Hematology WBC disorders Flashcards

1
Q

CD34+

A

Hematopoetic Stem Cells

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2
Q

GM-CSF & G-CSF treat?

A

Neutropenia

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3
Q

Decreased CD16

A

CD16= Fc rec

Immature Neutrophils

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4
Q

WBC increased by Type I hypersensitivity, Hodgkin’s lymphoma

A

Eosinophils

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5
Q

WBC increased by CML

A

Basophils

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6
Q

WBC increased by Bordatella pertussis

A

Lymphocytes

Can’t leave circulation

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7
Q

CD8+ Tcell leukocytosis

A

EBV

sometimes CMV

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8
Q

TdT+ stain

Down Syndrome after 5

A

ALL

Acute Lymphoblastic Leukemia

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9
Q

CD10, CD19, CD20

A

B-ALL
MC vs. T-ALL
TdT+

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10
Q

t(12:21)

A

B-ALL
MC in kids, good prognosis
t(9:22) philadelphia
TdT+

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11
Q

CD2-CD8

Negative CD10

A

T-ALL

TdT+

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12
Q

MPO+

A

Acute Myeloid Leukemia AML

Auer rods

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13
Q

t(15:17)

A

Acute Promyelotic Leukemia

RAR (17) -> blocks maturation -> promyelocyte^ -> DIC risk^

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14
Q

Infiltrate gums

A

Acute Monocytic leukemia
Lacks MPO
AML subtype

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15
Q

Down Syndrome before age 5

A

Acute megakaryoblastic leukemia
Lack MPO
AML subtype

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16
Q

CD5 and CD20

MC leukemia overall

A
Chronic Lymphocytic Leukemia
Smudge cells
Hypogammaglobulinemia
Infection MCC of death
Autoimmune hemolytic anemia
Transformation (Richter) to Diffuse Large B-cell Lymphoma
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17
Q

TRAP+

A

Hairy Cell Leukemia

Lacks LAD

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18
Q

Splenomegaly (Red Pulp)

A

Hairy Cell Leukemia
Dry tap on BM aspiration due to fibrosis.
Lacks LAD

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19
Q

Responds to 2-CDA

A

Hairy Cell Leukemia

(cladribine) Adenosine deaminase inhibitor - causes adenosine to accumulate and becomes toxic in neoplastic B-cells

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20
Q

Associated with HTLV-1

A

Adult T-Cell Leukemia/lymphoma (ATLL)

MC seen in Japan and Caribbean

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21
Q

Rash, Generalized LAD with Hepatosplemnomegaly, and lytic (punched-out) bone lesions with hypercalcemia

A

Adult T-Cell Leukemia/Lymphoma (ATLL)

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22
Q

Neoplastic proliferation of mature CD4+ T cells

A

Adult T-Cell Leukemia/Lymphoma (ATLL)

Mycosis Fungoides

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23
Q

Skin rash, plaques, and nodules created by neoplastic cells

-Pautrier Microabscesses

A

Mycosis Fungoides

Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin

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24
Q

Sezary syndrome

A

Mycosis Fungoides
Cells spread to involve blood.
Characteristic lymphoctytes with cerebriform nuclei on blood smear

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25
Q

Neoplastic proliferation with average age of 50-60 years

A

Myeloproliferative disorders

CML, PV, ET, Myelofibrosis

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26
Q

Hypercellular BM
Increased risk for hyperuricemia and gout
Progression to BM fibrosis or transformation to acute leukemia

A

Myeloproliferative disorders
(CML, PV, ET, Myelofibrosis)
Classified based on dominant myeloid cell produced
Hyperuricemia and gout due to high turnover of cells

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27
Q

Characteristic Basophilia

A

Chronic Myeloid Leukemia (CML)

Increased mature myeloid cells

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28
Q

t(9:22)

A

Chronic Myeloid Leukemia (CML)
Philadelphia chromosome -> BCR-ABL fusion -> ^tyrosine kinase activity
LAP-

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29
Q

Imatinib treatment

A

Chronic Myeloid Leukemia (CML)
Blocks tyrosine kinase activity
Enlarging Spleen suggests progression to AML (2/3) or ALL (1/3)
LAP-

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30
Q

JAK2 kinase mutation

A
Polycythemia Vera (PV)
Essential Thrombocythemia (ET)
Myelofibrosis - 50%
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31
Q

Blurry Vision and HA
Risk of venous thrombosis
Flushed face (plethora)
Itching post bath

A

Polycythemia Vera (PV)
Venous thrombosis in hepatic vein, portal vein, dural sinus
Itching due to histamine release from mast cells
EPO decreased

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32
Q

Hydroxyurea treatment

A

Polycythemia Vera (PV)
1st line tx Phlebotomy
2nd line tx Hydroxyurea

EPO decreased in PV

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33
Q

Increased risk of bleeding and/or thrombosis

JAK2 mutation

A

Essential thrombocythemia (ET)
Rarely progress to BM fibrosis or acute leukemia
No significant risk of hyperuricemia or gout

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34
Q

Excess PDGF

A

Myelofibrosis
causes BM Fibrosis
JAK2 mutation

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35
Q

Splenomegaly
Leukoerythroblastic smear
Increased risk of infection, thrombosis, and bleeding

A

Myelofibrosis

  1. Splenomegaly due to extramedullary hematopoiesis
  2. Leukoerythroblastic smear (tear-drop RBCs, nucleated RBCs, and immature granulocytes

JAK2 mutation

36
Q

Painless LAD

A
Chronic inflammation (chronic lymphadenitis)
Metastatic carcinoma
Lymphoma
37
Q

Follicular hyperplasia

A

B-cell region seen with RA and early stages of HIV infection

38
Q

Paracortex hyperplasia

A

T-cell region seen with viral infection (Infectious mononucleosis)

39
Q

Hyperplasia of sinus histiocytes

A

Seen in LN that are draining a tissue with CA

40
Q

Small B-cell lymphomas

A

Follicular lymphoma
Mantle cell lymphoma
Marginal zone lymphoma
Small Lymphocytic lymphoma (CLL that involves tissues)

CD20+

41
Q

Intermediate-sized B cells lymphoma

A

Burkitt lymphoma

42
Q

Large B cells lymphoma

A

Diffuse large B-cell lymphoma

43
Q

CD20+ that form follicle-like nodules

A
Follicular Lymphoma (NHL)
Presents in late adulthood with painless LAD
44
Q

t(14:18)

A

Follicular Lymphoma (NHL)
BCL2 on 18 to
IG heavy chain on 14
BCL2 inhibits apoptosis

45
Q

Rituximab treatment for symptomatic patients

A
Follicular Lymphoma (NHL)
anti-CD20 antibody, low dose
46
Q

Follicular Lymphoma with enlarging Lymph Node

A

Risk for progressing to Diffuse Large B-cell Lymphoma

47
Q

Follicular Lymphoma characteristics vs Reactive follicular hyperplasia

A
  1. Disruption of normal lymph node architecture
  2. Lack of tingible body macrophages in germinal centers
  3. Bcl2 espression in follicles
  4. Monoclonality
48
Q

t(11:14)

A

Mantle Cell Lymphoma
Late adulthood with painless LAD

CyclinD1 on 11 to
Ig heavy chain on 14

49
Q

cyclin D1

A

Mantle Cell Lymphoma
t(11:14)

Promotes G1/S transition in the cell cycle

50
Q

Lymphoma associated with Chronic inflammatory states like Hashimoto thyroiditis, Sjogren syndrome, and H. pylori gastritis

A

Marginal Zone Lymphoma
Marginal zone is formed by post-germinal center B cells (CD20+)

MALToma in mucosal sites
- gastric MALToma may regress with tx of H. pylori

51
Q

Lymphoma associated with EBV

Extranodal mass in child or young adult

A

Burkitt Lymphoma

African - lower jaw
Sporadic - abdomen

52
Q

t(8:14)

A

Burkitt Lymphoma

Translocation of c-myc on 8
Ig Heavy chain on 14

c-myc promotes cell growth

53
Q

Starry-sky

A

Burkitt Lymphoma

Characterized by high mitotic index

54
Q

Large B cells (CD20+) that grow diffusely in sheets

A

Diffuse Large B-Cell Lymphoma

Clinically aggressive

55
Q

MC form of NHL

A

Diffuse Large B-Cell Lymphoma

Clinically aggressive

56
Q

Late adulthood as enlarging LN or extranodal mass

Sporadic formation or transformation of low grade lymphoma

A

Diffuse Large B-Cell Lymphoma

57
Q

CD15 and CD30+

A

Reed-Sternberg cells

Hodgkin lymphoma

58
Q

Large B cells with multilobed nuclei and prominent nucleoli

A

Reed-Sternberg cells
Hodgkin lymphoma

‘owl-eye nucleoli’

59
Q

Symptoms of Reed-Sternberg cell’s secretion of cytokines

A
  1. Occasionally results in ‘B’ sx (F/C, weight loss, night sweats)
  2. Attract reactive lymphocytes, plasma cells, macrophages, and eosinophils
  3. May lead to fibrosis
60
Q

Hodgkin Lymphoma’s subtypes

A
  1. Nodular sclerosis
  2. Lymphocyte-rich
  3. Mixed cellularity
  4. Lymphocyte-depleted
61
Q

MC subtype of HL

A

Nodular sclerosis

62
Q

Presents as enlarging cervical or mediastinal lymph node in a young adult, usually female

A

Nodular sclerosis (HL)

63
Q

Lymph node is divided by bands of sclerosis

Lacunar cells

A
Nodular sclerosis (HL)
Lacunar cells - Reed-sternberg cells present in lake-like spaces
64
Q

HL subtype with best prognosis

A

Lymphocyte-rich

65
Q

HL subtype associated with abundant eosinophils

A

Mixed cellularity (RS cells produce IL-5)

66
Q

HL subtype that is the most aggressive; seen in elderly and HIV+ individuals

A

Lymphocyte-depleted

Seen in elderly and HIV+ individuals

67
Q

MC primary malignancy of bone

A

Multiple myeloma

68
Q

MC malignant lesions of bone

A

Metastatic cancer

69
Q

High serum IL-6 may be present

A

Multiple myeloma

IL-6 stimulates plasma cell growth and Ig production

70
Q

Bone pain with hypercalcemia

Lytic lesions on x-ray

A

Multiple myeloma
Cells activate the RANK rec on osteoclasts.
Lytic lesions in vertebrae and skull
Increased risk of fracture

71
Q

Elevated serum protein

A

Multiple myeloma
Cells produce Ig
M spike on SPEP
MC due to monoclonal IgG or IgA

72
Q

Increased risk of infection - Monoclonal Ab lacks diversity

A

Multiple myeloma

73
Q

MC cause of death in Multiple myeloma

A

Infection

74
Q

Rouleaux formation of RBCs on Blood smear

A

Multiple myeloma

Increased serum protein decreases charge b/w RBCs

75
Q

Primary AL amyloidosis

A

Multiple myeloma

Free light chains circulate in serum and deposit in tissues

76
Q

Proteinuria - Bence Jones protein

A

Multiple myeloma
Bence Jones - Free light chains excreted in Urine
Deposition in kidney tubules leads to risk for RF (myeloma kidney)

77
Q

Increased serum protein with M-spike on SPEP, Common in elderly
w/o sx

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)
No lytic bone lesions, Hypercalcemia, AL amyloidosis, Bence jones

5% of 70 y.o.
1% pts develop Multiple myeloma yearly

78
Q

B-cell lymphoma with monoclonal IgM production

A

Waldenstrom Macroglobulinemia

79
Q

Generalized LAD; w/o lytic bone lesions
Increased serum protein with M-spike on SPEP
Visual and neurologic deficits (retinal hemorrhage or stroke)
Bleeding

A

Waldenstrom Macroglobulinemia
IgM - causes hyperviscosity
Viscous serum - defective platelet aggregation

80
Q

Treated with plasmapheresis in acute complication

A

Waldenstrom Macroglobulinemia

Removes IgM from the serum

81
Q

CD1a+ and S100+ by immunochemistry

A

Langerhans cell Histiocytosis

82
Q

Birbeck granules

A

Langerhans cell Histiocytosis

tennis racket appearing

83
Q

Malignant proliferation of Langerhans cells

A

Letterer-Siwe Disease

Hand-Schuller-Christian Disease

84
Q

Skin rash and cystic skeletal defects in infant (

A

Letterer-Siwe Disease

Multiple organs may be involved; rapidly fatal

Malignant Langerhans cells

85
Q

Scalp Rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child

A

Hand-Schuller-Christian Disease

Malignant Langerhans cells

86
Q

Pathologic fracture in an adolescent
Skin not involved

Biopsy?

A

Eosinophilic Granuloma

Benign Langerhans cells in bone

Biopsy: Langerhans cells w/ mixed inflammatory cells, including eosinophils