LEUKOCYTE QUALITATIVE ABNORMALITIES - FUNCTIONAL ABNORMALITIES Flashcards
Part 2
what do you mean by ‘reactive’ in reactive lymphocytes
when lymphocytes exhibit morphology consistent with benign etiology
other term of infectious mononucleosis
epstein-barr virus
human herpesvirus 4
what do you mean by ‘abnormal’ in reactive lymphocytes
when lymphocyte morphology suggests malignant or clonal etiology
- smaller size
- indented to lobulated nucleus
- cytoplasmic granules
type I IM
- most common type
- abundant agranular cytoplasm, darker at the periphery, molds around RBCs
- radiating basophilia
type II IM
- ’ immunoblasts’
- fine to course chromatin
- nucleoli
- deeply basophilic cytoplasm
type III IM
also called Turk Irritation cell/ Plasmacytoid lymphocytes
type I IM
- autosomal dominant hyperimmunoglobulin e syndrome
- familial disorder; mutation in STAT3 gene
- random movement of phagocytes are normal, but directional motility is impaired - cells response to chemotactic factors is slow
Job syndrome
clinical triad of job syndrome
- atopic dermatitis
- recurrent skin staphylococcal infection
- recurrent pulmonary infection
- both random and direct movement are impaired
- release of cells from the bone marrow - POOR
- cells fail to respond to inflammatory stimuli but otherwise appear to have normal phagocytic and bactericidal activity
- low grade fever and recurrent infections
lazy leukocyte syndrome
consistent finding in lazy leukocyte syndrome
neutropenia
steps in nitroblue tetrazolium test
- blood
- net incubation (30-60 min)
- formazan production
- centrifugation
- smears from the blood + wright’s stain
- microscopic evaluation
A rare condition (immunodeciency disorder) caused by the decreased ability of neutrophils to undergo respiratory burst after phagocytosis of foreign organisms.
chronic granulomatus disease
principle of NBT dye test
nitro-blue tetrazolium —-> (activated NADPH oxidase) —-> formazan (blue black deposit)
positive result of NBT test
purple-blue
negative result of NBT test
yellow
- alius - grignaschi anomaly
- absence of MPO enzyme from neutrophils and monocytes, but not eosinophils
myeloperoxidase deficiency
- inherited, autosomal recessive trait
- mutation in the CHS1 LYST gene on chromosome 1q42.1-2
- abnormal large lysosomes, which contain fused dysfunctional granules
- the presence of giant granules in neutrophils impairs chemotaxis and killing
chediak - higashi syndrome
- impaired packaging of melanin into giant melanosome granules disturb melanin traffic
- fair skin, pale retina, and light blond frosted or silvery hair
hypopigmentation or partial albinism
impaired release of lytic secretory granules by NK cells, neutrophil defects
severe immunodeficiency
cytoplasmic inclusions, resemblinglarge lysosomes were present in all types of neurons; peripheral and cranial neuropathy
neurologic abnormalities
absent or reduced number and irregular morphology of platelet - dense bodies w/c are required for the second wave of platelet aggregation
mild bleeding tendencies
what is nadph
nicotinamide adenine dinucleotide phophate
abnormality in the lysosome of cell that affects not only wbc but also other cells
chediak-higashi syndrome
3 disorders that have deficiency in enzyme impairment
chronic ganulomatous disease
myeloperoxidase deficiency
chediak-higashi syndrome
decribe the type 1 IM
turk irritation cell
smaller in size
indented to lobulated nucleus
cytoplasmic granules
decribe the type 2 IM
flared ballerina skirt
most common form
abundant agranular cytoplasm
radiant basophilia
decribe the type 3 IM
swiss cheese
immunoblasts
fine to course chromatin
nucleoli
neu in soi
diapedesis
wbc in soi
chemotaxis
signaling molecule in chemotaxis
chemotaxin
what receptor causes random/zigzag movement
cxcr1 and cxcr2
what cell is affected in ebv
b cells
receptor of ebv
cd21
