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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
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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
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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
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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
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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
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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
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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
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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
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How does Lazy Leukocyte Syndrome affect cells?
- A disorder of chemotaxis
- Neutrophils don't migrate effectively
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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
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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
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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
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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
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Define the counts for lymphocytosis and relative lymphocytosis
- abs. infants: >1,000/uL
- abs. adults: >400/uL
- rel.: other cell types are decreased
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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
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What are the various causes of reactive lymphocytes?
- Infectious Mononucleosis (EBV)
- Cytomegalovirus and other viral infections
- Bacterial infections
- Malignancies (cancers)
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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
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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
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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
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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)
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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
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What are the types of Lysosomal Storage Diseases?
- Gaucher's disease
- Niemann-Pick disease
- Tay-Sachs disease
- Hurler's syndrome
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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
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Describe the appearance of Gaucher cells
- Histocyte, 20-100um (huge)
- 1+ round/oval eccentric nuclei
- pale blue/white cytoplasm with "crumpled paper" appearance
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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)
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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
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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
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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
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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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