White blood cell disorders Flashcards

1
Q

CLL “chronic lymphocytic leukaemia “

A

Neoplastic proliferation of naive B cells

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2
Q

CLL it’s cells coexpress

A

CD5 , CD20

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3
Q

CLL blood smear

A

Increased lymphocytes and smudge cells

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4
Q

CLL complications

A

Hypogammaglobulinemia
Autoimmune haemolytic anemia
Transformation to diffuse large B cell lymphoma

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5
Q

Hairy Cell leukaemia

A

Neoplastic proliferation of mature B cells

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6
Q

Hairy cell leukaemia characterized by

A

Hairy cytoplasmic processes

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7
Q

Hairy cell leukaemia are positive for

A

TRAP “tartrate resistance acid phosphatase “

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8
Q

Hairy cell leukaemia clinical features

A

Splenomegaly “ expansion of red pulp”
Dry tab with bone marrow aspiration
Lymphadenopathy is usually absent

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9
Q

Hairy cell leukaemia has excellent response to

A

2-CDA “ adenosine deaminase inhibitor “

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10
Q

ATLL “adult T cell leukaemia lymphoma “

A

Neoplastic proliferation of mature CD4 T cell

Associated with HTLV1 “ Japan and Caribbean “

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11
Q

ATLL clinical features

A

Rash
Generalised LAD and hepatosplenomegaly
Lytic bone lesions with hypercalcemia

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12
Q

Mycosis fungoides

A

Neoplastic proliferation of mature CD4 T cells

In which the cells infiltrate skin producing rash , plaque , nodules

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13
Q

Aggregates of neoplastic T cells in epidermis are called

A

Pautreir microabscesses

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14
Q

If the cells of mycosis fungoides involve the blood it cause

A

Sezary syndrome

Has characteristic lymphocytes with cerebriform nuclei on blood smear

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15
Q

Myeloproliferative disorders definition

A

Neoplastic proliferation of mature cells of myeloid lineage

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16
Q

Myeloproliferative disorders complications

A

Increase risk for hyperuricemia and gout
Progression to marrow fibrosis
Transformation into acute leukaemia

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17
Q

Chronic myeloid leukaemia highly associated with

A

Basophilia

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18
Q

CML driven by ,,,,,,,,,,,

A

t (9,22)BCR-ABL fusion increase tyrosine kinase activity

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19
Q

CML treated by

A

Imatinib “block tyrosine kinase activity “

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20
Q

CML can be transformed into either

A

AML (2/3) or ALL (1/3)

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21
Q

CML must be distinguished from leukemoid reation by

A

CML associated with :
LAP negative “leukocyte alkaline phosphatase”
Increased basophils
t(9,22)

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22
Q

Polycythemia vera is associated with

A

JAK2 kinase mutation

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23
Q

Polycythemia vera clinical symptoms

A

Blurred vision and headache
Venous thrombosis
Flushed face
Itching after bathing

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24
Q

Polycythemia vera must be distinguished from reactive polycythemia

A

PV : SaO2 normal , EPO low
In reactive polycythemia: SaO2 low , EPO increased “lung disease “
In reactive polycythemia : EPO high due to ectopic production and SaO2 normal

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25
Essential thrombocythemia
Also associated with JAK2 kinase mutation
26
No significant risk for hyperuricemia or gout with ,,,,,,,,,,,,,
Essential thrombocythemia
27
Myelofibrosis
Neoplastic proliferation of mature myeloid cells especially megakaryocytes
28
Painful LAD”lymphadenopathy” is seen with
Acute infection
29
Painless LAD is seen with
Chronic inflammation , metastatic carcinoma , lymphoma
30
Expansion of follicles is seen with
Rheumatoid arthritis ,early HIV
31
Expansion of paracortex is seen with
Viral infection
32
Expansion of region where the sinus histiocytes live is seen with
Cancer
33
Lymphoma may arise in ............ or ..............
Lymph node or extranodal tissue
34
Lymphoma classified into
Non Hodgkin lymphoma and Hodgkin lymphoma
35
Follicular lymphoma
Neoplastic small B cells (CD20+) that make follicle-like nodules
36
Follicular lymphoma driven by
t(14-18)BCL2 translocates from 18 to 14 result in overexpression of BCL2 which inhibit apoptosis
37
Follicular lymphoma treatment
Rituximab
38
Follicular lymphoma complication
Progression to diffuse large B cell lymphoma
39
Follicular lymphoma distinguished from follicular hyperplasia by
1. disruption of normal LN architecture 2. lack of tingible body macrophages in germinal centre 3. expression of BCL2 in follicles 4. monoclonality
40
Mantle cell lymphoma
Neoplastic small B cells CD20+that expand the mantle zone “region immediately adjacent to the follicle”
41
Mantle cell lymphoma driven by
t(11:14) cyclin D1 translocation leads to overexpression and promotion of G1/S transition in cell cycle
42
Marginal zone lymphoma
Neoplastic small B cells CD20+ that expand marginal zone
43
Marginal zone lymphoma associated with chronic inflammatory states like:
Hashimoto’s thyroiditis , sjogren syndrome , H pylori gastritis
44
Burkitt lymphoma
Neoplastic intermediate sized B cells CD20+ | Associated with EBV
45
Burkitt lymphoma has two forms :
African form usually involve jaw | Sporadic form usually involve abdomen
46
Burkitt lymphoma driven by
t(8;14) c-myc translocation
47
Burkitt lymphoma has a characteristic
Starry sky appearance
48
DLBCL “diffuse large B cell lymphoma”
Neoplastic large B cells that grow diffusely in sheets
49
Most common form of non Hodgkin lymphoma is
DLBCL
50
Hodgkin lymphoma definition
Neoplastic proliferation of Reed-Sternberg cells “large B cell with multilobed nuclei “ CD15+ and CD30+
51
Hodgkin lymphoma subtypes
1. Nodular sclerosis(70% of cases) 2. lymphocyte rich 3. mixed cellularity 4. lymphocyte depleted
52
Classic presentation for nodular sclerosis HL
Enlarging cervical or mediastinal LN in young adult usually female
53
Nodular sclerosis HL histology
Reed-Sternberg cells are present in lake like spaces(lacunar cells) LN separated by bands of fibrosis
54
HL subtype has the best prognosis
Lymphocyte rich HL
55
HL subtype has the worst prognosis
Lymphocyte depleted HL
56
Lymphocyte depleted HL seen in
Elderly and HIV+ indivuals
57
Mixed cellularity HL is associated with
Abundant eosinophils (due to IL5 secretion)
58
Multiple myeloma
Malignant proliferation of plasma cells in bone marrow
59
Most common primary malignancy of bone is
Multiple myeloma
60
High serum ........ is sometimes present in multiple myeloma
IL6
61
Neoplastic plasma cells activate
RANK receptor on osteoclasts
62
Multiple myeloma symptoms
1. Bone pain with hypercalcemia 2. Lytic (punched out) lesion seen on X-ray 3. Elevated serum protein
63
M spike is present on ‘serum protein electrophoresis’ SPEP in multiple myeloma due to
Monoclonal IgG or IgA
64
Monoclonal antibody lacks
Antigenic diversity ” lead to increase risk of infection “
65
Increase serum protein in multiple myeloma decrease charge between RBCs lead to
Rouleaux formation on blood smear
66
Proteinuria with multiple myeloma lead to
1. bence Jones proteins in urine “free light chains” | 2. deposition in kidney tubules “risk for renal failure”
67
MGUS (monoclonal gammopathy of undetermined significance)
.Increased serum protein with M spike on SPEP | .Other features of Multiple myeloma are absent
68
MGUS common in
Elderly
69
Waldenstrom macroglobulinemia
B cell lymphoma with monoclonal IgM production
70
Waldenstrom macroglobulinemia clinical features
.Generalized LAD .Increased serum protein with M spike (IgM) .Visual and neuronal deficits .Bleeding
71
Waldenstrom macroglobulinemia treatment
Plasmapheresis”remove IgM”
72
Langerhans cells histiocytosis
Neoplastic proliferation of langerhans cells
73
Langerhans cells histiocytosis characterized by
Birbeck(tennis racket) granules seen on EM | CD1a+ and S100+
74
Letterer-siwe disease
Malignant proliferation of langerhans cells Skin rash and cystic skeletal defects in infant <2yrs Multiple organ may be involved Rapidly fatal
75
Eosinophilic granuloma
.Benign proliferation of langerhans cells in bone .present with pathological fracture in adolescent .biopsy show langerhans cells with inflammatory cells including eosinophils
76
Hand-schuller-Christian disease
.Malignant proliferation of langerhans cells | .present with scalp rash, lytic skull defect , diabetes insipidus and exophthalmos in a child
77
Normal WBC count
5-10 K
78
Neutropenia seen in
Drug toxicity | Severe infection
79
Lymphopenia seen in
Immunodeficiency High cortisol state Autoimmune destruction Whole body radiation
80
In neutrophilic leukocytosis there is an increase in number of
Immature neutrophils “ with decreased Fc receptor”
81
A marker of decreased Fc receptors is
CD16
82
Neutrophilic leukocytosis seen in
.Bacterial infection .Tissue necrosis .High cortisol state”by disrupting adhesion of marginated neutrophils”
83
Monocytosis seen in
Chronic inflammatory state | Malignancy
84
Eosinophilia seen in
.Allergic reaction .Parasitic infection .Hodgkin lymphoma “due to high IL5”
85
Basophilia seen in
CML
86
Lymphocytic leukocytosis seen in
.Viral infection | .Bordetella pertussis
87
Infectious mononucleosis seen with
.EBV infection | .CMV (less common)
88
EBV primarily infect
Oropharynx Liver B cells
89
EBV infection results in CD8+ T cell response with
.Generalised LAD “ paracortex hyperplasia “ .Splenomegaly “ periarterial lymphatic sheath” .high white count with atypical lymphocytes
90
Monospot test is used for screening of
Infectious mononucleosis
91
Monospot test detect
IgM heterophile antibodies
92
Negative monospot test suggests
CMV infection
93
Definitive diagnosis for infectious mononucleosis by EBV is made by (زيادة تأكيد)
Testing for EBV viral capsid antigen
94
Complications of Infectious mononucleosis :
Increased risk of splenic rupture Rash if exposed to penicillin Dormancy of virus in B cells
95
Acute leukaemia
Accumulation of >20% blasts in the bone marrow
96
In AL the blasts crowd out normal hematopoiesis , results in
Acute presentation with anemia , thrombocytopenia or neutropenia
97
Blasts enter blood result in
High WBCs count
98
Blasts definition
Large immature cells , often with punched out nucleoli on blood smear
99
Acute leukaemia subdivided into
AML and ALL
100
ALL
Neoplastic proliferation of lymphoblasts
101
Positive nuclear staining for TdT(DNA polymerase) with
ALL
102
ALL most commonly arise in
Children associated with Down syndrome (after the age of 5)
103
The most common type of ALL
B-ALL
104
B-ALL classically express
CD10 , CD19 and CD20
105
B-ALL has excellent response to
Chemotherapy
106
B-ALL prognosis based on cryogenic abnormalities :
t(12;21) good prognosis >kids | t(9;22) poor prognosis > adults
107
T-ALL express
Ranging from CD2 to CD8
108
T-ALL present as
Mediastinal “thymic” mass in a teenager | Known as Acute lymphoblastic lymphoma
109
AML characterized by staining for
MPO (myeloperoxidase )
110
Crystal aggregate of MPO may be seen as
Auer rods
111
AML most commonly arise in
Older adults (50-60yrs)
112
Acute promyeloctyic leukaemia characterized by
t(15;17) , RAR “retinoacidic receptor” distrupted ; promyelocytes accumulate
113
Promyeloctyes contain
Numerous Auer rods , risk for DIC
114
Acute promyelocytic leukaemia treatment
ATRA (all-trans-retinoic acid) | Cause blasts to mature
115
Acute monocytic leukemia
Proliferation of monoblasts , lack MPO
116
Blasts in acute monocytic leukemia characteristically infiltrate
The gums
117
Acute magakaryblastic leukemia
Proliferation of megakaryblasts ; lack MPO
118
Acute megakaryoblastic leukemia associated with
Down syndrome ( before the age of 5)
119
AML may arise from exposure to................. or ...................
Alkylating agents or radiotherapy
120
Notes about Myelodysplastic syndrome
.cytopenia with hypercellular bone marrow .abnormal maturation with increased blasts (<20%) .may progress to acute leukemia (>20%) Most patients die from infection or bleeding