute decrease in neutrophils
  2. What is occurring in pseudoneutrophilia and pseudoneutropenia?
    • Normal: an equal amount of circulating and marginating neutrophils
    • Pseudoneutrophilia: more circulating and less marginating neutrophils
    • Pseudoneutropenia: more marginating and less circulating neutrophils
  3. Describe the neutrophil response to infection
    • Increased bone marrow production of neutrophils
    • shift to the left (younger cells)
    • absolute neutrophilia
    • Reactive changes in morphology
    • Toxic granulation (very pronounced granules)
    • Dohle bodies (small bluish off-center vacuole-type structure)
    • cytoplasmic vacuolization (slide artifact)
    • Increased enzyme activity
    • Leukocyte alkaline phosphatase (LAP)
    • Myeloperoxidase
  4. What are the various subclassifications of acquired neutropenia with a brief description of each
    • Infection: various viruses
    • reflects a lymphocyte increase
    • drug ingestion: often medications
    • Immune-mediated: caused by allo or auto antibodies
    • absolute neutrophil counts <500/μL
    • Alloimmune neonatal: IgG
    • similarl to HDN response
    • antibody from mother crosses placenta, attacks fetal neutrophils coated with foreign antigens
    • Autoimmune neutropenia: IgG, IgM, and IgA
    • primarily idiopathic
    • secondary to RA, SLE, chronic hepatitis
    • characterized by recurrent infections
  5. What is congenital neutropenia? What are its symptoms? What is the prognosis?
    • Intrinsic to bone marrow environment
    • Inherited abnormality in myeloid line cells (or those involved in regulating neutrophils)
    • Results in decrease (or arrested) neutrophil production (or release)
    • Prognosis varies from little risk of infection to severe infections resulting in death
  6. What characterizes qualitative disorders of neutrophils? What are the names of the 3 tha we discussed in class?
    • Characterized by bacterial infections caused by congenital functional abnormalities
    • Chediak-Higashi syndrome
    • Chronic granulomatous disease
    • Lazy leukocyte disorder
  7. What are the symptoms of Chediak-Higashi Syndrome? What do the affected cells look like? How are the cells affected? Prognosis? Treatment?
    • Individuals have partial albinism
    • Granulocytes, monocytes, lymphocytes, macrophages, platelets, and melanocytes contain GIANT lysosomal granules in cytoplasm
    • Results in impaired release of enzymes due to abnormal fusion of primary and specific granules
    • Ineffective bacterial killing abilities
    • Many die due to severe infections
    • Treated with antibiotics, immunization against EBV, and bone marrow transplantation
  8. What causes Chronic Granulomatous Disease? How are the cells affected? Special stain?
    • Results from failure or decrease in production of superoxide
    • Decreased bacterial killing ability
    • Phagocytosis is unaffected
    • Nitroblue tetrazolium test (NBT) is a special stain
  9. How does Lazy Leukocyte Syndrome affect cells?
    • A disorder of chemotaxis
    • Neutrophils don't migrate effectively
  10. What defines hypersegmentation in a neutrophil? How does it affect the cells? What causes the disorder?
    • 6+ lobes in neutrophil
    • Does not affect function of cell
    • Seen in megaloblastic anemia or hereditary hypersegmentation
  11. Describe how Pelger-Huet affects the appearance of the cells.  How does one contract Pelger-Huet? What else might is look like? What is the "telltale" sign?
    • heterozygotes: mature neutrophils with bilobed nucleus (dumbell)
    • homozygotes: mature neutrophils with round or oval nucleus
    • can be acquired after drug ingestion
    • May be confused with "shift to the left"
    • Over 70-90% of the neutrophils are affected
  12. Describe how Alder's anomaly affects the appearance of cells. Also seen in? Not to be confused with?
    • Alder-Reilly inclusions: dark staining cytoplasmic granules (composed of precipitated mucopolysaccharide)
    • Also seen in Hurler's syndrome
    • May be confused with toxic granulation
  13. How does one acquire the May-Hegglin anomaly? How are the cells affected? Other telltale signs?
    • Congenital disease
    • Neutrophils and monocytes contain Dohle bodies (large RNA granules in the Hoff region that stain blue)
    • Thrombocytopenia with giant platelets
  14. Define the counts for lymphocytosis and relative lymphocytosis
    • abs. infants: >1,000/uL
    • abs. adults: >400/uL
    • rel.: other cell types are decreased
  15. What is a reactive lymphocyte? Describe its morphology
    • Transformed or benign lymphocytes
    • "Atypical" is no longer a medically sanctioned term
    • Larger cells (9-30um)
    • Abundant cytoplasm (usually pale blue w/ darker edges)
    • unevenly stained
    • possible azurophilic granulation
    • Nucleus may be round, oval, or indented
    • Chromatin may be clumped and contain nucleoli
  16. What are the various causes of reactive lymphocytes?
    • Infectious Mononucleosis (EBV)
    • Cytomegalovirus and other viral infections
    • Bacterial infections
    • Malignancies (cancers)
  17. Describe the etiology of mononucleosis? What ages does it affect? What are its symptoms (not blood findings)? Its treatment?
    • EBV enters through oral cavity and infects B cells
    • reactive T cells respond to this infection
    • Most common at 17-25 (uncommon in elderly)
    • sore throat, malaise, fever, swollen lymph nodes
    • Palpable spleen and liver are possible
    • Treated with bed rest, pain relievers, and good diet
  18. Describe the peripheral blood findings of mononucleosis?
    • WBC counts: 10,000-20,000/uL
    • >50% lymphocytes (variable morphology)
    • >10% reactive lymphocytes
    • monocytosis
    • relative neutropenia
    • increased liver enzymes and bilirubin
  19. How is mononucleosis diagnosed?
    • Evaluation of blood film
    • patient serum has IgM antibodies that cross-react with sheep RBCs
    • Monospot test: rise in heterophile antibody parallels rise in EBV antibodies
  20. What is cytomegalovirus related to? How is it transmitted?  What are its symptoms? Prognosis? How is it differentiated from EBV?
    • Related to herpes virus
    • Transmitted via oral, respiratory, sexual, blood, and organ transplantation
    • Fever, sore throat, muscle pain, organomegaly (or may be asymptomatic)
    • can be life-threatening in immunocompromised
    • Gives similar appearance to EBV
    • Monospot test negative (EBV is positive)
  21. What are the general characteristics of Lysosomal Storage Diseases? (type of disorder? etiology? symptoms? treatment?)
    • Rare, inherited disorders (many ethnically related)
    • Etiology is enzyme deficiency in lipid or mucopolysaccharide metabolism
    • Asymptomatic to severe symptoms
    • Enzyme replacement has been effective
  22. What are the types of Lysosomal Storage Diseases?
    • Gaucher's disease
    • Niemann-Pick disease
    • Tay-Sachs disease
    • Hurler's syndrome
  23. What is the cause of Gaucher's Disease? How does this affect cells? Special cell names? What causes symptoms?
    • Deficiency of beta glucocerebrosidase
    • Accumulation of unmetabolized substrate (glucocerebroside) in reticuloendothelial cells of organs and skeleton
    • affected RE cells are termed "Gaucher cells"

    Gaucher cells replace bone cells which leads to skeletal disease
  24. Describe the appearance of Gaucher cells
    • Histocyte, 20-100um (huge)
    • 1+ round/oval eccentric nuclei
    • pale blue/white cytoplasm with "crumpled paper" appearance
  25. What is the cause of Niemann-Pick Disease? How does this affect cells? What makes this such a serious disorder?
    • Deficiency of sphingomyelinase
    • Accumulation of unmetabolized lipid (sphingomyelin) and cholesterol in RE cells of bone marrow, tissues, and organs
    • Serious disorder because sphingomyelin is in membranes of cells and organelles (including the nervous system)
  26. Describe the appearance of Niemann-Pick cells
    • histiocytes, 20-90um
    • inconspicuous, round nucleus
    • cytoplasm is very pale blue or white
    • cytoplasm filled with round uniformly-sized lipid droplets
  27. What is the cause of Tay-Sachs Disease? How does this affect cells? How does this affect the body? What cells would appear? Prognosis?
    • Deficiency of hexosaminidase A
    • Increase in hexosaminidase B
    • Accumulation of unmetabolized gangliosides in ALL tissues (most sever effects in CNS and eye)
    • Does not affect spleen, liver, or lymph nodes
    • Vacuolated lymphocytes found in blood
    • Death usually occurs by age 4
  28. What is the cause of Hurler's syndrome? How does this affect cells? What symptoms would be seen?  Prognosis?
    • Deficiency in a-1-iduronidase
    • Abnormal breakdown of mucopolysaccharides
    • Accumulation of heparin sulfate and dermatan sulfate in RE cells
    • Skeletal abnormalities, organomegaly, coarse facial features, widely-spaced eyes, opaque corneas, mental retardation
    • Life expectancy is ~10 years
  29. What type of disorder is Sea-Blue Histiocyte Syndrome? What are its symptoms? What do the cells look like? Other anatomical abnormalities and blood abnormalities?
    • A benign, genetic disorder
    • Named due to staining qualities of histiocyte cytoplasm
    • Minor splenomegaly and hepatomegaly
    • Possible abnormalities with eyes, skin, and nervous system
    • Thrombocytopenia in blood

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