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18 Cards in this Set
- Front
- Back
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What is neutropenia and what are lab values?
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blood neutrophil count below normal
Children: <1.0 x 10^9/L Afr-Amer: <1.3 x 10^9/L Caucasians: <1.5 x 10^9/L danger level at <0.2 x 10^9/L |
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4 mechanisms of neutropenia
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1. decreased production of neutrophils
2. increased cell loss 3. pseudoneutropenia 4. false neutropenia |
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What does decreased production of neutrophils result in?
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Decrease in BM storage pool
Decrease of egression to tissues Decrease in circulating & marginating neuts A LEFT SHIFT = more immature neuts released |
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What can cause decreased production of neutrophils?
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1. stem cell failure = 2ndary cond from aplastic or leukemia
2. radiotherapy / chemotherapy 3. megaloblastic anemias 4. cyclic neutropenia - rare, inherited, severe acute neutropenia 5. congenital disorders |
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What does increased cell loss result in (in Neutropenia)?
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neutrophil egression to tissues INCREASES. Looks like a decrease in PB smear
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What can caused an increased cell loss (egression of neuts to tissues)?
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-severe infection
-immune rxts w/ leukoagglutinins (by anti-leukocyte Ab, chemokines => autoimmunity) |
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What is pseudoneutropenia?
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transfer of neutrophils from circ to marginated zones. Total Neut count unchanged.
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What is false neutrophenia?
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caused by in vitro handling of PB sample
-EDTA has trace Ab able to activate neuts -old blood: neuts disintegrate faster than other blood cells -fragile leukocytes in pathologic disease -leukocyte clumping |
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What is neutrophilia, and what are the 4 classes?
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increase in total circulating mass of neutrophils
>7.0 x 10^9/L in adults a. immediate (pseudoneutrophilia) b. acute (rapidly follows pathogenic stimulus) c. chronic (continued neutrophilic response) d. misc (corticosteroid, leukemoid, leukoerythroblastic) |
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what is a leukemoid reaction?
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a benign abnormality, increase in leukocytes, particularly neuts. Usually not past 30,000 neuts/uL
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three disorders of cytoplasmic granules
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myeloperoxidase deficiency
Chediak-Higashi syndrome abnormal PMN-specific granules |
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what is Myeloperoxidase deficiency?
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usually inherited
reduction/absence of myeloperoxidase enzyme presents w/ immune deficiency |
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what is Chediak-Higashi syndrome?
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rare, inherited & serious
-fatal pyogenic infections -giant lysozomes w/ decr enzymes, impaired delivery of enzymes to phagozome -neutropenia/thrombocytopenia common complications |
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what are characteristics of abnormal PMN-specific granules?
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granules can decrease
granules can be fnctnly abnrml neuts appear hypogranular w/ irreg, bi-lobed nuclei Skin & sinus infections |
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What is Chronic Granulomatus Disease (CGD)?
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sex-linked, fatal & rare
neuts can't induce post-phagocytic oxidative burst recurrent infections of orgs of low pathogenicity chracteristic granulomas Dx'd by Nitroblue Tetrazolium Test (NBT) |
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What is Pelger-Huet Anomaly?
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autosomal dominant or drug induced
pince-nez shaped nuclei of neuts unlobulated eos possible normal function, asymptomatic |
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What is Alder-Reilly Anomaly?
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-abnormal granulation of granulocytes assoc w/ genetic mucopolysaccharidoses (MPS)
-dense azurophilic granules in neuts resembles toxic gran, but even intensity from cell 2 cell |
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What is May-Hegglin Anomaly?
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rare, autosomal dominant
leuks & platelets affected inclusions resembling Dohle bodies in leuks but bigger & more obvious giant platelets, thrombocytopenia asymptomatic usually |
