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Hematology > Leukocyte Disorders > Flashcards

Leukocyte Disorders Flashcards

1
Q

Pelger-Huet Anomaly presentation

A

hypo-lobated nucleus in neutrophils

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2
Q

what causes Pelger-Huet Anomaly

A

a mutation causing decreased expression of the lamin-beta receptor

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3
Q

what is the lamin-beta receptor

A

a protein in the inner nuclear membrane

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4
Q

what does a decrease in expression of the lamin-beta receptor do

A

affects chromatin distribution and nuclear shape

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5
Q

how is Pelger-Huet anomaly inherited

A

autosomal dominant

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6
Q

does Pelger-Huet anomoly effect cell function

A

no

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7
Q

in what conditions can you see pseudo Pelger-Huet

A

MDS, AML, CML multiple myeloma, drugs and chronic infections

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8
Q

Alder-Reilly anomaly presentation

A

large pink granules in neutrophils and lymphocytes

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9
Q

what is the cause of Alder-Reilly anomaly

A

cells are unable to breakdown mucopolysaccharides due to a lack of enzymes

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10
Q

what does Alder-Reilly anomaly cause

A

altered WBC function, severe mental retardation, dwarism, organomegaly and skeletal deformities

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11
Q

what is the inheritance of alder-reilly

A

recessive

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12
Q

what is the inheritance of Chediak-Higashi

A

autosomal recessive with a LYST gene mutation

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13
Q

what does Chediak-Higashi cause

A

aggregation of primary and secondary granules forming large abnormal lysosomes with defective degranulation and diminished delivery of enzymes to phagosomes

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14
Q

what is treatment for Chediak-Higashi

A

antibiotics and high doses of ascorbic acid

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15
Q

what other types of cells are effected by Chediak-Higashi

A

platelets and melanocytes

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16
Q

presentation of May-Hegglin anomaly

A

large blue inclusions in all granulocytes that resembled dohle bodies

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17
Q

what is the inheritance of May-Hegglin anomaly

A

autosomal dominant

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18
Q

what do you typically see in May-Hegglin anomaly

A

thrombocytopenia and giant platelets

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19
Q

what causes the May-Hegglin anomaly

A

a mutation in MYH9 gene that encodes cytoskeletal proteins in platelets

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20
Q

what does the May-Hegglin anomaly effect

A

usually asymptomatic but sometimes have bleeding issues

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21
Q

what is chronic granulomatous disease

A

a neutrophil disorder that is unable to initiate respiratory burst and produce superoxides

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22
Q

what is the inheritance of CGD

A

usually x-linked but also autosomal recessive

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23
Q

what is the cause of CGD

A

mutations in genes responsible for producing NADPH oxidase complex

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24
Q

what does CGD cause

A

reoccuring fungal and bacterial infections in lungs, deep tissues, skin, lymph nodes and liver

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25
Q

how id CGD diagnosed

A

testing of neutrophils using nitro-blue-tetrazolium reduction tests or glow cytometry

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26
Q

what causes infectious mononucleosis

A

epstein-barr virus

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27
Q

how does infectious mononucleosis present

A

elevated WBC, absolute lymphocytosis, reactive lymph

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28
Q

what can infectious mononucleosis lead to

A

cold agglutin syndrome

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29
Q

what are possible causes of leukemia

A

radiation, chemotherapy drugs, chemicals and drugs, viruses

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30
Q

what conditions can leukemia occur secondary to

A

hematological diseases, genetic predispositions, immunodeficiencies

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31
Q

what characterizes an acute leukemia

A

sudden onset of symptoms, >20% blasts in PBS

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32
Q

what are the general lab findings in the PBS of acute leukemia

A

anemia, thrombocytopenia, neutropenia, >20% blasts

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33
Q

what are the general lab findings in the BM of acute leukemia

A

high M:E ratio, infiltration of blasts

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34
Q

what are the characteristics of myeloid blasts

A

large with moderate dark blue/grey cytoplasm, azurophilic granules, chromatin is fine and lacy, numerous round or oval nucleoli, may contain auer rods

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35
Q

what are the characteristics of lymphoid blasts

A

smaller than myeloblasts but larger than mature lymphs, scant cytoplasm, chromatin is denser, scant nucleoli, usually no granules

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36
Q

what does myeloperoxidase stain

A

primary granules of granulocytes and monocytes

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37
Q

what does sudan black stain

A

cellular lipids (more sensitive for early myeloids)

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38
Q

what does NSE stain

A

monocytes and megakaryocytes

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39
Q

what does PAS stain

A

glycogen inside the cytoplasm of the cell (lymphoblasts will be strongly positive)

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40
Q

what is TdT

A

a DNA polymerase present in stem cells and early lymphoid cells

41
Q

what are the CD markers in B cell lymphoblastic leukemia

A

34, 10, 19, 20, 22, 24, 79a, TdT

42
Q

what are the CD markers in T cell lymphoblastic leukemia

A

1, 2, 3, 4, 5, 7, 8, TdT

43
Q

What are the general conditions for AML

A

blasts of myeloid lineage with >20% blasts, neutropenia, thrombocytopenia and anemia

44
Q

What are the lab findings for ALL

A

blasts
increased WBC
decreased PLT
normo/normo anemia
neutropenia

45
Q

What are confirmatory tests for ALL

A

MPO negative
PAS block positive

46
Q

What are lab findings for AML

A

blasts with auer rods
increased WBC
decreased PLT
normo/normo anemia
neutropenia

46
Q

What are confirmation tests for AML

A

MPO positive
SBB positive
NSE positive

47
Q

What are lab findings for APL

A

hypergranulated promyelocytes
faggot cells
bilobed nucleus

48
Q

What are confirmatory tests for APL

A

MPO positive
SBB positive

49
Q

What are lab findings for AMML

A

neutrophils and monocytes
20%+ blasts
decreased PLT
neutropenia
normo/normo anemia
increased monocytes in PBS

50
Q

What are confirmatory tests for AMML

A

MPO positive
SBB positive
NSE positive

51
Q

What are lab findings for AMonoL

A

monoblasts
monocytic precursors
auer rods in monocytic leukemia

52
Q

What are confirmatory tests for AMonoL

A

NSE positive

53
Q

What are lab findings for AEL

A

> /= 50% normoblasts in BM
/= 20% myeloblasts in BM
nRBC in PBS
dysplastic precursors

54
Q

What are confirmatory tests for AEL

A

PAS positive
NSE positive

55
Q

What are lab findings for AMegL

A

> /= 50% megakaryocytes
large-medium in size
can be agranular
circulating megakaryocytes in PBS
hypogranular neutrophils
giant PLT

56
Q

What are confirmatory tests for AMegL

A

PAS positive
NSE positive

57
Q

What can CLL progress into

A

autoimmune hemolytic anemia

58
Q

What are lab findings for CLL

A

normo/normo anemia
increased WBC
lymphocytosis
neutropenia
normal/decreased PLT
smudge cells

59
Q

What are confirmatory tests for CLL

A

CBC/smear
immunophenotyping
cytogenetics

60
Q

What are lab findings for hairy cell leukemia

A

small lymphs with hair like fibriae
normo/normo anemia
decreased PLT

61
Q

What are confirmatory tests for CLL

A

trephine biopsies (fibrosis)
CBC/smear
TRAP stain
immunophenotyping
annexin I positive

62
Q

What are lab findings for plasma cell myeloma

A

normo/normo anemia
plasma cells in PBS/BM
IgG/IgA increased
normal/decreased WBC
neutropenia
normal/decreaed PLT
rouleaux
decreased retics
flame cells in BM
mott cells in BM
ostolytic lesions

63
Q

What are confirmatory test for plasma cell myeloma

A

increased ESR
PT/PTT abnormal
bence jones proteins in urine
M protein on immunofixation
increased serum Ca

64
Q

What are lab findings for walderstrom macroglobinemia

A

increased IgM
normo/normo anemia
neutropenia
decreased PLT
rouleaux
plasmacytoid lymphs

65
Q

What are confirmatory test for walderstrom macroglobinemia

A

decreased serum calcium
increased ESR
PT/PTT abnormal
bence jones proteins in urine

66
Q

What are lab findings for CML

A

normo/normo anemia
increased WBC
normal/increased PLT
left shift with blasts
nRBC
increased eos/basos
increased M:E

67
Q

What causes CML

A

BCR-ABL-fusion gene of the philidelphia chromosome

68
Q

What are confirmatory tests for CML

A

CBC/smear
LAP stain negative
cytogenetics
molecular genetics
immunophenotyping

69
Q

What causes PV

A

a mutation of the Jak 2 gene V617F

70
Q

What are lab findings for PV

A

all cell lines proliferate uncontrollably (independent of EPO)
normo/normo anemia that can turn hypo/micro
increased HT
increased HGB
marrow fibrosis
splenomegaly

71
Q

What can PV progress to

A

acute leukemia

72
Q

What causes secondary PV

A

increased EPO due to tumor, chemical exposure, or drug abuse
only effects RBC lineage

73
Q

What causes relative PV

A

decreased plasma due to dehydration or smoking

74
Q

What are lab findings for primary myelofibrosis

A

increased prolifereation of granulocytes and megakaryocytes
fibrosis in the BM
tears, eliptos, myeloid precursors
hypercellular BM in stage 1
normo/normo anemia

75
Q

What can primary myelofibrosis progress to

A

AML

76
Q

What are confirmatory tests for primary myelofibrosis

A

trephine biopsy
genetic testing
molecular testing

77
Q

What are lab findings for ET

A

larg/hypogranular/clumping PLT
decreased HGB
increased PLT (600-1000+)
megakaryocyte fragments
normo/normo anemia
mild leukocytosis with left shift

78
Q

What are confirmatory tests for ET

A

iron stores in BM decrease over time
genetic testing
molecular testing

79
Q

What are lab findings for MDS of granulocytes

A

hypogranulation
hypersegmentation
hyposegmentation
karyorrhexis
megaloblastic changes
mitotic figures
decreased WBC

80
Q

What are lab findings for MDS of Erythroid

A

tears
macro/macroovalocytes
micro
hypo
schisto
acantho
spheros
elipto
HJ/BS
normo/normo anemia
dimorphic picture
nRBC

81
Q

What are lab findings for MDS of megakaryocytesB-lymphocytes

A

agranular or hypogranular
giant PLT
micromegakaryocytes
PLT clumps
decreased PLT
increased blasts
nRBC

82
Q

What are the main cells effected by CLL

A

B-lymphocytes

83
Q

What is hairy cell leukemia a subclass of

A

CLL

84
Q

What CD markers are unique for hairy cell leukemia

A

103, 11c

85
Q

What is monoclonal gammopathy of undetermined significance

A

when M protein is detectable but too low for diagnosis of other B-cell neoplasms

86
Q

What are the four subclasses of plasma cell myeloma

A

asymptomatic, non-secretory, symptomatic, plasma cell leukemia

87
Q

What is hodgkins lymphoma

A

a rare disorder of the lymph nodes of the upper body
reed-sternberg cells (owl-eyes) are found in lymph node biopsies

88
Q

What is non-hodgkins lymphoma

A

originates anywhere in the body with lymphatic tissue
does not have reed-sternberg cells

89
Q

What are the three phases of CML

A

chronic, accelerated, blast

90
Q

What are treatments for CML

A

tyrosine kinase inhibitors
inteferon-alpha
bone marrow/stem cell transplants
synthetic proteins that inhibit BCR-ABL protein
immunotherapy

91
Q

What is treatment for PV

A

therapeutic phlebotomy to reduce blood viscosity
myelosuppressive therapy
tyrosine kinase inhibitors
targeted molecular therapy

92
Q

What are the three phases of primary myelofibrosis

A

initial, fibrotic, blast

93
Q

What is the treatment for primary myelofibrosis

A

chemotherapy
drugs to improve cytopenia and anemia
blood transfusions
splenectomy
stem cell transplant
targeted molecular therapy

94
Q

What is the pathophysiology of primary myelofibrosis

A

an overproduction of hematopoetic stem cells leads to increased proliferation of granulocytes and megakaryocytes

malignant or neoplastic megakaryocytes will release fibroblastic growth factors which activates the fibroblasts which causes an uncontrolled assembly of collagen that brings fibrosis

95
Q

What can ET progress to

A

AML

96
Q

What is the treatment for ET

A

anticoagulant therapy
phlebotomy
plateletpheresis
targeted molecular therapy
drugs which suppress production of platelets

97
Q

What can cause myelodysplastic syndromes

A

exposure to chemicals
smoking
predisposition due to family history

98
Q
A

Hematology (7 decks)

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