Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

CSMLS > HEM Leukocyte Disorders > Flashcards

HEM Leukocyte Disorders Flashcards

1
Q

How to differentiate true vs Pseudo Pelger-Huet

A

Pelger-Huet = no clinical significance
Pseudo = disorders (MDS, AML, plasma cell myeloma, drugs) will have other affected characteristics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

PBS of Pelger-Huet

A

Hypo-lobed neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Clinical significance of Pelger-Huet

A

Normal cell function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Cause of Alder-Reilly

A

Recessive allele = lacks enzymes to breakdown mucopolysaccharide for storage = mucopolysaccharide accumulation in cytoplasm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

PBS of Alder-Reilly Anomaly

A
  • large pink granules in neutrophils
  • can be mistaken for toxicity but NO VACUOLES or NO DOHLE BODIES
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Cause of Chediak Higashi

A

LYST gene mutation = abnormal Lysosomal Trafficking (transport of material to lysosomes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Clinical Significance of Chediak Higashi

A
  • Defective de-granulation
  • Diminished delivery of enzymes to phagosomes
  • Abnormal chemotaxis
  • Short life expectancy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

PBS of Chediak Higashi

A
  • “bubbly” granules = aggregation of primary + secondary granules
  • NEUTROPENIA, THROMBOCYTOPENIA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Cause of May-Hegglin Anomaly

A

MYH9 gene mutation (Myosin-heavy chain-9) = abnormal cytoskeletal proteins in PLATELETS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

PBS of May-Hegglin Anomaly

A
  • Long BLUE inclusions in ALL GRANULOCYTES
  • THROMBOCYTOPENIA
  • GIANT PLTs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Cause of Chronic Granulomatous Disease

A

Mutation in gene responsible for producing NADPH oxidase complex = Neutrophils cannot produce super-oxides (anti-microbial)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Additional testing for Chronic Granulomatous Disease

A
  • Nitro-blue-tetrazolium reduction test
  • Flow cytometry
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Cause of Infectious Mononucleosis

A

Infection of B-lymphocytes by EPSTEIN-BARR VIRUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

PBS of Infectious Mononucleosis

A
  • WBC increased
  • Reactive lymphocytes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Additional testing for Infectious Mononucleosis

A
  • Monospot POSITIVE
  • Immunological testing = EBV specific antigens
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which non-malignant disorder may develop an anti-i/ cold agglutinin syndrome ?

A

Infectious mononucleosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Leukemia is a __ condition involving __ tissue.

A

Leukemia is a MALIGNANT condition involving HEMATOPOIETIC tissue.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Acute Leukemia characteristics

A
  • rapid onset
    >20% blasts
  • affects all ages
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

CBCD in Acute Leukemia

A
  • N/N anemia (RBC decreased)
  • WBC increased
  • THROMBOCYTOPENIA
  • NEUTROPENIA
    >20% blasts
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Testing for Acute leukemia

A
  • PBS and BM morph
  • Immunophenotyping (Flow cytometry)
  • Special cytochemical stains
  • Enzyme markers
  • Cytogenetics
  • Molecular genetics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Differentiate myeloid vs lymphoid blasts

A

Myeloid:
- larger (more cytoplasm)
+/- auer rods
- azurophilic granules
- lacier chromatin

Lymphoid:
- smaller than myeloblasts
- scant cytoplasm
- less lacy chromatin
- rare granules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

A positive Myeloperoxidase stain indicates __ BUT what does a negative result imply ?

A

A positive Myeloperoxidase stain indicates MYELOblasts BUT a negative result cannot exclude myeloid lineage (too young to produce MPO)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Which cells produce Myeloperoxidase ?

A

Granulocytes and Monocytes (primary granules)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Sudan black stains which cells positive ?

A

Myeloid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
alpha-Napthyl Esterate stains which cells ? What is an interference ?
- NSE pos = Monocytes > megakaryocytes - Sodium fluoride is an interference
26
TdT (enzyme marker) is detected via __ in leukemic __ cells, but rarely in __.
TdT (enzyme marker) is detected via FLOW CYTOMETRY in leukemic LYMPHOID cells, but rarely in AML
27
PAS stains which cells ?
Lymphoblasts = BLOCK POSITIVE Acute erythroleukemia (AEL), acute megakaryocytic leukemia (AMegL), acute lymphocytic leukemia (CLL) = POS
28
ALL can be further classified based on the origin of the cell. What are they?
B cell or T cell
29
How to differentiate B cell vs T cell ALL ?
FLOW CYTOMETRY/ Immunohistochemistry B cell: CD34, 10, 19, 20, 22, TdT+ T cell: CD1, 2, 3, 4, 5, 7, 8, TdT+
30
AML involves which cells ?
Granulocytes, monocytes, erythrocytes, and megakaryocytes (myeloids)
31
PBS/BM of APL
Acute promyelocytic leukemia: - increased promyelocytes - bundles of auer rods - thrombocytopenia (associated with DIC)
32
APL stain results (MPO, SBB)
MPO pos SBB pos
33
PBS/ BM of CML (chronic phase)
- **normo/normo anemia (decreased RBC)** - increased WBCs, **left shift** (myeloid) - increased PLTs - nRBC - increased EOS and BASOS - **blasts <2%**
34
PBS/ BM of CML as it progresses from chronic>accelerated>blast phase
Accelerated: - anemia worsens (decreased RBC) - WBC and PLTs increase - BASOS increase - blasts = 10-19% Chronic: - **BLASTS >20%**
35
3 stages of CML
chronic > accelerated > blast
36
LAP result for CML
negative; LAP enzyme is only detected in active neutrophils (leukemoid reaction)
37
Chronic Leukemia is classified based on __ and __
Chronic Leukemia is classified based on AFFECTED CELL LINE and GENETIC ABNORMALITIES
38
3 categories of Chronic Leukemia
1. Mature B-cell Neoplasms (CLL, hairy cell, MM, Hodgkins) 2. Myeloproliferative neoplasms (CML, PV, primary myelofibrosis, ET) 3. Myelodysplastic syndromes (MDS)
39
T or F: CLL does not terminate into an acute leukemia
TRUE; CLL does NOT terminate into an acute leukemia. - patients often die due to other complications/ infections
40
Which cells do CLL affect ?
B-cells/ plasma cells
41
Hairy cell leukemia is a subtype of __.
Hairy cell leukemia is a subtype of CLL
42
Additional testing for Hairy cell leukemia
TRAP pos Immunophenotyping = CD20, increased side scatter; co-expression of CD103 & CD22 TRAP = tartrate resistant acid phosphatase stain
43
Immunophenotyping results for Hairy cell leukemia
- Increased C20 - Co-expression of CD103 AND CD11c
44
Plasma cell neoplasms are a subtype of __.
Plasma cell neoplasms are a subtype of MATURE B-CELL NEOPLASMS
45
What is secreted by plasma cell neoplasms ?
- Immunoglobulins/ paraproteins - aka M-proteins
46
List 3 conditions under Plasma cell neoplasms
1. Monoclonal gammopathy of undetermined significance 2. Plasma cell myeloma (Multiple myeloma) 3. Waldenström macroglobulinemia
47
How to differentiate Waldenstrom’s macroglobulinemia vs Plasma cell myeloma
**Waldenstrom’s macroglobulinemia:** - serum electrophoresis = **IgM** - asymptomatic **Plasma cell myeloma:** - serum electrophoresis = **IgG or IgA** - **CRAB** symptoms (increased Ca2+) CRAB = calcium elevation, renal damage, anemia, bone lesions
48
Testing for Hodgkin’s lymphomas
**Reed-sternberg cells in lymph node biopsies** (rarely in PBS) NOTE: cells resembles “Owl-eyes”
49
What are Myeloproliferative neoplasms ?
- clonal disorders affecting MYELOID lineage - commonly in older adults - genetic stem-cell mutation = uncontrolled cell proliferation
50
What does uncontrolled tyrosine kinase activity lead to ?
- increased CELL PROLIFERATION/ CELL CYCLES - inability to perform apoptosis when they should
51
Cause of CML
- mutation between chromosome 9 and 22 = BCR-ABL fusion gene - forms Philadelphia chromosome - BCR-ABL protein increases tyrosine kinase activity in granulocytes
52
Cause of PV
Polycythemia Vera: - radiation/ toxin exposure = point mutation in JAK2 gene - valine amino acid replaced by PHENYLALANINE = **JAK2 V617F mutation**
53
Pathophysiology of PV
- JAK2 V617F mutation = **JAK2 kinase** continuously triggers EPO receptors WITHOUT EPO - **increased proliferation of RBCs INDEPENDENT OF EPO** - patients develop anemia, BM fibrosis, and splenomegaly
54
PBS/ BM of PV
PBS: - **INCREASED RBCs** - normo/ normo - increased PLTs and WBCS BM: - normal M:E - fibrotic over time - **decreased EPO and iron stores**
55
Testing for PV
Molecular genetics: JAK2 V617F mutation
56
How to differentiate PV, secondary PV, and relative PV
**PV:** *increased RBCs, WBCs and PLTs* with *decreased EPO* **Secondary PV:** *increased RBCs AND EPO,* but *normal WBC and PLTs* **Relative:** *increased RELATIVE RBCs* (less plasma volume = more concentrated), BUT *normal EPO, WBCs and PLTs* NOTE: all will have HIGH HEMATOCRIT and HEMOGLOBIN
57
Cause of Primary Myelofibrosis
- **Various genetic mutations** including **JAK2 V617F** - overproduction of HSC = increased proliferation of myeloid lines
58
PBS/ BM of Initial phase in Primary Myelofibrosis
PBS: - increased WBCs and PLTs - normo/ normo anemia (decreased RBCs) BM: - hypercellular; increased M:E
59
Which Myeloproliferative neoplasm may progress into hypo/ micro over time ?
Polycythemia Vera
60
PBS/ BM of Fibrotic phase in Primary Myelofibrosis
PBS: - normo/ normo anemia (decreased RBCs) - DECREASED WBCs and PLTs (large/ giant) - LEUKO ERYTHRO BLASTIC = myeloprecursors + nRBC + blasts - poikilocytosis (ELLIPTO + TEARS) BM: - dry tap - DECREASED WBCs and PLTs
61
Additional testing for Primary Myelofibrosis
- Trephine biopsy due to dry tap - Molecular genetics = multiple mutations
62
Which cells are affected in Essential Thrombocythemia ?
MEGAKARYOCYTES and PLATELETS
63
Cause of ET
Essential Thrombocythemia: - multiple mutations including JAK2, MPL, CALR = abnormal platelets (function + morph)
64
PBS/ BM of Essential thrombocythemia
PBS: - INCREASED and ABNORMAL PLATELETS (>600) - normo/ normo anemia (decreased RBCs) - megakaryocyte fragments BM: - INCREASED and ABNORMAL MEGAKARYOCYTES - decreased iron stores
65
Additional testing for ET
Essential Thrombocythemia: - Molecular genetics = multiple mutations including JAK2, MPL, CAR
66
MDS can progress into __.
MDS can progress into AML.
67
Abnormal findings of granulocytes in MDS
PBS: - decreased WBCs (being destroyed in BM) - hypolobed neuts - hypo/hypergranulated neuts BM: - karryohexis (mitotic figures) - <20% blasts
68
Abnormal findings of erythoids in MDS
PBS: - normo/normo OR dimorphic anemia (decreased RBCs) - POIKILOCYTOSIS (BASOPHILIC STIPPLING, OVAL MACROCYTES, tears, schistocytes, howell jolly bodies, acanthocytes, spherocytes, elliptocytes) - nRBCs
69
Abnormal findings of megakaryocytes in MDS
PBS: - GIANT PLTs - PLT clumps - hypogranular PLTs - megakaryocytic fragments BM: <20% blasts - hypercellular; increased M:E
70
PBS/ BM in Mature B-cell neoplasm (CLL)
PBS: - increased SMALL LYMPHS/ smudge cells; neutropenia - normo/ normo anemia (decreased RBCs) BM: - increased small lymphs; >30% - decreased granulocytes, erythrocytes
71
PBS/ BM of Hairy cell leukemia
PBS: - increased hairy cells (SMALL LYMPHS) - normo/ normo anemia (decreased RBCs) - decreased PLTs (due to fibrosis) BM: - dry tap due to fibrosis - decreased PLTs
72
PBS/ BM of Plasma cell myeloma/ multiple myeloma
PBS: - normo/ normo anemia (decreased RBCs) - DECREASED WBCs (neutropenia), PLTs, retics - plasma cells - rouleaux BM: - plasma/ flame/ mott cells - ostolytic lesions - hypercellular
73
PBS/ BM of Waldenstrom’s macroglobinemia
PBS: - normo/ normo anemia (decreased RBCs) - DECREASED WBCs (neutropenia), PLTs, retics - rouleaux BM: - plasma cytoid lymphs (resemble plasma cells WITHOUT halos)
74
Common test results for Plasma cell neoplasms (Plasma cell myeloma and Waldenstrom’s macroglobulinemia)
ESR increased PT and PTT prolonged Bence jones proteins in urine
75
Additional testing for CML
LAP stain = neg Cytogenetics = Philadelphia chromosome (chromosome 9 to 22 translation) Molecular genetics = BCR-ABL fusion gene
76
What causes the spherocytes seen in CLL ?
AIHA secondary to CLL; autoAb coat RBCs and are spliced by macrophages in the spleen
77
Why may a leukomoid reaction be mistaken with CML ?
Leukomoid reaction: - increased NEUTROPHILS due to infections, hemorrhage, hemolysis CML: - ALL MYELOIDS INCREASED
78
How to differentiate Leukemoid reaction from CML
Leukomoid reaction: - LAP stain POS - NEUTROPHILS increased CML: - LAP stain neg - ALL MYELOIDS INCREASED - Molecular genetics = BCR-ABL fusion gene - Cytogenetics = Philadelphia chromosome (9 to 22 translation)
79
Why may platelet satellite be observed in blood samples of healthy individuals ?
- EDTA chelates calcium - this exposes Ag on RBCs - autoAb binds to GP2 B3A Fc receptors - platelets satellite around RBCs
80
Which statement is correct with respect to toxic granulation? a. It appears in lymphocytes in response to inflammation b. It is associated with chronic myelogenous leukemia c. It is usually seen along with a 'left shift' d. It is similar to inclusions seen in Chediak-Higashi
c. It is usually seen along with a 'left shift'
81
Which of the following statements about left shift is FALSE? a. Bands and myeloids are present in the peripheral blood. b. It can be caused by a malignant or non-malignant process c. It often accompanies a high WBC count d. It affects the lymphocytic cell line
d. It affects the lymphocytic cell line
82
An absolute lymphocytosis with reactive lymphocytes suggests which of the following conditions? a. Bacterial infection b. Viral infection c. Parasitic infection d. Acute leukemia
b. Viral infection
83
Which of the following is NOT a cause of eosinophilia? a. Myeloproliferative neoplasms b. Helminth infection c. Malarial infection d. Bacterial infections
d. Bacterial infections
84
The following peripheral blood abnormalities are diagnostic clues in MDS EXCEPT: a. Oval macrocytes b. Target cells c. Agranular neutrophils d. Elliptocytes
b. Target cells
85
What are the abnormal inclusions in Chédiak-Higashi anomaly composed of? a. Ribosomal material that looks like dohle-like bodies b. Excess lipids that cannot be broken down c. fused granules that inhibit bactericidal functions d. Excess mucopolysaccharides that disrupt cell function
c. fused granules that inhibit bactericidal functions
86
Which of the following statements is true regarding infectious mono? a. Patient will have relative and absolute lymphocytosis b. The bone marrow will show a decreased M:E ratio c. The WBC differential will show a left shift d. B-lymphocytes will demonstrate characteristic reactive morphology
a. Patient will have relative and absolute lymphocytosis NOTE: T cells become reactive lymphs
87
Which of the following is a feature of secondary polycythemia? a. Increased oxygen saturation b. Decreased plasma volume c. Increased erythropoietin d. Normal hematocrit
c. Increased erythropoietin
88
Which of the following cytoplasmic inclusion is composed of RNA? a. May-Hegglin inclusions b. Alder-Reilly inclusions c. Heinz bodies d. Dohle bodies
d. Dohle bodies

CSMLS (46 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions