Hematology: Leukemia and Non-malignant Leukocyte Flashcards
abnormal uncontrollable proliferation of one or more hematopoietic stem cell in the BM and blood
Leukemia
3 manifestations of leukemia
bleeding
anemia
infection
classification of leukemia that is sudden in onset
acute or chronic?
acute
classification of leukemia that is variable wbc and higher proliferative state
acute
hematopoietic malignancy classification that divides acute leukemia to lymphblastic (L1 to L3) and Myeloblastic (M0 to M7)
FAB Classification
classification that is standard for diagnosis now
WHO
microscopic study of evaluation of chemical constituents within individual cells
Cytochemistry
PAS
Sudan Black
Toluidine blue
Iron stains
(PIS T)
are what classification of cytochemical stains
Non Enzymatic stains
cytochemical stain increased in pregnancy, infections, polycythemia, aplastic anemia
LAP
Is EDTA used in LAP stain? Yes or No.
No. It inhibits reaction.
Normal score of LAP
15-170
strong blue precipitated staining is graded what in LAP score
3+
deep blue with no visible cytoplasm
4+
stain that differentiates AML from ALL
MPO
AML is ___ on MPO.
Positive or negative?
positive
stains primary granules in granulocytes and monocytes
MPO
DIFFERENCE of SBB and MPO
In SBB smears does not have to be fresh
stains specific for granulocytic cells and stains black
Specific Esterase (NASDA)
differentiates monocytic leukemia from granulocytic leukemia
Non-Specific esterase
difference btwn nonspecific and specific esterase
NSE =
monocytes are +
granulocytes are -
SE =
monocytes are -
granulocytes are +
NSE that stains esterase in the monocytes and megakaryocytes
Alpha Naphtyl Acetate Esterase
ANBE NSE stains color _____.
dark red
ANAE NSE stains color ______.
red brown/orange red
PAS stains mycoproteins , glycoproteins and HMW CHONS present in almost all blood cell type except ____.
normoblasts
red purple stain in blocks in cytoplasm
PAS
normoblasts in PAS only stain positive in ______ and _______.
erythroleukemia, thalassemia
discrete purplish to dark red color stain
AP
marker for hairy cell leukemia
TRAP
only _______ is resistant to tartrate.
ACP isoenzyme 5
TdT (Terminal deoxyribonuecleotidyl Transferase) is positive in _____.
Lymphoblasts
Cytochemical stains AML is positive in
- MPO
- SBB
- SE
+ in erythroleukemia
PAS
Specific esterase
Positive cells: _____.
Negative cells: _____.
Positive
GRANULOCYTIC
Negative
MONOCYTIC AND LYMPHOCYTIC
What stains are positive for all cells?
PAS and ACP
pregnancy, infection, aplastic anemia, polycythemia (PIPA)in LAP
INCREASED
CML reaction in LAP
DECREASED
lamin β-receptor gene mutation
Pelger-Huet Anomaly
PHA is inherited. True or False
True. Autosomal Dominant.
the failure of neutrophils to segment (hyposegmentation) with coarseness of chromatin; sometimes also affects other WBCs
Pelger-huet Anomaly
pince-nez spectacle
appearance
Heterozygous PHA
WBC function is normal (asymptomatic)
normal granulation
63-93% of cells affected
PHA or PSEUDO PHA?
PHA
Seen in
MDS, AML, CMPDs, patients
receiving chemotherapy, severe infections, cancer metastizing to the bone, drugs
pseudo pelger-huet anomaly
Entire cell is greatly enlarged and
nuclear material fills the cell with long thin filaments
NEUTROPHILS HYPERSEGMENTATION
NORMAL granulocyte production,
IMPAIRED release;
component of WHIM
found in hereditary NH
Myelokathexis
Autosomal recessive with dense blue black granules in WBC
Alder Reilly Anomaly
Alder Reilly Anomaly is seen in what disease/s
Hunters and Hurlers disease
Maroteaux-Lamy polydystrophic dwarfism
Neutrophilia, Dohle bodies and left shift
toxic granules or alder reilly?
toxic granules
Round projection of nuclear chromatin connected to the neutrophil nucleus by a fine strand of chromatin (drumstick appearance)
nonpathogenic and represents x chromosomes in females
barr bodies
Very large reddish-purple or greenish-gray
granules in all WBCs
cell granules normal in content but abnormally packaged
also a rare and fatal disorder in children
CHEDIAK HIGASHI
albinism, mental retardation, poor resistance to infections
chediak higashi
Remnants of REC containing RNA
Seen in infections, poisoning, burns, following
chemotherapy, pregnancy
Dohle bodies
Autosomal dominant, disordered production of myosin heavy chain. Characterized by:
Leukopenia
Thrombocytopenia
Gray blue spindle shaped inclusions (resemble dohle bodies but larger)
MAY HEGGLIN ANOMALY
Seen in all WBC (granulocytes and monocytes)
Dohle bodies vs May Hegglin
May Hegglin
Seen in all neutrophils
Dohle bodies vs May Hegglin
Dohle bodies
chemokinesis is normal but chemotaxis is not; characterized by boils and furuncles
Job’s syndrome
both chemotaxis and chemokinesis are abnormal
neutropenia, but BM granulocytes are normal
lazy leukocyte
Mutation(s) in NADPH oxidase genes
Sex-linked recessive (disease of male infants) and Autosomal recessive
CHRONIC GRANULOMATOUS DISEASE (CGD)
Phagocytes ingest but can’t kill catalase (+) organisms due to lack of respiratory b
CHRONIC GRANULOMATOUS DISEASE (CGD)
yellow formazan reduced to blue insoluble blue formazan
NBT Test
Bacterial killing is slow but complete
Benign condition, patients rarely troubled by infections
MYELOPEROXIDASE (MPO) DEFICIENCY
aka glycosaminoglycan (GAG) storage diseases
Deficiency of specific enzymes involved in the
degradation of mucopolysaccharides
MUCOPOLYSACCHARIDOSES (MPSS)
SUBSTANCES stored in MPS III (San Filippo)
heparan sulfate only
Deficient enzyme in MPS II-severe
Iduronate sulfatase
Also called Hurler syndrome
MPS I-severe
MPS I deficient enzyme
a-l-iduronidase
gargoylism
Metachromatic Reilly bodies
diagnose by use of assays
MPS
Result from the lack of a functional enzyme
required for breakdown of lipids that have been
ingested by phagocytes
LIPIDOSES
GAUCHER DISEASE TYPE II IS AUTOSOMAL DOMINANT. TRUE OR FALSE.
TRUE. only lipidoses that is not autosomal recessive.
Deficiency in β-glucocerebrosidase
hepatomegaly, Gaucher cells in BM, markedly elevated serum ACP
GAUCHER’S DISEASE
Diagnostic biomarker of Gaucher’s disease
chitotriosidase
juvenile form; life expectancy is short of Gaucher’s disease
Type III
infantile or cerebral form; neurologic deterioration, death within first few years of life type of Gaucher’s
Type II
Crumpled tissue paper or cigarette butt cell
Fibrillar blue-grey cytoplasm with striated or wrinkled appearance (onion skin-like)
Cells strongly PAS (+)
gaucher’s cell
Deficiency in α-sphingomylinase
Most prevalent form develops in infancy and
resembles type II Gaucher disease
NIEMANN-PICK DISEASE
Also foam or bubble cell
macrophages with lipid filled lysosomes that appear as vacuole
NIEMANN-PICK CELL
Deficiency in Hexominidase A
High incidence in Ashkenazi, Jewish population
Vacuolated and foamy lymphocytes
TAY-SACHS DISEASE
Found in association with ↑ tissue stores of
phospholipids/glycolipids
Accumulation of histiocytes filled with lipid-rich
granules in the spleen and BM
Hepatosplenomegaly and thrombocytopenia
SEA-BLUE HISTIOCYTOSIS
Defective development of thymus, failure of T
cell development
NEZELOF’S SYNDROME
Almost indistinguishable from Nezelof’s
Not of genetic origin, cause is unknown
DIGEORGE’S SYNDROME
ALL classes of Ig are either absent or extremely low
Absent B cells, T cells normal
Prone to bacterial infections
INFANTILE SEX-LINKED (BRUTON) AGAMMAGLOBULINEMIA
One or combination of Ig is either missing entirely or synthesized only in small quantities
COMMON VARIABLE HYPOGAMMAGLOBULINEMIA
Depletions of T, B and NK cells
SCID
Migration, adhesion, and activation of T-cells, B
cells and NK cells are affected
SEX LINKED
WISKOTT-ALDRICH SYNDROME
Increased LAP score with rare blasts
normal platelets
NLR or CML?
NLR
Presence of immature neutrophils, nucleated RBCs and teardrop RBCs
Commonly associated with myelophthisis
and primary myelofibrosis
LEUKOERYTHROBLASTIC REACTION
Mechanism: Increase outflow from BM and shift from marginal pool to circulating pool
NEUTROPHILIA
circuLating = neutrophiLia
mild left shift
band and metamyelocyte
MARKED left shift
blast
seen in
Bacterial infection – TB, SBE, syphilis
monocytosis
Stem cell disorders – aplastic anemia
monocytopenia
definitive test for IM
EBNA
Indicative of depressed locomotion
Caused by bacterial and therapeutic agents
pseudopods
what type of neutrophil nuclear alteration is Metastatic carcinoma or after irradiation
Downey classification Type II size
15-25 um
irregular or scalloped type of downey’s class
Type II
fine chromatin; highly visible nucleoli type of downey’s class
Type III