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white blood cell disorders Flashcards

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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
ALL subclassified into
B-ALL T-ALL based on surface markers
26
Most common type of ALL
B-ALL | Lymphocytes extpress CD10, CD19, CD20
27
B-ALL TX
excellence response to CTX | Prophylaxis to scrotum and CSF
28
B-ALL prognosis based on cytogenetic abnormalities
t(12,21) good prognosis - more in kids t(9.22) poor prognosis - in adults (Ph+ ALL)
29
T-ALL
Lymphoblasts express markers ranging from CD2-8 | Blasts do not express CD10
30
T-ALL - mass where
Thymic mass (mediastinal) Teenager Not in blood so it's a lymphoma Acute Lymphoblastic Lymphoma
31
AML
neoplastic growth of myeloblasts Characterized by MPO Crystal aggregates of MPO seen as Auer Rods
32
Subclassification of AML
cytogenetic abnormalities Lineage of myeloblasts Surface markers
33
AML subclass APL
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)
34
AML subclass Acute monocytic leukemia
Proliferation of monoblasts - lack MPO | Blasts infiltrate gums
35
AML subclass Acute magaryoblastic leukemia
Proliferation of megakaryoblasts - lack MPO | Associated with Downs Syndrome before age 5
36
Prior exposure to what 2 things cause dysplasia and risk for AML
alkylating agents | radiotherapy
37
Myelodysplastic sydnrome
Cytopenia w/ hypercellular bone marrow | Abnormal maturation with increased blasts (20% blasts)
38
Chronic leukemia
Neoplastic proliferation of mature circulating lymphocytes High white blood cell count Insidious in onset Older adults
39
CLL
Neoplastic proliferation of Naive B cells Cells co-express CD5 & CD20 Increased lymphocytes and smudge cells on blood smear
40
Smudge cells seen in what leukemia
CLL
41
CLL involves lymph node
Generalized LAD | Called small lymphocytic lymphoma
42
Complications of CLL
hypogammaglobulinemia Autoimmune hemolytic anemia (antibody against own RBC) Transform to diffuse large B cell lymphoma
43
Most common cause of death in CLL
infection from hypogammaglobinemia
44
Chronic leukemia: Hairy cell leukemia
Neoplastic proliferation of mature B cells Characterized by hairy cytoplasmic processes TRAP +
45
Hairy cell leukemia clinical features
Splenomegaly (red pulp) Dry tap w/ bone marrow aspiration LAD absepnt
46
TRAP
T: tartate resistant R: red pulp A: absent LAD
47
TX for Hairy cell Leukemia
2-CDA: cladribine Adenosine deaminase inhibitor Adenosine accumulates to toxic levels in neoplastic B cells
48
Chronic leukemia: ATLL
Adult T-Cell Leukemia/Lymphoma Neoplastic proliferation of CD4+ T cells HTLV-1 (japan/caribbean)
49
Clinical features of ATLL
Rash Generalized LAH w/ HSM Lytic bone lesions with hypercalcemia
50
Chronic leukemia: mycosis fungoides
Neoplastic proliferation of mature CD4+ T cells cells produce rash, plaques, nodules Aggregates of neoplastic T cells in epidermis called Pautrier microabscesses
51
Pautrier microabscesses
Aggregates of neoplastic T cells in epidermis in mycosis fungoides
52
Sezary syndrome
Mycosis fungoides cells spread to blood cells have cerebriform nuclei
53
Myeloproliferative disorders (MPD)
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
Complications of MPD
hyperuricemia and gout Progression to marrow fibrosis Transformation to acute leukemia
55
CML
Chronic myeloid leukemia Neoplastic proliferation of mature myeloid cells - especially granulocyes Basophils increased t(9;22) - philadelphia chromosome
56
Translocation in CML
t(9,22) - fusion of BCR-ABL | fusion with increased tyrosine kinase activity)
57
First line tx of CML
imantinib | Blocks tyrosine kinase activity
58
Tranformation of CML to
AML (2/3) ALL (1/3) mutation is in a pluripotent stem cell
59
CML vs leukemoid rxn
CML granulocytes LAP - CML = increased basophils CML granulocytes have t(9,22)
60
MPD: polycythemia vera
Neoplastic proliferation of mature myeloid cells - RBC Granulocytes and platelets increased JAK2 kinase mutation
61
PV clincal symptoms
``` Hyperviscosity Blurred vision, HA Increased risk venous thrombosis Flushed face d/t congestion Itching after bathing ```
62
Most common cause of budd-chiari syndrome
PC | Hepatic vein venous thrombosis
63
TX PV
phelobotomy 2nd line - hydroxyurea Death in 1 year w/o tx
64
PV vs reactive polycythemia
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
MPD: Essential Thrombocythemia
Neoplastic proliferation of mature myeloid cells - esp platelets RBC & granulocytes increased JAK2 kinase mutation
66
Essential Thrombocythemia symptoms
Increased risk bleeding/thrombosis | Rarely marrow fibrosis, acute leukemai, hyperuricemia, gout
67
MPD: Myelofibrosis
Megakaryocytes JAK2 kinase mutation Produced exsses PDGF causing marrow fibrosis
68
Clincal featuers of myelofibrosis
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
LAD
enlarge lymph nodes painful: acute infection painless: chronic inflammation, metastatic carcinoma, lymphoma
70
Inflammation - enlargement is d/t hyperplasia of LN regions 1. follicles 2. paracortex 3. sinus histiocytes
1. follicles: RA/Early HIV 2. paracortex: virus 3. Sinus histiocytes: LN draining tissue w/ cancer
71
Lymphoma
neoplastic proliferation of lymphoid cells that form mass | LN or extranodal tissues
72
Lymphoma divisions
``` Non Hodgkin (60%) Hodgkin (40%) ```
73
NHL classification
Cell type/size Pattern of growth Expression of surface markers Cytogenetic translocations
74
B cell types of NHL (based on cell size)
small, intermediate, large
75
Small B cell NHL
Follicular, Mantle, Marginal (small lymphocytic lymphoma)
76
Follicular Lymphoma (NHL)
Neoplastic small B cell (CD20+) make follicle like nodules Late adulthood w/ painless LAD t(14,18)
77
Translocation of Follicular Lymphoma
t(14,18) 14- IgH normally - but translocates with BCL2 (normally on 18) Overexpression of BLC2 (inhibits apoptosis)
78
TX of follicular lymphoma
Low dose chemo or rituximab
79
Complication of Follicular lymphoma
Progression to diffuse large B cell lymphoma | Presents as enlarging LN
80
Follicular lymphoma vs follicular hyperplasia
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
Mantle Cell lymphoma
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
Mantle cell lymphoma translocation
t(11,14) IgH on 14 Cyclin D1 on 11 Overexpression of cyclin D1 promotes G1/S transition in cell cycle
83
Marginal zone lymphoma
Neoplastic small B cell (CD20+) expans marginal zone
84
Marginal zone lymphoma associated with
inflammatory states: Hashimoto's thryoiditis Sjogren H pylori gastritis
85
Most lymph nodes don't have marginal zone - what forms it?
post-geminal center B cells in chronic inflammation states
86
MALToma
marginal zone lymphoma in mucosal sties
87
Intermediate size B cell lymphoma
Burkitt Lymphoma
88
Burkitt lymphoma
Neoplastic intermediate-sized B cell (CD20+) EBV extranodal mass in child/young adult African: jaw mass Sporadic: abdomen C-myc (chromosome 8) translocation
89
Burnkitt Lymphoma translocation
``` 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
Burkitt Lymphoma histology
Starry sky High mitotic rate Blue cells are tumor "sky" Cells dying -so macrophages eat cells as they die "star"
91
Large size C cell lymphoma
Diffuse Large B cell lymphoma
92
DLBCL
neoplastic large B cell (CD20+) grows diffusely in sheets Most common form of NHL Aggressive
93
Most common form of NHL
DLBCL
94
DLBCL arises from
sporadically or from transformation of follicular lymphoma Late adulthood/enlarging LN or extranodal mass
95
Hodgkin lymphoma
Neoproliferation of Reed-Sternberg cells - large B cell with multilobed nuclei and prominent nucleoli CD15+ & CD30+
96
Reed-Sternberg cells
Owl eyed nuclei CD15+ and CD30+ Secrete cytokines - attract reactive lymphocytes, plasma cells, macrophages, eosinophils
97
B symptoms
fever, chills, night sweats | HL symptoms
98
HL classification
Nodular sclerosis (70%) Lymphocyte rich Mixed cellularity Lymphocyte depleted (minimal lymphocytes associated)
99
Nodular sclerosis most common type of HL
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
Best prognosis of HL
lymphocyte rich
101
Worst prognosis HL
Lymphocyte depleted | elderly/HIV
102
Mixed cellularity HL associated with
eosinophils (IL-5)
103
Plasma cells disorders also called
dysrpasia
104
Multiple Myeloma
malignant proliferation of plasma cells in bone marrow Most common malignancy of bone IL-6
105
Most common primary malignancy of bone
Multiple myeloma
106
IL-6
Stimulates plasma cell growth and immunoglobin production
107
Mutliple myeloma symtpoms
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
Most common cause of death in MM
infection
109
MGUS
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
Waldenstrom Macroglobulinemia
B cell lymphoma w/ monoclonal IgM production
111
Waldenstrom Macroglobulinemia clinical features
``` Generalized LAD Lytic bone lesions absent Increased protien with M spike (IgM) Visual & neurologic deficits (retinal hemorrhage or stroke) Bleeding ```
112
Waldenstrom Macroglobulinemia acute complications tx
plasmaphoresis
113
Langerhans Cell histiocytosis
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
Letter Siwe dz
Langerhan cell - malignant prolfieration 2 names malignant & skin rash Less than 2 yars of age Multiple organs involved - rapidly fatal
115
Eosinophilic granuloma
``` No name, benign Proliferation of Langerhan Cells in bone Adolescents Skin not involved Biopsy shows Langerhans cells with mixed inflammatory cells - numerous eosinophils ```
116
Hand-Schuller Christian Dz
``` 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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