Module 4
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1
Which WBC fall under granulocytes?
Neutrophils
Eosinophils
Basophils
Eosinophils
Basophils
2
What is the cell lineage for neutrophils? (starting at myeloblast
Myeloblast > promyelocytes > myelocytes > metamyelocytes > bands > neutrophils
3
What are characteristics of myeloblasts?
Small cells (diameter: 14-18µM)
Majority is a large oval nucleus with fine chromatin
2 or more nucleoli
Cytoplasm is basophilic and no granules.
Majority is a large oval nucleus with fine chromatin
2 or more nucleoli
Cytoplasm is basophilic and no granules.
4
What are characteristics of promyelocytes?
Large than myeloblasts
Basophilic cytoplasm with primary (azurophilic) granules.
Golgi zone
Nucleus is finely dispered
Nucleoli may be visible
Basophilic cytoplasm with primary (azurophilic) granules.
Golgi zone
Nucleus is finely dispered
Nucleoli may be visible
5
What are characteristics of myelocytes?
Contain primary AND secondary granules
Nucleus is round
No nucleoli
Chromatin starts to condense
Nucleus is round
No nucleoli
Chromatin starts to condense
6
What are characteristics of metamyelocytes?
Condensed chromatin
Slightly indented nucleus (bean-shaped)
Abundant of granules
(can be seen in PB in infectious or inflammatory states, and in other reactive and neoplastic conditions)
Slightly indented nucleus (bean-shaped)
Abundant of granules
(can be seen in PB in infectious or inflammatory states, and in other reactive and neoplastic conditions)
7
What are characteristics of bands?
Slightly less mature than segmented neutrophils: C shaped
~5-10% of PB
(Increase proportion of band neutrophils can be seen in infectious and inflammatory conditions)
~5-10% of PB
(Increase proportion of band neutrophils can be seen in infectious and inflammatory conditions)
8
What are characteristics of mature granulocytes?
Mature segmented granulocytes with condensed chromatin
Cytoplasm is pale pink with granules
~40-80% of PB are segmented neutrophiols
Cytoplasm is pale pink with granules
~40-80% of PB are segmented neutrophiols
9
What type of cell is this?
Myeloblast
10
What type of cell is this?
Promyelocytes
11
What type of cell is this?
Myelocyte
12
What type of cell is this?
Metamyelocyte
13
What type of cell is this?
Band
14
What type of WBC is this?
Hypersegmented neutrophils
15
what are characteristics in hypersegmented neutrophils?
6 or more nuclear lobes
Seen in megaloblastic anemia due to vitamin b12 or folic acid deficiency
Also seen in myelodysplastic syndromes and rare congenital conditions
Seen in megaloblastic anemia due to vitamin b12 or folic acid deficiency
Also seen in myelodysplastic syndromes and rare congenital conditions
16
What is pelger-huet anomaly?
rare autosomal dominant disorder
"pince-nez" in neutrophils
caused by failure of nuclear segmentation due to a genetic defect
"pince-nez" in neutrophils
caused by failure of nuclear segmentation due to a genetic defect
17
what condition is this?
Pelger-Huet Anomaly
18
Whats the difference between heterzygous and homozygous pelger-heut anomaly?
Heterzygous: pince-nez
Homozygous: singl, round eccentric nucleus with no segmentation
Homozygous: singl, round eccentric nucleus with no segmentation
19
If pelger-huet was aquired, what is it called?
Pseudo-pelger-huet-anomaly
20
In which conditions does Pseudo-pelger-huet-anomaly occur?
Myelodysplastic disorders (ex: MDS, or myelodysplastic syndrome)
Myeloproliferative diseases
Acute and chronic leukemia's
Reactions to drugs, etc
Myeloproliferative diseases
Acute and chronic leukemia's
Reactions to drugs, etc
21
How would you differentiate between congenital Pelger-huet vs psuedo (acquired) pelger-huet?
History and peripheral smear
22
What causes psuedo (acquired) pelger-huet?
Certain drugs like immunosuppressant's
23
How would you differentiate drug-induced VS MDS in PPHA?
Drug-induces: monolobated neutrophils with a single ovoid nucleus
MDS: bi-lobed neutrophils
MDS: bi-lobed neutrophils
24
What is the cause of this PPHA?
MDS
25
What is the cause of this PPHA?
Drug-induces
26
what is may-hegglin anomaly?
autosomal dominant abnormality characterized by large pale basophilic inclusions ("like -Dohle bodies" aka altered DNA)
27
What can be seen in may-hegglin anomaly?
Giant platelets and sometimes thrombocytopenia
Dohle-like bodies
Dohle-like bodies
28
What is the diagnosis for this PB?
may-hegglin anomaly
29
what is chediak-higashi syndrome?
Rare autosomal recessive disorder characterized by partial oculocutanous albinism due to defects in melanin granules and recurrent pyogenic bacterial infections due to abnormalities in granulocytes
30
what is seen in the PBS of chediak-higashi syndrome?
Fusion of lysosomal granules
ALL WBCs contain abnormally giant granules
ALL WBCs contain abnormally giant granules
31
What is the diagnosis of this PBS?
Chediak-Higashi syndrome
32
what is alder-reily anomaly?
rare autosomal recessive disorder. Protein and carb complexes called mucopolysaccharidoses caused by the dificeincy of 1/10 lysosomal enzymes
33
what characteristics are seen in alder-reily anomaly?
Metachromatic and darkly staining inclusions
34
What is the diagnosis for this PBS?
Alder-Reilly anomaly
35
What clinical characteristics are associated with A-R anomaly?
Neurological impairments, vision, hearing, and developmental issues
36
What syndromes are associated with A-R anomaly?
Tay-Sachs disease, Hunter syndrome, hurler syndrome, and maroteaux -lamy polydystrophic dwarfism
37
What are the 2 bone marrow macrophages abnormalities that are due to enzyme deficiency?
Guacher disease and Neimann-Pick Disease
38
What is gaucher disease?
rare genetic disorder characterized by the deposition of glucocerebroside in cells of the macrophage-monocyte system.
39
What deficiency does gaucher disease have?
Acid-glucocerebrosidase
40
what is neimann-pick disease?
group of lysosomal storage diseases that affect metabolism and that are caused by genetic mutations
41
Which enzyme is affects in neimann-pick disease?
Acid sphingomyelinase
42
What type of cells are these?
Gaucher cells
43
What type of cells are these?
Neimann-pick cells
44
What are myloproliferative disorders (neoplasms)?
a series of acquired genetic mutations that take place within the bone marrow compartmnet leading to a proliferation of clones of abnormal stem cells.
45
What are the different types of meyloproliferative neoplasms?
Chronic myeloid Luekemia (CML)
Chronic neutrophilic leukemia (CNL)
Chronic esoinophilic leukemia (CEL)
Essential thromobocythemia
Primary meylofibrosis ---fibroblast
Polycythemia vera
Unclassifiable
Chronic neutrophilic leukemia (CNL)
Chronic esoinophilic leukemia (CEL)
Essential thromobocythemia
Primary meylofibrosis ---fibroblast
Polycythemia vera
Unclassifiable
46
What is seen on the PBS of chronic myeloid leukemia (CML)?
Neutrophilia with prominent left shift
Eosinophilia
Basophilia!!! (especially)
Numerous myelocytes and segmented neutrophils
LEUKOCYTOSIS with blasts, promyelocytes, myelocytes, and metamyelocytes
Eosinophilia
Basophilia!!! (especially)
Numerous myelocytes and segmented neutrophils
LEUKOCYTOSIS with blasts, promyelocytes, myelocytes, and metamyelocytes
47
What type of leukemia is this?
CML
48
What type of leukemia is this?
CMML due to the 3 monocytes
49
What is leukemoid reaction?
a condition where WBCs have some resemblance to leukemia cells.can be mistaken by CML
50
How is Luekemoid reaction characterized by?
High nuetrophil count with myelocytes (immature cells). LAP score is high in leukemoid reaction
51
What does LAP stand for?
Leukocyte alkaline phophatase
52
What is LAP?
an enzyme present in secondary granules of nuetrophils (and not present in eosinophils or basophils)
53
What is LAP used for?
To dinstinguish between leukemoid reaction from CML (score between 0-4)
54
What is PV?
polycythemia vera
55
How is PV characterized?
increase in RBC, granulocytic WBC and platelets with normal morphology
56
What is seen in essential thromobocythemia?
THROMBOCYTOSIS
Increased megakaryocytes in marrow
Increased WBC (occasionally metamyelocytes and myelocytes present on peripheral smear)
Increased megakaryocytes in marrow
Increased WBC (occasionally metamyelocytes and myelocytes present on peripheral smear)
57
What MDS is this?
Essential thrombocythemia
58
Which MDS is this?
PV
59
What is primary meylofibrosis?
Fibrosis of marrow cavity; extramedullary hematopoiesis
60
What is seen in the PBS for primary myelofibrosis?
nRBCs, immature granulocytes, micromegakaryocytes
61
Which MDS is this?
Primary myelofibrosis
62
What are the different types of acyte myelogenous leukemias (FAB)?
M0-M7
63
What does MPO stand for?
meyloperoxidase
64
what is MPO?
an enzyme found in the primary granules of all granuloctyes and monocytes
NOT present in lymphocytes
NOT present in lymphocytes
65
Why is MPO used for?
To differentiate between ALL and AML
66
Which of the myeloid leukemias of FAB are MPO positive?
M1, M2, M3, AND M4