Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology > Chapter 6 ( WBC Disorders ) > Flashcards

Chapter 6 ( WBC Disorders ) Flashcards

1
Q

Causes and ttt of neutropenia ?

A

1- drug toxicity ( chemotherapy with alkylating agents )
2- sever infection ( gram negative sepsis )

GM-CSF and G-CSF may be used to boost granulocyte production to decrease risk of infection

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

Causes of lymphopenia ?

A

Immunodeficiency
High cortisol state
Autoimmune destruction (SLE)
Whole body radiation

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

Causes of neutrophilic leucocytosis ?

A

Bacterial infection or tissue necrosis ( increase release of marginated pool and bone marrow neutrophils including immature forms ( shift to the left )

High cortisol state release marginated pool of neutrophils

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

Causes of monocytosis ?

A

Chronic inflammatory state ( autoimmune and infectious )

Malignancy

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

Causes of eosinophilia ?

A

Allergic reactions ( type I hypersensitivity )
Parasitic infections
Hodgkin lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Causes of basophilia ?

A

CML

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

Causes pf lymphocytic leucocytosis ?

A 
Viral infections ( T lymphocytes undergo hyperplasia ) 
Bordetella pertussis infection ( this bacteria produce lymphocytosis promoting factor which prevents lymphocytes from leaving blood to enter lymph nodes )
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

EBV affects which organs ?

A

1- oropharynx resulting in pharyngitis
2- liver resulting in hepatitis and hepatomegaly
3- B cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What does reactive CD8+ T cells response EBV lead to ?

A

1- generalized lymphadenopathy ( T cell hyperplasia in lymph nodes paracortex )
2- splenomegaly ( T cell hyperplasia in periarterial lymphatic sheath )
3- high WBC count with atypical lymphocytes ( reactive CD8+ T cells )

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

Mechanism of monospot test ?

A

Detects IgM antibodies that cross react with horse or sheep RBCs ( heterophile antibodies )
Turns positive after 1 week of infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What a negative monospot test suggests in case of IM ?

A

CMV as a possible cause for IM

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

Definitive diagnosis of IM ?

A

By serologic testing for the EBV viral capsid antigen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Complications of IM ?

A

Splenic rupture
Rash if exposed to ampicillin
Dormancy of virus in B cells increases the risk for both Recurrence and B cell Lymphoma especially if immunodeficiency develops

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

Acute leukemia ?

A

Accumulation of > 20% blasts in the bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Blast appearance ?

A

Large immature cells with punched out nucleoli

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Histological characteristic of lymphoblasts ?

A

Positive nuclear staining for TdT ( DNA polymerase )

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

Which part of the population has increased risk for acute lymphoblastic leukemia ?

A

Children associated with down syndrome ( after the age of 5 years )

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

Character of B-ALL ?

A

Lymphoblasts that express CD10 , CD19 , CD20

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Excellent response to chemotherapy in B-ALL requires what ?

A

Prophylaxis to scrotum and CSF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Prognosis of B-ALL based on what ? Explain .

A

On cytogenetic abnormalities :
1- t(12;21) has a good prognosis ( seen in children )

2- t(9;22) has a poor prognosis ( seen in adults ) ( Ph+ ALL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Character of T-ALL ?

A

Lymphoblasts that express markers ranging from CD2 to CD8

The blasts don’t express CD10

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

Presentation of T-ALL ?

A

Usually in teenagers as mediastinal mass ( thymic mass ) thats why its called acute lymphoblastic lymphoma

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

Histological characteristic of Myeloblasts ?

A

Positive cytoplasmic staining for myeloperoxidase

Crystal aggregates of MPO may be seen as Auer rods

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

Which part of the population has increased risk of developing AML ?

A

Older adults ( 50-60 years )

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

AML subclassified based on what ?

A

Cytogenetic abnormalities , lineage of myeloblasts , surface markers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Acute promyelocytic leukemia mechanism ? Character ? Ttt ?

A

Mechanism : t(15;17) translocation of the retinoic acid receptor on chromosome 17 to chromosome 15 , RAR disruption blocks maturation and promyelocytes accumulation

Character : contain numerous primary granules ( Auer rods ) that increase risk for DIC

Ttt : all trans retinoic acid ( which binds the altered receptor and causes the blasts to mature )

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Acute moncytic leukemia characters ?

A

Usually lack MPO

Blasts infiltrate gums

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Acute megakaryoblastic leukemia characters ?

A

Lack MPO

Associated with down syndrome ( arises before age of 5 )

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Presentation pf myelodysplastic syndrome ?

A

Cytopenias
Hypercellular bone marrow
Abnormal maturation of cells
Increased blasts >20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Mechanism of CLL ?

A

Neoplastic proliferation of naive B cells that coexpress CD5 , CD20

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Histologic characters of CLL ?

A

Increased lymphocytes and smudge cells on blood smear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Small lymphocytic lymphoma ?

A

Involvement of lymph nodes in CLL that leads to generalized lymphadenopathy

33
Q

Complications of CLL ?

A

Hypogammaglobulinemia ( infections is the most common cause of death )

Autoimmune hemolytic anemia

Transformation into diffuse large B cell lymphoma ( marked clinically by an enlarging lymph node or spleen )

34
Q

Richter transformation ?

A

Transformation of CLL into diffuse large B cell lymphoma

35
Q

Mechanism of hairy cell leukemia ?

A

Neoplastic proliferation of mature B cells characterized by hairy cytoplasmic processes

36
Q

Histological characteristic of hairy cell leukemia ?

A

Cells are positive for tartrate-resistant acid phosphatase (TRAP)

37
Q

Clinical features of hairy cell leukemia ?

A

TRAP disease :
1- B cells Trapped in bone marrow ( marrow fibrosis leading to Dry Tap )
2- B cells Trapped in red pulp of spleen ( splenomegaly )
3- No lymphadenopathy as the B cells are Trapped in bone marrow and spleen

38
Q

Drug used in Ttt of hairy cell leukemia ? Its mechanism ?

A

2-CDA ( Cladribine )

An adenosine deaminase inhibitor leads to accumulation of adenosine to toxic levels in neoplastic B cells

39
Q

Mechanism of Adult at cell Leukemia/Lymphoma (ATLL) ? Caused virus ? Seen where ?

A

Neoplastic proliferation of mature CD4 T cells
Associated with HTLV-1
Seen in Japan and Caribbean

40
Q

Clinical features of ATLL ?

A 
Rash 
Generalized lymphadenopathy 
Hepatosplenomegaly
Lytic ( punched out ) bone lesions 
Hypercalcemia
41
Q

Mechanism of Mycosis Fungoides ?

A

Neoplastic proliferation of mature CD4 T cells that infiltrate the skin producing :
Rash
Plaques
Nodules

42
Q

Pautrier microabscesses ?

A

Aggregates of neoplastic cells in the epidermis seen in Mycosis Fungoides

43
Q

Sezary syndrome ?

A

Occurs when mature CD4 neoplastic mature T cells spread to involve the blood in Mycosis Fungoides
Characterized by Presence of Sezary cells ( lymphocytes with cerebriform nuclei ) on blood smear

44
Q

General complications of myeloproliferative disorders ?

A

1- increased risk for hyperuricemia and gout due to high turnover of cells
2- progression to marrow fibrosis
3- transformation to acute leukemia

45
Q

Mechanism of chronic myeloid leukemia ?

A

Neoplastic proliferation of mature myeloid cells specially Granulocytes and their precursors ( Basophils are increased )

Driven by t(9;22) which generates BCR-ABL fusion protein with increased tyrosine kinase activity

46
Q

First line ttt in CML ?

A

Imatinib , which blocks tyrosine kinase activity

47
Q

To which type of acute leukemia can CML transfer to ?

A

To both typer since in a pluripotent stem cell
2/3 of cases to AML
1/3 of cases to ALL

48
Q

How is CML distinguished from leukemoid reaction ?

A

1- negative leukocyte alkaline phosphatase stain (LAP)
2- increased basophils
3- t(9:22)

49
Q

Polythycemia Vera defect ? Clinical symptoms ? Ttt ?

A

Defect : associated with JAK2 kinase mutation

Clinical symptoms : (due to hyperviscosity of blood)
Blurry vision and headache
Increased risk of venous thrombosis (hepatic vein , portal vein , dural sinus)
Flushed face due to congestion
Itching ( histamine release from increased mast cells)

Ttt: phlebotomy , Hydroxyurea

50
Q

Mechanism of myelofibrosis ?

A

Neoplastic proliferation of mature myeloid cells especially megakaryoctes
Associated with JAK2 kinase mutation ( 50% of cases )
Megakaryocytes produce excess PDGF causing marrow fibrosis

51
Q

Clinical features of myelofibrosis ?

A

1- splenomegaly
2- Leukoerythroblastic smear ( tear-drop RBCs - nucleated RBCs - immature granulocytes )
3- increased risk of infection, thrombosis , bleeding

52
Q

Types of lymph nodes enlargement due to inflammation according to the hyperplastic zone ?in Which conditions they occur ?

A

1- follicular hyperplasia : seen with RA and early stages of HIV infection

2- paracortex hyperplasia : with viral infections as IM

3- hyperplasia of sinus histiocytes : LNs draining tissue with cancer

53
Q

Classification of non-Hodgkin lymphoma according to cell size ?

A

1- small B cells : follicular lymphoma
Mantle cell lymphoma
Marginal zone lymphoma
Small lymphocytic lymphoma

2- intermediate sized B cells : Burkitt lymphoma

3- large B cells : diffuse large B cell lymphoma

54
Q

Follicular lymphoma presentation ? Ttt ? Complication ?

A

Presents in late adulthood with painless lymphadenopathy

Ttt : for symptomatic patients :
Low dose chemotherapy
Rituximab ( annti CD20 antibody )

Important complication : progression to diffuse large B cell lymphoma presents as an enlarging lymph node

55
Q

How is follicular lymphoma distinguished from reactive follicular hyperplasia ?

A

1- Disruption of normal LN architecture
2- Lack of Tingible body macrophages in germinal centers
3- Bcl2 expression in follicles
4- Monoclonality

56
Q

Marginal cell lymphoma associated with what ?

A

Chronic inflammatory states as
Hashimoto thyroiditis
Sjorgen syndrome
H pylori gastritis

57
Q

Marginal zone in LN is formed by ?

A

Post germinal center B cells

58
Q

MALToma ?

A

Marginal zone lymphoma in mucosal sites

Gastric MALToma may regress with ttt of H pylori

59
Q

Burkitt lymphoma associated with what ? Presentation ?

A

EBV
Presents as an extranodal mass in child or young adults :
1- African form : involves jaw
2- Sporadic form : involves abdomen

60
Q

Histological characteristic of Burkitt lymphoma ?

A

High mitotic index

Starry-sky appearance on microscope

61
Q

Most common form of NHL ? Prognosis ? Histology appearance ? Presentation ?

A

Diffuse large B cell lymphoma
Clinically aggressive - grows diffusely in sheets
Presents in late adulthood as an enlarging LN or an extranodal mass

62
Q

Mechanism of Hodgkin lymphoma ?

A

Neoplastic proliferation of Reed-Sternberg cells

63
Q

Characters of Reed Sternberg cells ?

A

Large B cells
With multilobed nuclei
And prominent nucleoloi ( owl eyed nuclei )
Positive for CD15 and CD30

64
Q

What does RS cells secret ? It action ?

A 
Cytokines that lead to : 
1- B symptoms ( fever,chills,weight loss,night sweats ) 
2- attraction of 
Reactive lymphocytes 
Plasma cells 
Macrophages 
Eosinophils
3- fibrosis
65
Q

Subtypes of Hodgkin lymphoma ?

A

1- Nodular sclerosis
2- Lymphocyte-rich
3- Mixed cellularity
4- Lymphocyte-depleted

66
Q

Most common subtype of Hodgkin lymphoma ? Presentation ? Histological characters ?

A

Nodular sclerosis
Presents as enlarging cervical or mediastinal LN in a young adult , usually female
Histology :LN is divided by bands of sclerosis and RS cells present in lake like spaces ( lacunar cells )

67
Q

Subtype of HL that has the best prognosis ?

A

Lymphocyte-rich

68
Q

Characteristic of mixed cellularity HL ?

A

Abundant eosinophils

69
Q

The most aggressive subtype of HL ? Seen in which patients ?

A

Lymphocyte-depleted

Seen in elderly and HIV positive individuals

70
Q

Mechanism of multiple myeloma ?

A

Most common primary tumor of bone
Due to malignant proliferation of plasma cells in bone marrow , high serum IL-6 maybe present which stimulates plasma cell growth and immunoglobulin production

71
Q

Clinical features of multiple myeloma ?

A

I BM and RAP
1- bone pain with hypercalcemia ( neoplastic plasma cells activate the RANK receptor on osteoclasts leading to bone destruction )
2- elevated serum proteins ( M spike on SPEP due to monoclonal IgG or IgA )
3- increased risk of infection ( monoclonal antibodies lacks antigenic diversity )
4- Rouleaux formation of RBCs ( increased serum proteins decrease charge between RBCs )
5- primary AL amyloidosis ( free light chains deposit in tissues )
6- proteinuria ( free light chains are excreted in urine as Bence Jones proteins , deposition in kidney tubules leads to risk of renal failure ( Myeloma kidney )

72
Q

Monoclonal Gammpathy of Underestimated Significance ( MGUS ) ?

A

Increased serum proteins with M spike on SPEP but other features of MM are absent
Common in elderly , 1 % of patients with MGUS develop MM each year

73
Q

Waldenstorm Macroglobulinemia mechanism ? Clinical features ? Ttt ?

A

B cell lymphoma with monoclonal IgM production
Clinical features :
1-Generalized Lymphadenopathy
2-Increased serum proteins with M spike on SPEP ( due to ⬆️ IgM )
3-Visual and neurological deficits ( retinal hemorrhage or stroke ) ( IgM causes hyperviscosity )
4-Bleeding ( viscous proteins results in defective platelet aggregation )
Ttt : plasmapheresis

74
Q

Source and function of Langerhans cells ?

A

Derived from bone marrow monocytes

Present antigen to naive T cells

75
Q

Langerhans cell histiocytes ?

A 
Neoplastic proliferation of Langerhans cells 
Characteristic Birbeck ( tennis racket ) granules are seen on electron microscopy 
Cells are CD1a+ and S100+ by immunohistochemistry
76
Q

Letterer Siwe disease mechanism ? presentation ?

A

Malignant proliferation of langerhans cells
Skin rash
Cystic skeletal defects in an infant < 2 years
Multiple organs maybe involved
Rapidly fatal

77
Q

Eosinophilic granuloma mechanism ? Presentation ? Histological chacters ?

A

Benign proliferation of langerhans cells
Pathologic fracture in an adolescent
Skin is not involved
Biopsy :langerhans cells with mixed inflammatory cells including numerous eosinophils

78
Q

Hand-Schuller-Christian disease mechanism ? Presentation ?

A 
Malignant proliferation of Langerhans cells 
LEDS 
Scalp rash 
Lytic skull defects 
Diabetes insipidus
Exophthalmos 
In a child > 3 years

Pathology (18 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions