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Hematology - Granulocytic Disorders


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What is neutropenia and what are lab values?
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
4 mechanisms of neutropenia
1. decreased production of neutrophils
2. increased cell loss
3. pseudoneutropenia
4. false neutropenia
What does decreased production of neutrophils result in?
Decrease in BM storage pool
Decrease of egression to tissues
Decrease in circulating & marginating neuts
A LEFT SHIFT = more immature neuts released
What can cause decreased production of neutrophils?
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
What does increased cell loss result in (in Neutropenia)?
neutrophil egression to tissues INCREASES. Looks like a decrease in PB smear
What can caused an increased cell loss (egression of neuts to tissues)?
-severe infection
-immune rxts w/ leukoagglutinins
(by anti-leukocyte Ab, chemokines => autoimmunity)
What is pseudoneutropenia?
transfer of neutrophils from circ to marginated zones. Total Neut count unchanged.
What is false neutrophenia?
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
What is neutrophilia, and what are the 4 classes?
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)
what is a leukemoid reaction?
a benign abnormality, increase in leukocytes, particularly neuts. Usually not past 30,000 neuts/uL
three disorders of cytoplasmic granules
myeloperoxidase deficiency
Chediak-Higashi syndrome
abnormal PMN-specific granules
what is Myeloperoxidase deficiency?
usually inherited
reduction/absence of myeloperoxidase enzyme
presents w/ immune deficiency
what is Chediak-Higashi syndrome?
rare, inherited & serious
-fatal pyogenic infections
-giant lysozomes w/ decr enzymes, impaired delivery of enzymes to phagozome
-neutropenia/thrombocytopenia common complications
what are characteristics of abnormal PMN-specific granules?
granules can decrease
granules can be fnctnly abnrml
neuts appear hypogranular w/ irreg, bi-lobed nuclei
Skin & sinus infections
What is Chronic Granulomatus Disease (CGD)?
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)
What is Pelger-Huet Anomaly?
autosomal dominant or drug induced
pince-nez shaped nuclei of neuts
unlobulated eos possible
normal function, asymptomatic
What is Alder-Reilly Anomaly?
-abnormal granulation of granulocytes assoc w/ genetic mucopolysaccharidoses (MPS)
-dense azurophilic granules in neuts resembles toxic gran, but even intensity from cell 2 cell
What is May-Hegglin Anomaly?
rare, autosomal dominant
leuks & platelets affected
inclusions resembling Dohle bodies in leuks but bigger & more obvious
giant platelets, thrombocytopenia
asymptomatic usually

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