Inherited & Reactive WBC and PLT Disorders Flashcards
Neutrophils in the blood stream can be divided into what categories?
- Circulating Granulocyte Pool (CGP)
- WBC in CBC
- Marginating Granulocytic Pool (MGP)
- move into circulating pool when needed
At what stage do granulocytes enter the blood? How long do they stay there? Where do they go?
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
What situations cause the peripheral blood neutrophil count to increase almost immediately? How?
- infection
- intense exercise
- release of catecholamines
shift from marginating pool to circulating pool & from early release from BM
The peripheral blood total leukocyte count is influenced by what factors?
- 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
What does it mean for a cell to be “marginating”?
adherent to blood vessel walls
What are causes of increased production of myeloid & lymphoid precursors in the bone marrow?
infections
paraneoplastic syndromes
myeloproliferative neoplasms
What are causes of increased release of leukocytes from bone marrow stores?
endotoxemia & infection
hypoxia
trauma/surgery
What substance causes decreased extravasation into tissues?
glucocorticoids
(therefore increases WBC count)
Serious infections cause increase of what specific WBC?
neutrophil & bands (b/c early release form BM)
What pathologic cells are seen in the provided slides? They are indicative of what condition?
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
What is a Leukemoid reaction? I usually includes what 3 components?
severe, persistent, reactive neutrophilic leukocytosis above 50,000/microliter when the cause is something other than leukemia
- leukocytosis
- granulocytic left shift
- thrombocytosis
What other conditions are on the differential along with Leukemoid reaction?
CML & chronic neutrophilic leukemia
What is leukoerythroblastosis? What is the usual cause?
immature myeloid & nucleated erythroid precursors in peripheral blood - variable leukocytosis on CBC
myelophthisic process (bone marrow disruption/infiltration by tumor/fibrosis)
What reactive condition is shown in the peripheral blood smear?
leukoerythroblastosis
immature granulocytes & nucleated RBCs
What non-neoplastic conditions can cause eosinophilia?
- allergic disorders
- drug hypersensitivities
- parasitic infections
- collagen vascular disorders, particularly involving skin
- some vasculitides
- some malignancies
What are the causes of basophilia?
- usually neoplastic - myeloproliferative
- non-neoplastic
- myxedema
- ulcerative colitis
- lymphoma
- hypersensitivity reaction
How are lymphocytes levels different in adult than children/infants?
lymphocyte counts are higher in infants & children
What are the possible causes of monocytosis?
- chronic infections
- inflammatory disorder
- paraneoplastic syndromes (lung cancer)
- recovery from bone marrow suppression
What are the possible causes of lymphocytosis?
- transient stress reaction
- MI
- cardiac arrest
- trauma
- viral infections
- EBV, CMV, Hantavirus
- Bordatella pertussis
- Drug Reaction & cigarettes
- Chronic Infections
- tuberculosis, brucellosis, toxoplasmosis, malaria
What are the non-neoplastic causes of eosinophilia?
- allergic disorder
- drug hypersensitivities
- parasitic
What are the non-neoplastic causes of neutropenia?
drugs
radiation
toxins
intrinsic defects
immune-mediated
hematologic
infectious
What would you expect to see in a bone marrow sample from a patient with proliferation defect resulting in neutropenia?
myeloid hypoplasia
decreased proliferation
What would you expect to see in a bone marrow sample from a patient with a maturation defect resulting in neutropenia?
myeloid hyperplasia
precursors proliferate appropriately but don’t mature properly
What would you expect to see in a bone marrow sample from a patient with a survival defect resulting in neutropenia?
precursors reproduce & mature but are destroyed early in blood (infections/autoimmune)
hyperplasia in bone marrow
What are the potential consequences of severe neutropenia?
opportunistic infections
(more likely when falls below 500)
What are the possible causes of Lymphocytopenia?
- Infectious diseases
- HIV, other viruses
- Erlichiosis
- Iatrogenic (corticosteroids)
- autoimmune
- malnutrition
What are the causes of monocytopenia?
- aplastic anemia
- Hairy cell leukemia
- corticosteroids
- acute infections whith endotoxemia
Plasma cell usually comprise what percent of nucleated cells in marrow?
10%
Is reactive plasmacytosis monoclonal or polyclonal?
polyclonal by definition
monoclonal is neoplastic
Reactive plasmacytosis is seen in what diseases?
usually found along the capillaries
- HIV
- Post-chemo
- cirrhosis
- various solid tumors
- autoimmune disorders
- drug reactions
What blood & bone marrow features do you see with an HIV infection?
reactive plasmacytosis
pancytopenia
lymphocytopenia
granulomas
lymphoid aggregates
What can you stain for to more easily identify plasma cells?
CD138 - they look brown
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)
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
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
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
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
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
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)
What are the clinical features or Alder-Reilly Anomaly?
- Coarse facies
- cardiac & skeletal abnormalities
- developmental delay
- hepatosplenomegaly
- joint stiffness
- corneal clouding
- obstructive airway disease
