WBC disorders Pathoma Flashcards

1
Q

Leukopenia

A

low WBC count (less than 5K)

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2
Q

Leutocytosis

A

high WBC count (above 10K)

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3
Q

Myeloid Stem cells can become

A

Erythroblast (RBC), Myeloblast (granulocytes = neutrophils, basophils), monoblast (monocytes), & megakaryocytes

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4
Q

Lymphoid stem cells can become

A

B Cells (plasma cells) & T cells (CD8 & CD4)

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5
Q

Neutropenia

A

Low neutrophils
Caused by drug toxicity (chemotherapy) & severe infection (out of blood & in tissue)
Treated with granulocyte stimulating factor

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6
Q

Lymphopenia

A

Low number of circulating lymphocytes
Caused by immunodeficiency , high cortisol state (induce apoptosis), autoimmune destruction, whole body radiation (most sensitive cell in body to radiation)

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7
Q

Neutrophilic leukocytosis

A

Increase in neutrophils
Caused by bacterial infection, tissue necrosis, & high cortisol states
Bone marrow releases immature neutrophils (left shift) - decreased Fc receptors
CD 16 is marker for FC receptor (also decreased)

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8
Q

Monocytosis

A

Increases WBC count

Seen in chronic inflammatory states & malignancy

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9
Q

Eosinophilia

A

Increased number in circulating eosinophils

Caused by allergic reactions, parasitic infection, & Hodgkin lymphoma (by increased IL-5 production)

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10
Q

Basophilia

A

High basophil count

Seen in CML

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11
Q

Lymphocytic leukocytosis

A

High lymphocyte count
Seen in viral infections (fought by CD8 T cells) & Bordetella pertussis (even though it is a bacteria - one exception; blocks lymphocytes from entering lymph nodes - stuck in blood)

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12
Q

Infectious Mononucleosis

A

EBV infection that results in a lymphocytic leukocytosis comprised of reactive CD8 T cells
CMV is less common case
Risk splenomegaly & rupture - avoid contact sports for 1 year

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13
Q

Acute leukemia is a neoplastic proliferation of

A

blasts

over 20% blasts in bone marrow; 1-2% is normal number

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14
Q

As blasts pile up, they cause

A

acute presentation of anemia, thrombocytopenia, or neutropenia (as they crowd out normal hematopoiesis)

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15
Q

Blasts enter blood, resulting in

A

high WBC count.

Look like large, immature cells with punched out nucleoli

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16
Q

Accumulation of myeloid blast is

A

AML

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17
Q

Accumulation of lymphoblast is

A

ALL

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18
Q

Hallmark marker for lymphoblast is

A

TdT in nucleus - DNA polymerase only present in lymphoblasts

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19
Q

Marker for myeloid blast is

A

MPO
(myeloperoxidase)
Can crystalize into structure called Auer Rod

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20
Q

ALL most commonly arises in

A

Children

especially associated with Down syndrome (after age 5)

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21
Q

Most common type of ALL

A

B-ALL

Classical surface markers CD10, CD19, CD20

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22
Q

B-ALL prognosis

A

t(12;21) good prognosis - in children

t(9;22) poor prognosis - more common in adults (Ph+ ALL)

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23
Q

T-ALL markers

A

CD2-CD8

Do not express CD10

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24
Q

T-ALL commonly presents in

A

thymic (mediastinal) mass in teenager

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25
Q

Auer Rod

A

seen in AML

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26
Q

Acute promylocytic leukemia

A

Characterized by t(15;17)
Disruption of RAR (retinoic acid receptor) - promyelocytes accumulate
Risk for DIC (activation of coagulation casscade by auer rods)
Treated by ATRA

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27
Q

Acute monocytic leukemia

A

proliferation of monoblasts; lack MPO

Infiltrate gums

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28
Q

Acute megakaryoblastic leukemia

A

Proliferation of megakaryoblasts; lack MPO

Association with Down Syndrome (before age of 5)

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29
Q

Myelodysplastic syndrome

A

Cytopenias (low count of cells in blood) with hypercellular bone marrow
(abnormal maturation with increased blasts,

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30
Q

Chronic leukemia

A

neoplastic proliferation of mature circulating lymphocytes.

High WBC, usually insidious onset & in older adults

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31
Q

CLL

A

Neoplastic proliferation of naive B-cells

Co-express CD5 & CD20

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32
Q

CLL blood smear shows

A

Increased lymphocytes & smudge cells

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33
Q

Small lymphocytic lymphoma

A

CLL that involves the lymph nodes causing generalized lymphadenopathy

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34
Q

CLL complications

A

hypogammaglobulinemia (low immunoglobulin - die of infection)
Autoimmune hemolytic anemia (antibodies against own RBC)
Transformation to diffuse large B-cell lymphoma (enlarging lymph node or spleen)

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35
Q

Hairy Cell Leukemia

A

Neoplastic proliferation of mature B cells
Hairy cytoplasmic processes
Positive for TRAP

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36
Q

Hairy Cell Leukemia clinical features

A

Splenomegaly (red pulp expansion)
Dry tap with bone marrow aspiration
Lymphadenopathy usually absent

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37
Q

Hairy Cell Leukemia treated with

A

2-CDA (adenosine deaminase inhibitor)

Adenosine accumulates to toxic levels in neoplastic B cells

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38
Q

ATLL

A

Neoplastic proliferation of mature CD4 T cells

Associated with HTLV-1 (Japan & Caribbean)

39
Q

ATLL clinical features

A

Rash
Generalized lymphadenopathy with hepatosplenomegaly
Lytic bone lesions with hypercalcemia

40
Q

Mycosis fungoides

A

neoplastic proliferation of mature CD4 T cells

Infiltrate skin - rash, plaques, or nodules

41
Q

Pautrier microabscesses

A

aggregates of neoplastic T cells in epidermis

42
Q

Sezary syndrome

A

CD4 T cells (in mycosis fungoides) spread to blood

Cerebriform nuclei on blood smear

43
Q

Myeloproliferative Disorder

A

Overproduction of mature myeloid cells
Get overproduction of all myeloid cells (RBC, granuclocytes, etc.) but named based on predominant cell
Late adulthood disease
High WBC count with hypercellular bone marrow

44
Q

Myeloproliferative disorder complications

A

Increased risk for hyperuricemia & gout
Progression to marrow fibrosis
Transformation to acute leukemia

45
Q

Chronic Myeloid Leukemia (CML)

A

Neoplastic proliferation of mature myeloid cells, especially granulocytes
Basophils characteristically increased

46
Q

CML translocation

A

t(9;22) - Philadelphia chromosome

BCR-ABL fusion with increased tyrosine kinase activity

47
Q

CML treatment

A

Imatinib - blocks tyrosine kinase activity

48
Q

CML chronic phase

A

Enlarged spleen

49
Q

Enlarging spleen (getting bigger) is marker of (what CML phase)

A

Accelerated phase; transformation to acute leukemia follows shortly (either AML or ALL)

50
Q

CML granulocytes are

A

LAP negative (leukemoid reaction are LAP positive)

51
Q

Polycythemia Vera

A

Neoplastic proliferation of mature myeloid cells, especially RBC
EPO decreased due to negative feedback
Granulocytes & platelets also increased
JAK2 kinase mutation

52
Q

Polycythemia Vera symptoms

A
hyperviscosity
Blurry vision & headache
Increased risk of venous thrombosis
Flushed face due to congestion
Itching after bathing (high number of mast cells)
53
Q

Polycythemia Vera treatment

A

Phlebotomy

2nd line treatment hydroxyurea

54
Q

Reactive polycythemia

A

Polycythemia due to lung disease (has SaO2 low & EPO increased)
Due to ectopic EPO production, EPO is high & SaO2 is normal

55
Q

Essential Thrombocythemia

A

Neoplastic proliferation of mature myloid cells - platelets!
RBC & granulocytes also increased
JAK2 mutation

56
Q

Essential Thrombocythemia symptoms

A

Increased risk of bleeding and/or thrombosis
Rarely develops into marrow fibrosis or acute leukemia
No risk for hyperuricemia or gout

57
Q

Myelofibrosis

A

Neoplastic proliferation of mature myeloid cells, esp megakaryocytes
JAK2 mutation

58
Q

Myelofibrosis

A

Splenomegaly due to extramedullary hematopoiesis
Leukoerythroblastic smear
Increased risk of infection, thrombosis, & bleeding
Teardrop cells

59
Q

Lymphadenopathy

A

enlarged lymph nodes
Painful with acute infection
Painless with chronic inflammation, metastatic carcinoma, or lymphoma

60
Q

Lymphoma

A

neoplastic proliferation of lymphoid cells that forms a mass

Can be in lymph node or extranodal tissue

61
Q

Most common lymphomas

A

Non-Hodgkin lymphomas

62
Q

Follicular lymphoma

A

Neoplastic small B cells (CD20) that make the follicle-like nodules - disrupts normal lymph node architecture
Late adulthood with painless LAD
Driven by t(14;18), over-expression of BCL2

63
Q

BCL2

A

Stabilizes mitochondrial membrane & prevents cytochrome c from leaking into the cytoplasm
Blocks apoptosis

64
Q

Follicular lymphoma treatment

A

low dose CTX or rituximab

for symptomatic patients

65
Q

Follicular lymphoma complications

A

Progression to diffuse large B-cell lymphoma - presents as enlarging lymph node

66
Q

Mantle Cell Lymphoma

A

Neoplastic small B cells (CD20) that expand mantle zone (immediately adjacent to follicle)
Late adulthood with painless LAD
Driven by t(11;14) - Cyclin D1 overexpression

67
Q

Cyclin D1

A

promotes G1/S transition in cell cycle through protein phosphorylation

68
Q

Marginal Zone Lymphoma

A

Neoplastic small B cells (CD20) that expand marginal zone (outside of the mantle)
Associated with chronic inflammatory states (like Hashimoto thyroiditis, Sjogren syndrome, H pylori gastritis)

69
Q

MALToma

A

marginal zone lymphoma in mucosal sites (stomach example)

Gastric MALToma may regress with treatment of H pylori

70
Q

Burkitt Lymphoma

A

Neoplastic intermediate-sized B cells

Associated with EBV

71
Q

Burkitt Lymphoma presents as

A

extranodal mass in child or young adult
African form - jaw
Sporadic form - abdomen

72
Q

Burkitt lymphoma is driven by

A

translocations of c-myc (chromosome 8)
t(8;14) most common
Overexpression of c-myc oncogene promotes cell growth

73
Q

Burkitt lymphoma looks like _____ on histology

A

starry-sky appearance

Due to rapid growth & turnover

74
Q

DLBCL (Diffuse large b cell lymphoma)

A
Neoplastic large B cells that grow diffusely in sheets
Clinically aggressive (poorly differentiated)
75
Q

Most common type of nonHodgkin lymphoma is

A

DLBCL

76
Q

DLBCL presents

A

late in adulthood as enlarging lymph node or extranodal mass

Arise sporadically or transformation of follicular lymphoma

77
Q

Hodgkin Lymphoma

A

Reed-Sternberg cells - Large B cell with multilobed nuclei & prominent nucleoli
CD15 & CD30

78
Q

RS cells secrete cytokines that

A

Can cause B symptoms (fevers, chills, night sweats)
Attract reactive lymphocytes, plasma cells, macrophages, & eosinophils
May lead to fibrosis

79
Q

Most common type of HL is

A

Nodular sclerosis:
Enlarging cervical neck or mediastinal LN & usually a young female
LN divided by broad bands of fibrosis
RS cells sit in big empty spaces

80
Q

Multiple Myeloma

A

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

81
Q

Osteoclast activating factor

A

Can be produced by plasma cells
Activate osteoclasts that eat away at bone - punched out lesions on x-ray seen in vertebrae & skull
Leads to bone pain, hypercalcemia, & increase risk of fracture

82
Q

M spike

A

indicates monoclonal immunoglobulin

Tight & high band on SPEP

83
Q

Most common cause of death from multiple myeloma is

A

infection

Monoclonal antibody lacks antigenic diversity

84
Q

Immunoglobulin with MM

A

overproduced by plasma cells

Gives high serum protein with M spike (IgG or IgA) & decreased antigenic diversity

85
Q

Light chain production with MM

A

Overproduced by plasma cells
Deposits in tissue as amyloid (amylodosis) & comes out in urine as Bence-Jones proteins
Can deposit in kidney tubules & cause renal failure

86
Q

MGUS

A

Increased serum protein with M spike on SEP

Other features of multiple myeloma are absent

87
Q

MGUS common in

A

elderly

can develop into MM

88
Q

Waldenstrom Macroglobulinemia

A

B cell lymphoma with monoclonal IgM production
Presents with generalized LAD; absent lytic bone lesions
Increased serum protein with M spike (IgM)
Visual & neurological deficits
Bleeding

89
Q

Acute complications of Waldenstroms are treated with

A

plasmapheresis - removes IgM from serum

90
Q

Langerhans cells

A

specialized dendritic cell found mostly in skin

From bone marrow monocytes

91
Q

Langerhans cell histiocytosis

A
Neoplastic proliferation 
Characteristic Birbeck (tennis racket) cell
92
Q

Letterer-Siwe disease

A

Malignant proliferation of Langerhans cells

Classic presentation is skin rash and cystic skeletal defects in an infant (

93
Q

Eosinophilic granuloma

A

Benign proliferation of Langerhans cells in bone

Pathologic fracture in adolescent; skin not involved

94
Q

Hand-Schuller-Christian disease

A

Malignant proliferation of Langerhans cells
Scalp rash, lytic skull defects, diabetes insipidus, exopthalmos
In child