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 non-neoplastic causes of eosinophilia?
- allergic disorder
- drug hypersensitivities
- parasitic
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
Reactive plasmacytosis is seen in what diseases?
usually found along the capillaries
- HIV
- Post-chemo
- cirrhosis
- various solid tumors
- autoimmune disorders
- drug reactions
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)
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)
