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white blood cell disorders Flashcards

1
Q

Hematopoietic stem cell

A

CD34+

produce myeloid stem cells or lymphoid stem cells

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2
Q

Normal white blood cell count

A

5-10K

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3
Q

low WBC count

A

leukopenia

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4
Q

high WBC count

A

leukocytosis

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5
Q

Neutropenia

A

Low neutrophils
D/t drug tox or severe infection
TX: GM-CSF or G-CSF

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6
Q

Lymphopenia

A 
Low lymphocytes
Immunodeficiency 
High cortisol state
AI destruction
Whole body radiation (lymphocytes are most sensitive to radiation)
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7
Q

Neutrophilic leukocytosis

A

High circulating number
Bacterial infection
Tissue necrosis
High cortisol state - impairs leukocyte adhesion - leading to increased marginated pool of neutrophils

Release slightly immature cells (left shift)

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8
Q

Left shift

A

Release of precursor neutrophils
Decrease Fc receptor - which help recognize immunoglobin (so don’t function as well as mature)

Fc = CD16 - decreased

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9
Q

Mnoocytosis

A

Chronic inflammatory states

Malignancy

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10
Q

Eosinophilia

A

Allergic Rxn
Parasitic infection
Hodgkin lymphoma

Increased IL-5 production

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11
Q

Basophilia

A

CML

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12
Q

Lymphocytic leukocytosis

A 
Viral infection
Bordetella pertussis (one exception for bacteria that increases lymphocytes - blocks lymphocytes from entering lymph node so stuck in blood)
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13
Q

Infectious mononucleosis

A

EBV - lymphocytic leukocytosis
CD8+ T cells

CMV less common cause

EBV - oropharynx (pharyngitis), liver, B cells

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14
Q

CD8+ T cell response for mono

A

Generalized LAD (paracortex hyperplasia)
Splenomegaly (periarterial lymphatic sheath)
High white count with atypical lymphocytes (CD8+ T cells)

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15
Q

Monospot test

A

IgM antibodies cross react with horse or sheep RBC (heterophile antibodies)
Turns + after 1 week
Definitive dx: serologic testing for EBV viral capsid antigen

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16
Q

Complications of EBV

A

increased risk of splenic rupture
Rash w/ PCN exposure
Dormancy of virus in B cells - risk for recurrence and B cell lymphoma

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17
Q

Acute leukemia

A

neoplastic proliferation of blasts

> 20% blasts in bone marrow
blasts crowd out normal hematopoiesis

Acute presentation w/ anemia, thrombocytopenia, neutropenia

blasts enter blood resulting in increased WBC on smear

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18
Q

Blast characteristics

A

large, immature cells w/ punched out nucleoli

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19
Q

Myeloblasts

A

AML

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20
Q

Lymphobasts

A

ALL

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21
Q

Key marker for lymphoblasts

A

TdT+ found in the nuclues

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22
Q

Key marker for myeloblasts

A

MPO+

  1. chemical study to detect enzyme
  2. Crystalize into auer rod seen by microscope
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23
Q

ALL

A

Neoplastic accumulation of lymphoblasts
+ TdT (DNA polymerase)
TdT absent in myeloid blasts and mature lymphocytes

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24
Q

ALL associated with what syndrome after age 5

A

Downs Syndrome

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25
Q

ALL subclassified into

A

B-ALL
T-ALL
based on surface markers

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26
Q

Most common type of ALL

A

B-ALL

Lymphocytes extpress CD10, CD19, CD20

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27
Q

B-ALL TX

A

excellence response to CTX

Prophylaxis to scrotum and CSF

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28
Q

B-ALL prognosis based on cytogenetic abnormalities

A

t(12,21) good prognosis - more in kids

t(9.22) poor prognosis - in adults (Ph+ ALL)

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29
Q

T-ALL

A

Lymphoblasts express markers ranging from CD2-8

Blasts do not express CD10

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30
Q

T-ALL - mass where

A

Thymic mass (mediastinal)
Teenager
Not in blood so it’s a lymphoma
Acute Lymphoblastic Lymphoma

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31
Q

AML

A

neoplastic growth of myeloblasts
Characterized by MPO
Crystal aggregates of MPO seen as Auer Rods

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32
Q

Subclassification of AML

A

cytogenetic abnormalities
Lineage of myeloblasts
Surface markers

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33
Q

AML subclass APL

A

Acute promyelocytic leukemia
t(15,17)
RAR receptor disrupted; promyelocytes accumulate
Promyelocytes contain numerous Auer rods - risk for DIC
ATRA causes blasts to mature (all trans retinoic acid)

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34
Q

AML subclass Acute monocytic leukemia

A

Proliferation of monoblasts - lack MPO

Blasts infiltrate gums

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35
Q

AML subclass Acute magaryoblastic leukemia

A

Proliferation of megakaryoblasts - lack MPO

Associated with Downs Syndrome before age 5

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36
Q

Prior exposure to what 2 things cause dysplasia and risk for AML

A

alkylating agents

radiotherapy

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37
Q

Myelodysplastic sydnrome

A

Cytopenia w/ hypercellular bone marrow

Abnormal maturation with increased blasts (20% blasts)

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38
Q

Chronic leukemia

A

Neoplastic proliferation of mature circulating lymphocytes
High white blood cell count
Insidious in onset
Older adults

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39
Q

CLL

A

Neoplastic proliferation of Naive B cells
Cells co-express CD5 & CD20
Increased lymphocytes and smudge cells on blood smear

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40
Q

Smudge cells seen in what leukemia

A

CLL

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41
Q

CLL involves lymph node

A

Generalized LAD

Called small lymphocytic lymphoma

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42
Q

Complications of CLL

A

hypogammaglobulinemia
Autoimmune hemolytic anemia (antibody against own RBC)
Transform to diffuse large B cell lymphoma

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43
Q

Most common cause of death in CLL

A

infection from hypogammaglobinemia

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44
Q

Chronic leukemia: Hairy cell leukemia

A

Neoplastic proliferation of mature B cells
Characterized by hairy cytoplasmic processes
TRAP +

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45
Q

Hairy cell leukemia clinical features

A

Splenomegaly (red pulp)
Dry tap w/ bone marrow aspiration
LAD absepnt

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46
Q

TRAP

A

T: tartate resistant
R: red pulp
A: absent LAD

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47
Q

TX for Hairy cell Leukemia

A

2-CDA: cladribine
Adenosine deaminase inhibitor
Adenosine accumulates to toxic levels in neoplastic B cells

48
Q

Chronic leukemia: ATLL

A

Adult T-Cell Leukemia/Lymphoma
Neoplastic proliferation of CD4+ T cells
HTLV-1 (japan/caribbean)

49
Q

Clinical features of ATLL

A

Rash
Generalized LAH w/ HSM
Lytic bone lesions with hypercalcemia

50
Q

Chronic leukemia: mycosis fungoides

A

Neoplastic proliferation of mature CD4+ T cells

cells produce rash, plaques, nodules
Aggregates of neoplastic T cells in epidermis called Pautrier microabscesses

51
Q

Pautrier microabscesses

A

Aggregates of neoplastic T cells in epidermis in mycosis fungoides

52
Q

Sezary syndrome

A

Mycosis fungoides
cells spread to blood
cells have cerebriform nuclei

53
Q

Myeloproliferative disorders (MPD)

A

Neoplastic proliferation of mature cells with myeloid lineage

Dz of late adulthood
High WBC count w/ hypercellular bone marrow (granulocytes increased)

Cells of all myeloid lineages are increased - classified based on dominant myeloid cell produced

54
Q

Complications of MPD

A

hyperuricemia and gout
Progression to marrow fibrosis
Transformation to acute leukemia

55
Q

CML

A

Chronic myeloid leukemia
Neoplastic proliferation of mature myeloid cells - especially granulocyes
Basophils increased

t(9;22) - philadelphia chromosome

56
Q

Translocation in CML

A

t(9,22) - fusion of BCR-ABL

fusion with increased tyrosine kinase activity)

57
Q

First line tx of CML

A

imantinib

Blocks tyrosine kinase activity

58
Q

Tranformation of CML to

A

AML (2/3)
ALL (1/3)
mutation is in a pluripotent stem cell

59
Q

CML vs leukemoid rxn

A

CML granulocytes LAP -
CML = increased basophils
CML granulocytes have t(9,22)

60
Q

MPD: polycythemia vera

A

Neoplastic proliferation of mature myeloid cells - RBC
Granulocytes and platelets increased
JAK2 kinase mutation

61
Q

PV clincal symptoms

A 
Hyperviscosity
Blurred vision, HA
Increased risk venous thrombosis
Flushed face d/t congestion 
Itching after bathing
62
Q

Most common cause of budd-chiari syndrome

A

PC

Hepatic vein venous thrombosis

63
Q

TX PV

A

phelobotomy
2nd line - hydroxyurea
Death in 1 year w/o tx

64
Q

PV vs reactive polycythemia

A

PV: SaO2 normal & EPO decreased
Reactive polycythemia d/t lung dz - SaO2 low & EPO increased
Reactive polycythemia d/t ectopic EPO production - EPO high and SaO2 normal (RCC commonly does this)

65
Q

MPD: Essential Thrombocythemia

A

Neoplastic proliferation of mature myeloid cells - esp platelets
RBC & granulocytes increased
JAK2 kinase mutation

66
Q

Essential Thrombocythemia symptoms

A

Increased risk bleeding/thrombosis

Rarely marrow fibrosis, acute leukemai, hyperuricemia, gout

67
Q

MPD: Myelofibrosis

A

Megakaryocytes
JAK2 kinase mutation
Produced exsses PDGF causing marrow fibrosis

68
Q

Clincal featuers of myelofibrosis

A

Splenomegaly (d/t extramedullary hematopoiesis)
Leukoerythroblastic smear (tear drop RBC, nucleated RBC, immature granulocytes - normally reticulin gate blocks premature cells from exiting)
Increased risk of infection, thrombosis, bleeding (BM can’t properly produce and spleen can’t keep up)

69
Q

LAD

A

enlarge lymph nodes

painful: acute infection
painless: chronic inflammation, metastatic carcinoma, lymphoma

70
Q

Inflammation - enlargement is d/t hyperplasia of LN regions

  1. follicles
  2. paracortex
  3. sinus histiocytes
A
  1. follicles: RA/Early HIV
  2. paracortex: virus
  3. Sinus histiocytes: LN draining tissue w/ cancer
71
Q

Lymphoma

A

neoplastic proliferation of lymphoid cells that form mass

LN or extranodal tissues

72
Q

Lymphoma divisions

A 
Non Hodgkin (60%)
Hodgkin (40%)
73
Q

NHL classification

A

Cell type/size
Pattern of growth
Expression of surface markers
Cytogenetic translocations

74
Q

B cell types of NHL (based on cell size)

A

small, intermediate, large

75
Q

Small B cell NHL

A

Follicular, Mantle, Marginal (small lymphocytic lymphoma)

76
Q

Follicular Lymphoma (NHL)

A

Neoplastic small B cell (CD20+) make follicle like nodules
Late adulthood w/ painless LAD

t(14,18)

77
Q

Translocation of Follicular Lymphoma

A

t(14,18)
14- IgH normally - but translocates with BCL2 (normally on 18)
Overexpression of BLC2 (inhibits apoptosis)

78
Q

TX of follicular lymphoma

A

Low dose chemo or rituximab

79
Q

Complication of Follicular lymphoma

A

Progression to diffuse large B cell lymphoma

Presents as enlarging LN

80
Q

Follicular lymphoma vs follicular hyperplasia

A

Disruption of normal LN architecture
Lack on tingible body macrophages in GC
Expression of BCL2 in follicles - normal follicles don’t have BCL
Monoclonality (follicular hyperplasia is polyclonal)

81
Q

Mantle Cell lymphoma

A

Neoplastic small B cell (CD20+) expands mantle zone (region immediately adjacent to follicle)
Late adulthood, painless LAD

t(11,14)
IgH on 14
Cyclin D1 on 11
Overexpression of cyclin D1 promotes G1/S transition in cell cycle

82
Q

Mantle cell lymphoma translocation

A

t(11,14)
IgH on 14
Cyclin D1 on 11
Overexpression of cyclin D1 promotes G1/S transition in cell cycle

83
Q

Marginal zone lymphoma

A

Neoplastic small B cell (CD20+) expans marginal zone

84
Q

Marginal zone lymphoma associated with

A

inflammatory states:
Hashimoto’s thryoiditis
Sjogren
H pylori gastritis

85
Q

Most lymph nodes don’t have marginal zone - what forms it?

A

post-geminal center B cells in chronic inflammation states

86
Q

MALToma

A

marginal zone lymphoma in mucosal sties

87
Q

Intermediate size B cell lymphoma

A

Burkitt Lymphoma

88
Q

Burkitt lymphoma

A

Neoplastic intermediate-sized B cell (CD20+)

EBV
extranodal mass in child/young adult
African: jaw mass
Sporadic: abdomen

C-myc (chromosome 8) translocation

89
Q

Burnkitt Lymphoma translocation

A 
C-myc on chromosome 8
t(8,14)
IgH on chromosome 14
C-myc on 8
Overexpression of c-myc oncogene promotes cell growth
90
Q

Burkitt Lymphoma histology

A

Starry sky
High mitotic rate
Blue cells are tumor “sky”
Cells dying -so macrophages eat cells as they die “star”

91
Q

Large size C cell lymphoma

A

Diffuse Large B cell lymphoma

92
Q

DLBCL

A

neoplastic large B cell (CD20+) grows diffusely in sheets
Most common form of NHL
Aggressive

93
Q

Most common form of NHL

94
Q

DLBCL arises from

A

sporadically or from transformation of follicular lymphoma

Late adulthood/enlarging LN or extranodal mass

95
Q

Hodgkin lymphoma

A

Neoproliferation of Reed-Sternberg cells - large B cell with multilobed nuclei and prominent nucleoli
CD15+ & CD30+

96
Q

Reed-Sternberg cells

A

Owl eyed nuclei
CD15+ and CD30+
Secrete cytokines - attract reactive lymphocytes, plasma cells, macrophages, eosinophils

97
Q

B symptoms

A

fever, chills, night sweats

HL symptoms

98
Q

HL classification

A

Nodular sclerosis (70%)
Lymphocyte rich
Mixed cellularity
Lymphocyte depleted (minimal lymphocytes associated)

99
Q

Nodular sclerosis most common type of HL

A

Presentation:
enlarging cervical neck or mediastinal LN in young adult, usually female

LN divided by bands of sclerosis - cut LN into nodules

RS cells present in lake like spaces “lacunar cells”

100
Q

Best prognosis of HL

A

lymphocyte rich

101
Q

Worst prognosis HL

A

Lymphocyte depleted

elderly/HIV

102
Q

Mixed cellularity HL associated with

A

eosinophils (IL-5)

103
Q

Plasma cells disorders also called

104
Q

Multiple Myeloma

A

malignant proliferation of plasma cells in bone marrow
Most common malignancy of bone
IL-6

105
Q

Most common primary malignancy of bone

A

Multiple myeloma

106
Q

IL-6

A

Stimulates plasma cell growth and immunoglobin production

107
Q

Mutliple myeloma symtpoms

A

Bone pain with hypercalacemia (RANK receptor)
-lytic punched out skeletal lesions
Immuoglobin - elevated serum protein (M spike on SPEP) - d/t monoclonal igG or IgA
Increased risk of infection (d/t lack of antigenic diversity from monoclonal antibody) - infection most common cause of death
Rouleaux formation - decreased charge between RBC (high serum protein)
Plasma cells over produce light chain - primary AL amyloidosis (deposits in tissues)
Proteinuria - Bence Jones proteins (light chains extreted in urine), Myeloma kidney - light chains depostied in kidney tubules (renal failure)

108
Q

Most common cause of death in MM

109
Q

MGUS

A

plasma cell dysrcrasia
Increased serum protein with M spike
No other MM features (no lytic lesions, hypercalcemia, AL amyloid or Bence Jones proteinuria)

Common in edlery
1% will develop MM

110
Q

Waldenstrom Macroglobulinemia

A

B cell lymphoma w/ monoclonal IgM production

111
Q

Waldenstrom Macroglobulinemia clinical features

A 
Generalized LAD 
Lytic bone lesions absent
Increased protien with M spike (IgM)
Visual & neurologic deficits (retinal hemorrhage or stroke)
Bleeding
112
Q

Waldenstrom Macroglobulinemia acute complications tx

A

plasmaphoresis

113
Q

Langerhans Cell histiocytosis

A

Langerhans - specialized dendritic cell in skin
Derived from bone marrow monocytes
Present antigen to Naive T cell

Histiocytosis: neoplastic proliferation
Characteristic Birbeck ganules
CD1a+ and S100 +

114
Q

Letter Siwe dz

A

Langerhan cell - malignant prolfieration
2 names malignant & skin rash
Less than 2 yars of age
Multiple organs involved - rapidly fatal

115
Q

Eosinophilic granuloma

A 
No name, benign
Proliferation of Langerhan Cells in bone
Adolescents
Skin not involved
Biopsy shows Langerhans cells with mixed inflammatory cells - numerous eosinophils
116
Q

Hand-Schuller Christian Dz

A 
3 names (malignant - skin involvment) over age of 3
Scalp rash, lytic skull defects, diabetes insipidus, exophthalmos in child

pathoma (12 decks)

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