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101 Cards in this Set

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  • Back
What can myeloid stem cells differentiate into?
Basophils
Eosinophils
Neutrophils
Monocytes
Platelets
Erythrocytes
Basophils
Eosinophils
Neutrophils
Monocytes
Platelets
Erythrocytes
What can myeloblasts differentiate into?
Basophils
Eosinophils
Neutrophils
Basophils
Eosinophils
Neutrophils
What disease is associated with N. promyelocytes?
Severe congenital neutropenia

There's an arrest in the bone marrow maturation
What are the properties of the neutrophil?
Common
Nucleus is 2-5 segments
Light pink granules
Diameter: 12-15 uM
Common
Nucleus is 2-5 segments
Light pink granules
Diameter: 12-15 uM
What are the properties of the monocyte?
Commonly observed
Horse-shoe shaped nucleus
Diameter: 16-20 uM
Commonly observed
Horse-shoe shaped nucleus
Diameter: 16-20 uM
What are the properties of the eosinophil?
Rare
Bi-lobed nucleus
Vibrant pink-orange granules
Diameter: 12-17 uM
Rare
Bi-lobed nucleus
Vibrant pink-orange granules
Diameter: 12-17 uM
What are the properties of the basophil?
Very rare
Bi-lobed nucleus
Large blue-purple granules
Diameter: 9-10 uM
Very rare
Bi-lobed nucleus
Large blue-purple granules
Diameter: 9-10 uM
Where does neutrophil differentiation take place?
Bone marrow: proliferiation, maturation
Circulation: final differentiation
How long does neutrophil maturation take?
14 days
How many of the neutrophils are in the proliferation phase?
25%

6-7 days while proliferating
What are the proliferative neutrophils?
Myeloblasts
Promyelocyte
Myelocyte

Mitotic
Myeloblasts
Promyelocyte
Myelocyte

Mitotic
How many of the neutrophils are in the maturation phase?
65%

Total of 6-7 days maturing
What are the maturing neutrophils?
Metmyelocyte
Bands
Neutrophils
Metmyelocyte
Bands
Neutrophils
What percentage of neutrophils are normally in the tissue? Circulation?
Tissue: 2%

Circulating: 10%
What;s the NL for neutrophils?
1.5-8.0 E 9 cells/L
What's the definition of moderate neutropenia? Severe?
Moderate: .5-1.0 E9 cells/L
Severe: 0-.5 E9 cells/L

The lower the neutrophil levels, the more severe the risk of infection.
What are the normal levels of neutrophils for term infants? Infants? Kids?
Term infant: 3,000
Infant: 1,100
Kids, adolescent, adults: >1,500
What ethnic groups have lower absolute neutrophil counts?
African-americans.

They don't have symptoms, though!
How do you calculate the absolute neutrophil count (ANC)?
ANC = (% bands + % segmented neutrophils) * total WBC
When should you have a clinical suspicion for neutropenia?
Acute, severe bacterial infection
History of recurrent/rare infections
Prolonged or recurrent fevers
Recurrent mouth ulcers, chronic gingivitis, cellulitis, perirectal abscesses
Findings associated with malignancy, immunodeficiency or syndrome
What is unique about the height and weight of patients with neutropenia?
Short stature compared to weight
Where are there often abnormal findings in a neutropenic patient?
Oral cavity!
What parts of the extremities are commonly compromised in patients with neutropenia?
Forearms
Thumbs
What oral findings are characteristic of a patient with neutropenia?
What's usually the cause of neutropenia?
Infection (virus, normally)
Drug-induced
Immune-based
What patients are particularly vulnerable to neutropenia?
Neonates

They can exhaust their bone marrow/reserve neutrophils easily->bacterial sepsis
What are the common mechanisms for drug-induced neutropenia?
Toxic
Immunologic
Hypersensitivity
What are some common drug classes that cause neutropenia?
Anticonvulsants
Antipsychotics
Antibiotics: chlorampehicol, bactram
What are the different types of immune-based acquired neutropenias?
Alloimmune neutropenia of infancy
Autoimmune neutropenia of infancy
How does the alloimmune neutropenia of infancy occur?
Passive transfer of maternal IgG against fetal neutrophils
Once the antibodies are cleared, the patient gets better
This can happen with the first pregnancy - a way to differentiate from Rh disease
How does the autoimmune neutropenia of infancy occur?
Appears around 1 year
ANC of 200-300 is classic

Treatment isn't really indicated; the reserve is still there.
What are the different types of inherited neutropenias?
Primary disorders of myelopoiesis
Disorders of ribosomal function
Disorders of granule sorting/trafficking
Disorders of metabolism
Neutropenia in disorders of immune fuction
What is the most common kind of congenital neutropenia?
Severe congenital neutropenia
What is the presentation of severe congenital neutropenia?
Infant presents in the first year of life with recurrent/severe infections
What's required for a diagnosis of severe congenital neutropenia?
Three ANCs < 500
What mutations are associated with severe congenital neutropenia?
ELANE (autosomal dominant)
HAX1 (autosomal recessive)
What's the treatment for severe congenital neutropenia?
G-CSF
Bone marrow transplant
What diseases are more common in patients with severe congenital neutropenia?
AML
MDS
What is cyclic neutropenia?
Cycles of neutropenia
How long are the cycles in cyclic neutropenia?
21 days

7-10 days of neutropenia
What's the presentationof cyclic neutropenia?
Presents before 1

Recurrent fevers, malaise, apthous ulcers, bacterial infections
What bacteria are especially pathogenic to people with cyclic neutropenia?
Clostridium septicum
How do you diagnose cyclic neutropenia?
You do CBCs 2-3 times/week for 6 weeks
What gene is mutated in cyclic neutropenia?
ELANE gene
What's the treatment for cyclic neutropenia?
G-CSF

Shortens the duration of the neutropenia

NO RISK OF LEUKEMIA
What is the definition of neutrophilia?
Abnormally high neutrophil counts
What's the cause of neutrophilia?
Acute shift from the marginating pool to the circulating pool
Chronic stimulation with excess cytokines increasing the proliferating pool
What are some things that can cause neutrophils to shift to the circulating pool from the marginating pool?
Steroids
Exercise
Epinephrine
Hypoxia
Seizures
What are somethings that can cause chronic proliferation of neutrophils?
Infections
G-CSF
Pregnancy
Trisomy 21
Recovery from chemo
Myeloproliferative disorders
What are the stages of neutrophil killing?
1. Mobilization
2. Chemotaxis
3. Recognition/opsinization
4.Ingestion
5. Degranulation
6. Peroxidation
7. Killing and digestion
8. Net formation
What is myelokathexis?
Apoptosis and retention of neutrophils in the bone marrow
What gene is mutated in myelokathexis?
CXCR4 chemokine receptor
What are the clinical characteristics of myelokathexis?
WHIM!

W: warts
H: hypergammaglobulinemia
I: infections
M: myelokathexis
What's the treatmnet for myelokathexis?
G-CSF
CXCR4 antagonist
What is the apperance of neutrophils in WHIM syndrome?
Lots of segmentation of the neutrophils in the bone marrow.
What are the steps of neutrophil migration into tissues?
What molecules are involved in neutrophil rolling?
Selectins
What molecules are involved in adhesion of the neutrophils to the endothelium?
CD11/CD18
What are the findings in leukocyte adhesion deficiency?
Neutrophilia
Infections
Absence of pus formation
Impaired wound healing
Delayed umbilical cord separation (LAD1)
What is the classic oral finding in LAD?
Peridonitis
Peridonitis
What defect is present in LAD1? What process is inhibited?
B2 integrin CD18

Adhesion
What's the treatment for LAD1?
Bone marrow transplant
What molecules are mutated in LAD1? What process is inhibited?
Selectins

Rolling; can't be tethered to the tissue
What are the findings in LAD2?
Neurologic defects
Craniofacial anamolies
Rare erythrocyte phenotype
What's the treatment for LAD2?
Very little we can do for them.
What gene is mutated in LAD3? What process is inhibited?
Kindlin

Integrin activation
What are the findings in LAD3?
Neutrophil AND bleeding problems.
What is Chediak higashi syndrome?
Autosomal recessive disorder
What are the findings in Chediak higashi syndrome?
Immunodeficiency
Partial oculo-cutaneous albinism
Bleeding diathesis
Progressive neurologic deterioration
Oddly colored hair
What process are people with Chediak Higashi syndrome unable to perform?
Granule trafficking in neutrophils

You see giant neutrophils!
What gene is mutated in Chediak higashi syndrome?
CHS1/LYST gene

Responsible for lysosomal trafficking
What are people with chediak higshi syndrome vulnerable to?
Hemophagocytic lymphohistiocytosis: can't turn off phagocytois

Hepatomegaly, splenomegaly
What's the treatment for Chediak higashi syndrome?
Antibiotics
G-CSF
Bone marrow treatment (cures hematologic, immunodeficiency problems, doesn't affect albinism/neurologic features)
What are the different types of neutrophil granules?
Primary: neutral proteases, myeloperoxidase
Secondary: lactoferrin
Tertiary: gelatinase
Secretory: alkaline phosphatase
What are the findings in myeloperoxidase deficiency?
Largely asymptomatic

Only see disease in people with diabetes
What is the action of myeloperoxidase?
Forms bleach from H2O2 and chloride
What organism is particularly targeted by the formation of HOCl in the body?
Candida
How does acquired MPO deficiency happen?
You get a disruption of the bone marrow that causes an induced mutation
What is the treatment for MPO?
Very little for the disease itself

Aggressively manage the fungal diseases
Control the glucose tightly if there's DM
What occurs in chronic granulomatous disease?
Mutation in NADPH oxidase-->no superoxide

Can't destroy catalase-positive microbes
What kinds of infections are people with CGD vulnerable to?
S. aureus
Aspergillus
S. aureus
Aspergillus
Where do you get infections in CGD?
Lungs
Skin
Lymph nodes
LIVER
What's commonly mutated in NADPH in CGD?
gp91phox: 65%; X-linked

p47phox: 25%; autosomal recessive
How do you go about diagnosing CG?
Rhodamine dye test: dye is taken up by phagocytes-->oxidized; fluoresence

Nitroblue tetrazolium test: changing the color of a dye when superoxide is produced
What's the treatment for CGD?
Antimicrobial prophylaxis: bactram
Immune modulation: interferon gamma
Aggressive treatment of infections

Bone marrow transplant is the only cure
What process needs to be intact for net formation to occur? What disease states cannot perform this?
Hydrogen peroxide production

CGD
MPO
What are the stages of monocyte differentiation?
Proliferation
Maturation
SHorter tissue phase

They then differentiate into macrophages
What's the usual monocyte count?
300/uL
How long does it take monocytes to arrive at the scene?
12 hours
What are causes of a low monocyte count?
Stress
Endotoxemia
Glucocorticoids
What are causes if high monocyte counts?
Bone marrow disease
Infection
Inflammatory disease
What are the stages of eosinophil naturation?
Proliferation (9 days)
Maturation

Intravascular
What is the precursor to eosinophils?
Myeocytes
What are some causes of eosinopenia?
Infection
Corticosteroids
Prostaglandins
Epinephrine

No additional risks from having this
What is eosinophilia? What are causes?
>500/ uL

Causes:
Neoplasms
Allergies
Asthma
Collagen vascular diseases
What are the characteristics of idiopathic hypereosinophilic syndrome?
>1500 cells/uL for 6 months

Happens in people who are 20-50

Lots of organ dysfunction
What is the cause of pathology in idiopathic hypereosinophilic syndrome?
Large scale eosinophil degranulation and release of major basic protein into the tissues

You can also get an eosinophilic leukemia
How long do basophils live in the tissues?
A long time!

Up to days
What are causes of low basophil counts?
Hypersensitivity
Glucocorticoids
What's the role of the basophil?
Unknown.
What causes a high basophil count?
Allergies
Infections
Endocrinopathies
Myeloproliferative disorders
Systemic mastocytosis