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S3B5 Pathology > Inherited & Reactive WBC and PLT Disorders > Flashcards

Inherited & Reactive WBC and PLT Disorders Flashcards

1
Q

Neutrophils in the blood stream can be divided into what categories?

A
  • Circulating Granulocyte Pool (CGP)
    • WBC in CBC
  • Marginating Granulocytic Pool (MGP)
    • move into circulating pool when needed
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2
Q

At what stage do granulocytes enter the blood? How long do they stay there? Where do they go?

A

band or neutrophil stage- leave BM & enter blood

stay in blood for ~10 hrs

randomly enter body tissue - never re-enter blood; die in the tissue

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3
Q

What situations cause the peripheral blood neutrophil count to increase almost immediately? How?

A
  • infection
  • intense exercise
  • release of catecholamines

shift from marginating pool to circulating pool & from early release from BM

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4
Q

The peripheral blood total leukocyte count is influenced by what factors?

A
  • size of myeloid & lymphoid precursor and storage cell pools
  • rate of release of cels from storage pools into the circulation
  • proportion of cells that are marginating at any time
  • rate of extravasation of cells from blood ito tissue
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5
Q

What does it mean for a cell to be “marginating”?

A

adherent to blood vessel walls

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6
Q

What are causes of increased production of myeloid & lymphoid precursors in the bone marrow?

A

infections

paraneoplastic syndromes

myeloproliferative neoplasms

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7
Q

What are causes of increased release of leukocytes from bone marrow stores?

A

endotoxemia & infection

hypoxia

trauma/surgery

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8
Q

What substance causes decreased extravasation into tissues?

A

glucocorticoids

(therefore increases WBC count)

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9
Q

Serious infections cause increase of what specific WBC?

A

neutrophil & bands (b/c early release form BM)

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10
Q

What pathologic cells are seen in the provided slides? They are indicative of what condition?

A

Sepsis / serous infection

  • Toxic granulation (increase in secondary granules)
  • Vacoules in cytoplasm (not specific but common in sepsis)
  • Dohle Bodies (blue arrows) - blue bodies found in the cytoplasm in reaction to sepsis
  • Increased # bands in peripheral blood
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11
Q

What is a Leukemoid reaction? I usually includes what 3 components?

A

severe, persistent, reactive neutrophilic leukocytosis above 50,000/microliter when the cause is something other than leukemia

  • leukocytosis
  • granulocytic left shift
  • thrombocytosis
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12
Q

What other conditions are on the differential along with Leukemoid reaction?

A

CML & chronic neutrophilic leukemia

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13
Q

What is leukoerythroblastosis? What is the usual cause?

A

immature myeloid & nucleated erythroid precursors in peripheral blood - variable leukocytosis on CBC

myelophthisic process (bone marrow disruption/infiltration by tumor/fibrosis)

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14
Q

What reactive condition is shown in the peripheral blood smear?

A

leukoerythroblastosis

immature granulocytes & nucleated RBCs

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15
Q

What non-neoplastic conditions can cause eosinophilia?

A
  • allergic disorders
  • drug hypersensitivities
  • parasitic infections
  • collagen vascular disorders, particularly involving skin
  • some vasculitides
  • some malignancies
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16
Q

What are the causes of basophilia?

A
  • usually neoplastic - myeloproliferative
  • non-neoplastic
    • myxedema
    • ulcerative colitis
    • lymphoma
    • hypersensitivity reaction
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17
Q

How are lymphocytes levels different in adult than children/infants?

A

lymphocyte counts are higher in infants & children

18
Q

What are the possible causes of monocytosis?

A
  • chronic infections
  • inflammatory disorder
  • paraneoplastic syndromes (lung cancer)
  • recovery from bone marrow suppression
19
Q

What are the possible causes of lymphocytosis?

A
  • transient stress reaction
    • MI
    • cardiac arrest
    • trauma
  • viral infections
    • EBV, CMV, Hantavirus
  • Bordatella pertussis
  • Drug Reaction & cigarettes
  • Chronic Infections
    • tuberculosis, brucellosis, toxoplasmosis, malaria
20
Q

What are the non-neoplastic causes of eosinophilia?

A
  • allergic disorder
  • drug hypersensitivities
  • parasitic
21
Q

What are the non-neoplastic causes of neutropenia?

A

drugs

radiation

toxins

intrinsic defects

immune-mediated

hematologic

infectious

22
Q

What would you expect to see in a bone marrow sample from a patient with proliferation defect resulting in neutropenia?

A

myeloid hypoplasia

decreased proliferation

23
Q

What would you expect to see in a bone marrow sample from a patient with a maturation defect resulting in neutropenia?

A

myeloid hyperplasia

precursors proliferate appropriately but don’t mature properly

24
Q

What would you expect to see in a bone marrow sample from a patient with a survival defect resulting in neutropenia?

A

precursors reproduce & mature but are destroyed early in blood (infections/autoimmune)

hyperplasia in bone marrow

25
What are the potential consequences of severe neutropenia?
opportunistic infections | (more likely when falls below 500)
26
What are the possible causes of Lymphocytopenia?
* Infectious diseases * HIV, other viruses * Erlichiosis * Iatrogenic (**corticosteroids**) * autoimmune * malnutrition
27
What are the causes of monocytopenia?
* aplastic anemia * **Hairy cell leukemia** * corticosteroids * acute infections whith endotoxemia
28
Plasma cell usually comprise what percent of nucleated cells in marrow?
10%
29
Is reactive plasmacytosis monoclonal or polyclonal?
**polyclonal by definition** monoclonal is neoplastic
30
Reactive plasmacytosis is seen in what diseases?
usually found along the capillaries * **HIV** * Post-chemo * cirrhosis * various solid tumors * autoimmune disorders * drug reactions
31
What blood & bone marrow features do you see with an HIV infection?
reactive **plasmacytosis** pancytopenia lymphocytopenia granulomas lymphoid aggregates
32
What can you stain for to more easily identify plasma cells?
CD138 - they look brown
33
Lymphoid aggregates are most commonly seen in what situations?
more common in elderly * infections, drug therapy, immune disorders * hematogones (maturing, immature lymphoid cells - so will see spectrum of markers)
34
Granulomas are most commonly associated with what condition?
infections TB, *M. avium* complex, MAC, Brucellosis but may be seen in almost **any** type of infection
35
What is the inherited condition that causes hyposegmented neutrophils? Inheritance pattern? Clinical significance?
**Pegler-Huet Anomaly** autosomal dominant - present at birth not clinically significant (function & # are normal) -usually bilobed with thin bridge
36
What are the acquired causes of hyposegmented neutrophils? Clinical significance?
hypogranular & dysfunctional (typically one lobe is a bad sign) * myelodysplastic neoplasms * medication effect * some infections
37
The provided peripheral blood smear is form a patient with what inherited condition?
Chedia-Higashi autosomal recessive * giant granules in neutrophils, eosinophils, monocytes & lymphocytes * most patients die in childhood from infections/bleeding
38
The provided peripheral blood smear is form a patient with what inherited condition?
May-Hegglin Anomaly (asymptomatic) autosomal dominant * large platelets & mild/moderate thrombocytopenia * neutrophils with large Dohle body-like inclusions
39
The provided peripheral blood smear is form a patient with what inherited condition? Inheritance pattern?
**Alder-Reilly** autosomal recessive lysosomal storage diseases - accumulation mucopolysaccharides, glycosphingolipids, or glycoproteins within WBC lysosomes * cytopenia * prominent azurophilic granules in all leukocytes (larger than toxic granules)
40
What are the clinical features or Alder-Reilly Anomaly?
* Coarse facies * cardiac & skeletal abnormalities * developmental delay * hepatosplenomegaly * joint stiffness * corneal clouding * obstructive airway disease

S3B5 Pathology (13 decks)

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