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My Pathoma Unit 2 > Chapter 6 White Blood Cell Disorders > Flashcards

Chapter 6 White Blood Cell Disorders Flashcards

1
Q

What is the CD marker of a hematopoietic stem cell?

A

CD34

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2
Q

What are two major causes of neutropenia?

A 
Drug toxicity ( eg chemo with alkylating agents) - damage to stem cells results in decreased production of WBCs especially neutrophils.
Severe infection (Gram neg sepsis) increased movement of neutrophils into tissues
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3
Q

What treatment can be used to boost granulocyte production?

A

G-CSF or GM-CSF

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4
Q

What are some major causes of lymphopenia?

A

1 Immunodeficiency (DiGeorges and HIV)
2 High Cortisol state (exogenous coricosteroids or cushing syndrome) induces apoptosis in lymphocytes
3 Autoimmune destruction ( SLE)
4 Total Body irradiation

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5
Q

Name two causes of neutrophilic leukocytosis?

A

1 Bacterial infection or tissue necrosis induces release of marginal pool, and bone marrow neutrophils including immature forms (Bands)
2 High Cortisol states impair leukocytes adhesion leading to release of marginated pools.

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6
Q

What cell marker will band forms be lacking in?

A

CD16 which is Fc receptors

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7
Q

What are a couple causes of monocytosis?

A

chronic inflammatory states (autoimmune and infectious) and malignancy

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8
Q

What are 3 causes of eosinophilia?

A

allergic reactions, parasitic infections, hodgkin lymphoma.

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9
Q

What is the eosinophilic chemotactic factor?

A

IL-5

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10
Q

What is a classic setting for basophilia?

A

CML

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11
Q

What are two causes of lymphocytic leukocytosis?

A

Viral infections- Tcell hyperplasia

Bordetella pertussis infection

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12
Q

In bordetella pertussis infection what causes lymphocytosis?

A

release of lymphocytosis-promoting factor blocks circulating lymphocytes from entering lymph node.

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13
Q

What type of leukocytosis is caused by EBV?

A

Lymphocytic reactive CD8 T cells

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14
Q

What does EBV primarily infect in IM? (body parts)

A

Oropharynx
liver resulting in hepatitis with hepatomegaly and elevated liver enzymes
B - Cells

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15
Q

What does the CD8 Tcell response in EBV IM lead to?

A

Generalized LAD
Splenomegaly due to proliferation in PALS
High WBC with atypical lymphocytes (reactive CD8)

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16
Q

What does the monospot test detect? if it is negative what does this point to?

A

Detects IgM antibodies against horse or sheep RBCs (heterophile antibodies)
negative indicates CMV as IM cause.

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17
Q

What are some complications of IM?

A

risk fro splenic rupture
rash with penicillin
dormancy in B cels can lead to recurrance and B-cell lymphoma.

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18
Q

What is acute leukemia a neoplastic proliferation of? What is the threshold for % of blasts

A

blasts; defined as the accumulation of >20% blasts in the bone marrow.

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19
Q

What are the effects of increased blasts in acute leukemia on bone marrow production?

A

increased blasts crowd-out normal hematopoiesis resulting in pancytopenia

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20
Q

What effect does acute leukemia have on the WBC and peripheral smear?

A

blasts usually enter the blood stream resulting in a high WBC with blasts on peripheral smear.

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21
Q

What are some histological features of blasts (what do they look like)?

A

they are large immature cells often with punched out nucleoli

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22
Q

What nuclear stain can be used to identify ALL and differentiate it from AML and CLL?

A

Tdt, a DNA polymerase that is not present in myeloid blasts or mature lymphocytes.

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23
Q

What is the most common ALL demographic? What other pathology is it associated with?

A

Commonly arises in children after the age of 5; associated with DS

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24
Q

What is more common B-ALL or T-ALL?

A

B-ALL

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25
Q

What is the treatment of B-ALL, does it work?

A

Excellent response to chemotherapy; requires prophylaxis to scrotum and CSF

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26
Q

What markers are present for good prognosis in ALL?

A

early precursor B-cell type, hyperdiploidy, patient between 7-10, chromosomal trisomy, and t(12;21)

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27
Q

What identifying histochemical characteristics are seen in B-ALL (markers)

A

TdT+, CD10, CD19, CD20

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28
Q

What are the cell markers for T-ALL?

A

TdT+ and markers ranging from CD2-CD8.

NO CD10

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29
Q

What demographic does T-ALL present in?

A

Teenagers as a mediastinal mass (usually thymus) it is called acute lymphoblastic lymphoma due to the presence of a mass

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30
Q

What cytoplasmic staining is seen in AML?

A

myeloperoxidase (MPO)

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31
Q

What is myeloperoxidase function in the immune system?

A

MPO is in neutrophils to produce hypochlorus acid from H2O2 and Cl- during the respiratory burst.

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32
Q

What are auer rods?

A

crystal aggregates of MPO in AML cells

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33
Q

What demographic does AML normally arise in?

A

older adults (50-60 years old)

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34
Q

What translocation is seen in APL?

A

t(15;17) prevents maturation

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35
Q

What serious hematological complications can arise from APL?

A

DIC, auer rods increase risk.

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36
Q

What area of the body do blasts in Acute monocytic leukemia usually infiltrate? How do these cells differ from APL cells as far as stains go?

A

Gums, Lack MPO

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37
Q

What condition is acute megakaryoblastic leukemia associated with? Do these blasts contain MPO?

A

DS (usually presents before 5)

No MPO

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38
Q

What underlying condition can lead to AML?

A

myelodysplastic syndrome especially after exposure to alkylating agents or radiotherapy.

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39
Q

Chronic Leukemia is a neoplastic proliferation of what cell? What hematological finding is it characterized by (general)?

A

Mature circulating lymphocytes resulting in a high WBC

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40
Q

Describe the onset and demographic in Chronic leukemia?

A

Insidious onset and seen in older adults

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41
Q

What neoplastic proliferation is seen in CLL? What cell markers are present?

A

proliferation of Naive B-Cells express CD5 and CD20

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42
Q

What abnormalities are seen on smears in CLL?

A

Increases lymphocytes and smudge cells

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43
Q

Involvement of the lymph nodes in CLL leads to generalized LAD and is called what?

A

Small lymphocytic lymphoma

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44
Q

Name 3 complications seen in CLL?

A

1 hypogammaglobulinemia (Cells dont differentiate into plasma cells)
2 Autoimmune hemolytic anemia
3 transformation to diffuse large B-cell lymphoma (marked by focal lymph enlargement)

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45
Q

What is the most common cause of death in CLL?

A

Infection (due to hypogammaglobulinemia)

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46
Q

What proliferation is seen in Hairy Cell Leukemia

A

proliferation of mature B-Cells characterized by hairy cytoplasmic processes

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47
Q

What are HCL cells positive for?

A

Tartrate-resistant acid phosphatase (TRAP)

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48
Q

What clinical features are seen in HCL?

A

splenomegaly due to accumulation in red pulp, dry tap aspiration due to marrow fibrosis, LAD is usually absent

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49
Q

What is the treatment fro HCL?

A

2-CDA (Cladribine) an adenosine deaminase inhibitor; adenosine accumulates to toxic levels

50
Q

What neoplastic proliferation is seen in Adult T Cell leukemia/lymphoma?

A

Mature CD4+ Tcells

51
Q

What demographic and virus is ATLL seen in?

A

HTLV-1 virus in japan and caribbean

52
Q

What clinical features are seen in ATLL

A

rash (skin infiltration), generalized LAD with hepatosplenomegaly. lytic punched out bone lesions with hypercalcemia

53
Q

What neoplastic proliferation is seen in Mycosis Fungoides?

A

Mature CD4+ Tcells

54
Q

What are the clinical features of mycosis fungoides?

A

cells infiltrate the skin, producing localized skin rash, plaques, and nodules. Aggregates of neoplastic cells in the epidermis are called Pautrier microabscesses

55
Q

What is Sezary Syndrome? what is seen on smears?

A

When mycosis fungoides spread to the blood, it is characterized by lymphocytes with cerebriform nuclei (sezary cells)

56
Q

What neoplastic proliferation is seen in MPDs?

A

mature cells of myeloid lineage

57
Q

What demographic are MPDs seen in?

A

Late adulthood

58
Q

What hematological changes are seen in all MPDs?

A

high WBC due to increase synth of all lineages with one predominant and hypercellular marrow

59
Q

What 2 general complications can arise from MPDs?

A

1 Increased risk for hyperuricemia and gout due to high turnover of cells
2 Progression to marrow fibrosis or transformation to acute leukemia.

60
Q

What are the 4 main MPDs?

A

CML, PV, ET, and myelofibrosis

61
Q

What particular proliferation is seen in CML?

A

granulocytes; characteristically basophils

62
Q

What marks the accelerated phase of CML?

A

splenomegaly is common in general but an actively enlarging spleen indicates the accelerated phase

63
Q

What can CML transform into? what does this say about the cell of origin?

A

AML or ALL meaning it must be a mutation in a pluripotent stem cell

64
Q

How is CML distinguished from a leukemoid reaction?

A

Negative leukocyte alkaline phosphatase (LAP)
increased basophils
t(9;22)

65
Q

What mutation is associated with polycythemia vera and what cell dominates proliferation?

A

JAK2 kinase dominated by RBCs

66
Q

What are the clinical symptoms of PV and what are they due to?

A

Symptoms due to hyperviscosity
blurry vision
increased risk of venous thrombosis
flushed face due to congestion (plethora)
itching, especially after bathing due to increased mast cells

67
Q

What is the treatment form PV?

A

Phlebotomy; second line is hydroxyurea

68
Q

What mutation is seen in essential thrombocytopenia and what is the dominant cell?

A

JAK2 kinase and platelets

69
Q

What are the symptoms of ET related to? Does it commonly progress to marrow fibrosis or AL, is there a significant risk for hyperuricemia?

A

increased risk for thrombosis and or bleeding (platelets can be under or overfunctioning)
Rarely progresses, no increased risk of gout bc no nucleus turnover

70
Q

What mutation is Myelofibrosis associated with and what is the dominant cell?

A

JAK2 kinase and megakaryocyte

71
Q

What causes marrow fibrosis in myelofibrosis?

A

excess production of platelet derived growth factor by megakaryocytes

72
Q

What are the clinical features of Myelofibrosis (3)?

A

1 Splenomegaly due to extramedullary hematopoiesis
2 Leukoerythroblastic smear (tear drop RBCs, nucleated RBCs, and immature granulocytes)
3 increased risk of infection, thrombosis, and bleeding (spleen cannot completely compensate)

73
Q

What are two examples of when you would see follicular hyperplasia in a lymph nodes?

A

rheumatoid arthritis and early stage HIV

74
Q

What is an example of when you would see paracortex hyperplasia in lymph nodes?

A

Viral infections

75
Q

What is an example of when you would see hyperplasia of sinus histiocytes in lymph nodes?

A

Node draining a tissue with cancer

76
Q

What type of cells are proliferating a follicular lymphoma?

A

small B-cells CD20+ that form a follicle like nodule

77
Q

What translocation is seen in follicular lymphoma?

A

t(14;18) (Bcl2 and IgH)

78
Q

How is follicular lymphoma distinguished from reactive follicular hyperplasia. 4 ways

A

1 disruption of normal lymph node architecture
2 lack of tingible macrophages
3 overexpression of bcl2
4 monoclonality

79
Q

What proliferation is seen in mantle cell lymphoma? what translocation?

A

small B-cells (CD20+) t(11;14) Cyclin D1 and IgH

80
Q

What proliferation is seen in marginal zone lymphoma? what type of cells are these

A

small B-cells (CD20+) formed by POST germinal center B-cells

81
Q

What is marginal zone lymphoma associated with?

A

Associated with chronic inflammatory states such as hashimotos, Sjorgen, and H pylori. MALToma is a marginal zone lymphoma of mucosal sites

82
Q

What proliferation is seen in Burkitt cell lymphoma? what is the translocation?

A

intermediate size b cells (CD20+) t(8;14) cMYC

83
Q

What demographic does burkitt lymphoma generally present in?

A

extranodal mass in a child or young adult

84
Q

What two variants of burkitt lymphoma are there and what sites do they present in?

A

African - jaw

sporadic involves abdomen

85
Q

What microscopic characteristics are seen in burkitt lymphoma?

A

High mitotic index and ‘starry skied’ appearance

86
Q

What is the proliferation seen in diffuse large B cell lymphoma? how does it usually present? how do the cells grow?

A

Large B-cells (CD20+)
Arises sporadically or from transformation in late adulthood as an enlarging lymph node
cells grow diffusely in sheets

87
Q

What is the proliferation seen in Hodgkins lymphoma and what are the cell markers?

A

Reed-Sternberg cells which are large B-cells with multilobed nuclei and prominent nucleoli classically positive for CD15 and CD30
NO CD20

88
Q

RS cells in HL secrete cytokines, what does this lead to?

A

Occasional ‘B’ symptoms (fevers chills, weight loss, and night sweats)
attract lymphocytes, plasma cells, macrophages, and eosinophils
may lead to fibrosis

89
Q

What is the most common type of HL? What is it characterized by and how does it present?

A

Nodular sclerosis characterized by lymph node divided by bands of sclerosis with RS cells in lake like spaces (lacunar). Presents as an enlarging cervical or mediastinal lymph node in a young adult usually a female

90
Q

Which type of HL has the best prognosis?

A

Lymphocyte rich

91
Q

Which type of HL is associated with abundant eosinophils?

A

Mixed cellularity (secretion of IL 5)

92
Q

Which type of HL is most aggressive?

A

Lymphocyte depleted

93
Q

What type of proliferation is seen in multiple myeloma?

A

Plasma cells in bone marrow

94
Q

Levels of what cytokine are raised in multiple myeloma? What does this do?

A

IL 6 which stimulates plasma cell growth and immunoglobulin production

95
Q

Name six clinical signs of multiple myeloma?

A 
1 Bone pain with hypercalcemia
2 elevated serum protein
3 increased risk of infection
4 rouleaux formation of RBCs on smear
5 Primary AL amyloidosis
6 Proteinuria
96
Q

What causes bone pain and hypercalcemia in Multiple myeloma?

A

neoplastic plasma cells secrete RANKL which activates osteoclasts

97
Q

What causes elevated serum protein in multiple myeloma?

A

plasma cells produce immunoglobulin which presents as an M Spike on SPEP most commonly due to monoclonal IgA or IgG

98
Q

What caused increased risk of infection in multiple myeloma?

A

Monoclonal antibodies lack antigenic diversity

99
Q

What causes rouleaux formation in multiple myeloma?

A

increased serum protein decreases charge between RBCs

100
Q

What causes primary AL amyloidosis in multiple myeloma?

A

free light chains circulate in serum and deposit in tissues

101
Q

What causes proteinuria in multiple myeloma?

A

free light chain is secreted in the urine as Bence Jones protein; deposition in kidney tubules leads to risk for renal failure

102
Q

What is Waldenstrom Macroglobulinemia?

A

B-cell lymphoma with monoclonal IgM production

103
Q

What are clinical signs of waldenstrom macroglobulinemia?

A

Generalized LAD
M spike due to IgM
Visual and neuro deficits due to serum hyperviscosity
treat with plasmapheresis

104
Q

What are langerhans cells?

A

Specialized dendritic cells found predominantly in the skin

105
Q

What are langerhans cells made from and what do they do?

A

They are derived from bone marrow monocytes and present antigen to T cells

106
Q

What is characteristic of langerhans cell histiocytosis on EM and immunohistochemistry?

A

Characteristic Birbeck (tennis racket) granules and cells are CD1a+ and S100+

107
Q

What is Letterer Siwe Disease?

A

Malignant proliferation of langerhans cells classically presents as skin rash and cystic skeletal defects in infant (<2). multiple organs may be involved and it is rapidly fatal

108
Q

What is Eosinophillic granuloma?

A

Benign proliferation of langerhans cells in the bone. Classic presentation is pathologic fracture in aldolescent, skin not involved. Biopsy shows langerhans cells with mixed inflammatory cells, including numerous eosinophils

109
Q

What is Hand-Schuller-christian disease?

A

Malignant proliferation of langerhans cells. Classic presentation is scalp rash, lytic skill defects, diabetes insipidus, and exopthalmos in a child (>3)

110
Q

What are the atypical lymphocytes seen in IM? what makes them atypical?

A

Reactive CD8+ T cells which look like monocytes. They are atypically large and have blue cytoplasm.

111
Q

how do myelodysplastic syndromes typically present?

A

cytopenias, hypercellular marroy, abnormal maturation, and increased blasts (However <20%)

112
Q

What are some cytological markers of post-therapy myelodysplasia?

A

Chromosomal deletions such as 5q

113
Q

What type of HL is EBV associated with in over 90% of cases?

A

Lymphocyte depleted

114
Q

What histological properties can help differentiate myeloblasts from lymphoblast?

A

Azurphilic, peroxidase positive granules.

115
Q

What chromosomal rearrangement is seen in Digeorges syndrome?

A

22q11.2

116
Q

What markers indicate poor prognosis in ALL?

A

T cell phenotype, patient younger than 2, WBC > 100K, and t(9;22)

117
Q

What is the characteristic gene rearrangement seen in Anaplastic large cell lymphoma (t cells)

A

rearrangement 2p23 that results in production of anaplastic lymphoma kinase with tyrosine kinase activity.

118
Q

What is cat scratch disease caused by, what are the signs?

A

caused by rochailimaea Henselae and results in lymphadenopathy with microscopic stellate necrosis. self limiting.

119
Q

What is CD11c a marker for?

A

monocytes

120
Q

What symptom is seen in about 1/3 of thyomas?

A

myasthenia gravis

121
Q

What type of infections do people with multiple myeloma have difficulty fending off?

A

encapsulated bacteria.

My Pathoma Unit 2 (6 decks)

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