Leukocyte Disorders Flashcards
what is the normal relative neutrophil count?
50-70%
determined the number of segmented and band neutrophils
absolute neutrophil count
nonpathologic causes of neutrophilia
strenuous exercise, emotional stress, shock, burns, trauma, labor, pregnancy, increase in epinephrine
reactive leukocytosis above 50x10^9/L with neutrophilia and a marked left shift (presence of immature forms)
leukemoid reaction
what is neutrophilic leukemoid reaction may be cocnfused as?
chronic myelogenous leukemia
presence of immature neutrophils, nucleated red blood cells. and teardrop RBCs in the same sample. may be accompanied by neutrophilia but not always
leukoerythroblastic reaction
what does leukoerythroblastic reaction point to?
space-occupying lesion in the bone marrow
sever form of neutropenia
agranulocytosis
maternal IgG crosses the placenta and binds to neutrophil-specific antigens inherited from the father
alloimmune neonatal neutropenia
a primary illness in children in which moderate to severe neutropenia develops as a result of antibodies to HNA-1
autoimmune neutropenia
autosomal recessive disorder characterized by marrow failure, pancreatic insufficiency, and skeletal abnormalities. intermittent neutropenia that fluctuates from severely low to near normal
Shwachman-Diamond syndrome
autosomal recessive disease characterized by severe neutropenia that presents shortly after birth and bone marrow granulocyte hypoplasia with maturation arrest at promyelocyte stage
Kostmann syndrome
gene that codes for neutrophil elastase
ELANE / ELA2
approximately 50% of px have mutations in ELANE/ELA2 and have periods of severe neutropenia every 21 days
cyclic neutropenia
shows more immature neutrophils than mature neutrophils, suggesting that cells are lost during maturation
chronic idiopathic neutropenia
autosomal recessive or x-linked inherited disease characterized by variable degrees of bone marrow failure, peripheral cytopenias, and increased risk for hematologic malignancies and other cancers
fanconi anemia
sex-linked recessive, autosomal dominant or autosomal recessively inherited disorder with a heterogenous presentation. patients have mucocutaneous abnormalities, abnormal skin pigmentation, nail dystrophy, and leukoplakia, bone marrow failure
dyskeratosis congenita
nonmalignant eosinophilia is generally caused by
cytokine stimulation especially from IL3 and IL5
major function of eosinophil wherein substances are released that damage and offending organism or target cell
degranulation
px with >1.5x10^9/L lasting more than 6 months without any identifiable cause, what is the diagnosis
hypereosinophilic syndrome
accompanied by neutrophilia during infection or inflammation
eosinophilia
common cause of basophilia
presence of malignant myeloproliferative neoplasm
frequently first sign of recovery from acute overwhelming infection or severe neutropenia (most common after cancer chemo) which is positive
monocytosis
absolute monocyte count of <0.2x10^9/L
monocytopenia
found in patients receiving steroid therapy or hemodialysis or in sepsis. epstein-barr virus infected px
monocytopenia
reference range for relative lymphocytes
20-40%
an inner nuclear membrane protein that combines beta-type lamins and heterochromatin and plays a major role in leukocyte nuclear shape changes
lamin beta-receptor gene
autosomal dominant disorder characterized by decreased nuclear segmentation and coarse chromatin clumping pattern
pelger-huet anomaly
where is neutrophil hypersegmentation most often associated with?
megaloblastic anemia
it can be seen in myelodysplastic syndromes and represent a form of myeloid dysplasia.
neutrophil hypersegmentation
in this disorder, neutrophil hypersegmentation can be seen but px shows no sign of megaloblastic anemia
hereditary neutrophil hypersegmentation
hereditary condition characterized by normal granulocyte production but with impaired release into circulation that leads to neutropenia
myelokathexis
neutrophils appear hypermature, hypersegmented, hypercondensed chromatin, and pyknotic changes. cytoplasmic vacuoles may also be observed
myelokathexis
a syndrome in which warts, neutropenia, hypogammaglobinemia, infections, and myelokathexis are common findings
WHIM syndrome
incomplete degradation of mucopolysaccharides
alder-reilly anomaly
neutrophil that has a normal size 4-6 lobes in the nucleus found in the stage of recovery from infection
polycyte
Larger than normal neutrophil and has 5-10 nuclear lobes
macropolycyte
where is macropolycyte found?
pernicious anemia
Nucleus of the neutrophil becomes smaller and denser; nuclear segments disappear, leaving several balls of dense chromatin.
pyknocyte
Cell has a chromatin arrangement which
gives the cell a “moth-eaten” or “tunneled” appearance or “swiss-cheese”. Cell has prominent azurophilic granules
virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell
It looks like a sunny side up egg. activated to respond to a viral infection. Or it can also respond to bacterial or parasitic infection.
virocyte/ atypical lymphocyte/ downey type cell/ turk irritation cell
Myeloblast that is characterized by having a nucleus with deep indentations often suggesting lobulations
rieder cell
where is rieder cell commonly seen in?
acute myeloid leukemia
Cell with holes or vacuoles in the cytoplasm. Signs of degeneration in severe infections, chemical poisoning and leukemia
vacuolated cell
Net-like nucleus from a ruptured white cell especially a PMN
basket cell/ smudge cell
where is basket cell/ smudge cell found?
chronic lymphocytic leukemia
the cause of this cell could be due to CLL or maybe the smear made is not good
basket cell/ smudge cell
A PMN which had engulfed the nuclear material of another PMN or a lymphocyte
lupus erythematous cell
There’s an ingestion or engulfment. Neutrophils or Macrophage that has phagocytized the denatured nuclear materials of other cells.
lupus erythematous cell
where is lupus erythematous cell found?
systemic lupus erythematous
A monocyte with an engulfed nucleus usually of a lymphocyte or maybe the whole lymphocyte itself
tart cell
what does tart cell exhibit?
necleophagocytosis
Lymphocytes with hair like cytoplasmic projections surrounding the nucleus
hairy cell
where is hairy cell found?
hairy cell leukemia
Rough lymph cell with nucleus that is grooved or convoluted
sezary cell
where is sezary cell found?
sezary syndrome, mycosis fungoides
Are linear or spindle-shaped red-purple inclusions in myeloblasts and monoblasts
auer bodies/ rods
derivatives of azurophilic granules
auer bodies/ rods
Caused by unusual development of lysosomes
auer bodies/ rods
cytoplasmic inclusion which result from abnormal fusion of primary azurophilic granules
auer bodies/ rods
what is the classification of auer rods?
pathological
These are red-staining needle-like bodies seen in the cytoplasm of either the myeloblast or monoblast.
auer bodies/ rods
Dark blue to purple cytoplasmic granules in the metamyelocyte, band or in neutrophil stage
toxic granules
Remnants of free ribosomes from an earlier stage of development
double-amato bodies
cytoplasmic inclusion mostly seen in bacterial infections, severe burns, exposure to cytotoxic agents and complicated pregnancies
double-amato bodies
Found in the cytoplasm of multiple myeloma and plasma cells
after therapy with amidine drugs. Has an intracytoplasmic
spherical shape
snapper-scheid bodies
Gamma globulins bodies in the cytoplasm of plasma cells and inflamed tissue
russell/ fuch’s bodies
Bodies which gave a grape or berry or morula cell appearance
russell/ fuch’s bodies
Has large peroxidase lysosomes inclusions that are deficient in enzymes for phagocytosis
Chediak-Higashi Syndrome
associated condition for chediak-higashi syndrome
albinism
Dohle bodies, thrombocytop enia,giant platelets and leukopenia
may-hegglin anomaly
vacuolization of leukocytes
Jordan’s Anomaly
Peroxidase depletion in PMN and monocytes
Alius–Grignaschi Anomaly
Random movement of phagocytes is normal, but directional motility is impaired
job’s syndrome
both random and directed movement of the cells are defective
lazy leukocyte syndrome
intracellular killing mechanism of granulocyte is defective. disease usually seen in childhood
Chronic Granulomatous Disease
phagocytes ingest but can’t kill catalase + organisms because of lack of appropriate respiratory burst
Chronic Granulomatous Disease
Asymptomatic carriers have half the normal C3 activity (heterozygous)
Congenital C₃ Deficiency
carriers fail to opsonize bacteria
Congenital C₃ Deficiency
MPO is decreased or absent in PMN and monocyte
Myeloperoxidase Deficiency
Deficiency of glucocerebrosidase enzyme
Gaucher’s Disease
deficiency in sphingomyelinase
Niemann-Pick Disease
macrophage with cholesterol overload due to increase in foam cells
Schuller-Christian Disease
This is the most common of the lysosomal lipid storage
Gaucher’s Disease
deficiency in hexosaminidase A
Tay-Sachs Disease
reduced Ig production in blood. associated with B-cell deficiency
Bruton Agammaglobulinemia
reduced production of Ig due to overactivity of T8 cells
common variable hypogamaglobulinemia
underdevelopment of the thymus; t-cell deficiency
Nezelof’s Syndrome
deletion of a small piece of chromosome 22
DiGeorge’s syndrome
Loss of both T cell and B Cells function
Swiss-Type Agammaglobulinemia
Failure of T-cell response Only IgA and IgG are present; IgM
is absent
Wiskott-Aldrich Syndrome
decreased T cell production; Characterized as having progressive loss of muscular coordination
ataxia telangiectasia
inherited leukocyte disorder caused by a mutation in the lamin B receptor
Pelger-Huet anomaly
inherited leukocyte disorder in which it is one of a group of disorders with mutations in nonmuscle myosin heavy-chain IIA?
May-Hegglin anomaly
What inherited leukocyte disorders might be seen in Hurler syndrome?
Alder-Reilly anomaly
lysosomal storage disease is characterized by macrophages with striated cytoplasm and storage of glucocerobroside
gaucher disease
the neutrophils in chronic granulomatous disease are incapable of producing:
hydrogen peroxide, hypochlorite, superoxide
individuals with X-linked SCID have a mutation that affects their ability to synthesize:
IL-2 receptor
an absolute lymphocytosis with reactive lymphocytes suggests what condition?
viral infection
what leukocyte cytoplasmic inclusion is composed of ribosomal RNA?
Dohle bodies
