WBC Disorders (Sloka) Flashcards

1
Q

Name the WHO classification system of lymphoid neoplasms.

A
  1. Precursor B cells Neoplasms
  2. Peripheral B cell neoplasms
  3. Precursor T cell neoplasms
  4. Peripheral T cell and NK neoplasms
  5. Hodgkin’s Lymphoma
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2
Q

Know the markers (CD10 for example) by using Dillon’s quizlet!

A

pryo6619

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3
Q

MC cancer and leukemia in children less than 15 y/o. Peak age is 3 y/o

A

ALL (B-ALL)

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4
Q

Neoplastic accumulation of lymphoblasts (> 20%) in the bone marrow

Lymphoblasts are characterized by positive nuclear staining for TdT, a DNA polymerase.

TdT is absent in myeloid blasts and mature lymphocytes.

A

ALL

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5
Q

Characteristics of B-ALL

A

CALLA: CD10
TdT+
T(12,21) or philadelphia chromosome (9,22)
Hyperdiploidy (SS)

“Hyper kids like playing w/B-ALLS”

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6
Q

Prognosis of B-ALL

A

95% complete remission

75-85% cures

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7
Q

Lab findings of B-ALL?

A

WBC elevated but large range. Can be normal initially.
NN anemia!!!!
Thrombocytopenia!!!! (all acute leukemia’s have both)
Lymphoblasts- greater than 30% in BM (acute)

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8
Q

CD10 neg but TdT pos
Anterior mediastinal mass/acute leukemia found in an adolescent male.

Likely dx?

A

T-ALL!

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9
Q

MC leukemia of all?

A

CLL

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10
Q

Disorder:

Neoplastic proliferation of Bcells (CD20+); associated with EBV

A

Burkitt’s Lyphoma

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11
Q

MC leukemia of patients older than 60?

A

CLL

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12
Q

Burkitt Lymphoma is driven by translocations of _____. t(&)

A

C-myc. t(8,14)

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13
Q

Starry sky is caused by and is associated with Burkitt’s.

A

clear spaces with macrophages containing debris (“tingible” body macrophages)

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14
Q

MCC NHL?

A

Diffuse large B cell lymphomas

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15
Q

MCC of generalized lymphadenopathy?

SS

A

CLL

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16
Q

In CLL, lymphoblast count in BM?
How about:
anemia
thrombocytopenia

what characteristic cell will you see in CLL?
MCC of death in CLL?

A
WBC count varies 
“Smudge” cells
NN anemia 50%
Thrombocytopenia 40%
Hypogammaglobulinemia- MCC of death due to infections. 
BM -
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17
Q

Disorder?

BCL2 on chromosome 18 translocates to the Ig heavy chain locus on chromosome 14.

Results in over-expression of Bcl2, which inhibits apoptosis

A

Follicular Lymphoma

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18
Q

Neoplastic proliferation of mature B-cells.

Middle-aged men
B cell
TRAP, BRAF
Splenomegaly 90%, lymphadenopathy is rare
Pancytopenia (especially monocytopenia)
A

Hairy Cell Leukemia

Pancytopenia is in the peripheral blood.

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19
Q

Increased lymphocytes and smudge cells are seen on blood smear

A

CLL

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20
Q

MCC of death in CLL?

A

Infections due to Hypogammaglobulinemia

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21
Q

Disorder?

Neoplastic proliferation of small B cells (CD20+) that form follicle-like nodules?

A

Follicular Lymphoma

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22
Q

Neoplastic proliferation of small B-cells.

Associated with chronic inflammatory states such as Hashimoto thyroiditis
Sjogren syndrome
H pylori gastritis

if in mucosal sites, called MALToma

A

Marginal zone lymphoma

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23
Q
Patient comes in with: 
WBC elevated
NN anemia
Lymphoblasts greater than 30% in BM
Thrombocytopenia

Likely dx?

A

Leukemia

Acute.

Lymphoblastic

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24
Q

Patient does not have lympadenopathy, has splenomegaly, and is pancytopenic in peripheral blood.

Trap +

Most likely dx?

A

Hairy cell leukemia

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25
Q

Neoplastic proliferation of naive B cells that co-express CDS and CD20

A

CLL

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26
Q

Neoplastic proliferation of small B cells (CD20+).

Clinically presents in late adulthood with painless lymphadenopathy.

Driven by t(11;14)
Cyclin Dl gene on chromosome ll translocates to Ig heavy chain locus on chromosome 14.

Over expression of cyclin D1 promotes Gl/S transition in the cell cycle, facilitating neoplastic proliferation.

A

Mantle Cell Lymphoma

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27
Q

SS.
Disorder?

Often develop in patients with abnormal immune states (e.g. SLE, Sjogren’s’ syndrome, renal transplantation)

May occur at any age
Usually elderly adults

A

Diffuse Large B cell lymphoma

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28
Q

SS.

The MC form indolent lymphoma?

A

Follicular lymphoma

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29
Q

SS:

Most common monoclonal Plasma Cell Dyscrasia?

A

Monoclonal gammopathy of undetermined significance (MGUS)

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30
Q

What is multiple myeloma?

A

Malignant proliferation of plasma cells in the bone marrow. One plasma cell clone is proliferated while the others are suppressed leading to monoclonality.

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31
Q

Plasma Cell Dyscrasia:

Most commonly due to an increase in IgG
Other plasma cell clones are suppressed

A

Multiple Myeloma

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32
Q

Describe Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)

A

B-cell lymphoma with monoclonal IgM production.

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33
Q

Plasma Cell Dyscrasia:

Small IgG M spike in elderly patients
Plasma cells

A

Monoclonal gammopathy of undetermined significance (MGUS)

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34
Q

Characteristics of multiple myeloma?

A

High IgM

Bone pain with hypercalcemia
lytic bone lesions

Elevated serum protein –> Rouleaux formation

Bence Jones protein (free light chain excreted in urine; myeloma kidney: Renal failure 30-50%)

Amyloidosis- free light chains circulate in serum and deposit in tissues

Monoclonal antibody

NO generalized lymphadenopathy! (compare to Waldenstrom’s)

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35
Q

MCC of death in multiple myeloma?

A

infection due to monoclonal antibody

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36
Q

M spike with IgM
BJ protein is present
Generalized lymphadenopathy

Anemia and bone marrow but no lytic lesions

liver, and spleen involvement

Hyperviscosity syndrome due to increased IgM:
retinalhemorrhages, strokes, platelet aggregation defects

A

Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)

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37
Q

Which disorder?

Epidemiology
MC in blacks
Rare under age 40
Peak incidence at age 50-60
Increased risk with radiation exposure
M-spike in 80-90%
A

Multiple Myeloma

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38
Q

SS:
Plasma Cell Dyscrasia:

Bone sites: vertebra, ribs, pelvis
Slight increase in monoclonal protein
No plasmablasts in bone marrow
No BJ protein
75% develop multiple myeloma
A

Solitary Skeletal Plasmacytoma

39
Q

SS:
Plasma Cell Dyscrasia:

Sites: upper respiratory tract (nasopharynx, sinuses, larynx)
Slight increase in monoclonal protein
Absence of malignant plasma cells in the bone marrow
Absence of BJ protein
Small percentage may develop multiple myeloma

A

Extramedullary plasmacytoma

40
Q

Plasma Cell Dyscrasia:

Sheets of malignant plasma cells in biopsy
Greater than 30% plasma cells in aspirate

A

Multiple Myeloma

41
Q

Similarities and differences between MM and Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)?

A

Similarities: IgM, BJ protein present.

Differences: W has no lytic bone lesions &
W has generalized lymphadenopathy unlike MM.

42
Q

Plasma Cell Dyscrasia:

M protein heavy chain without light chains
Absence of BJ protein

A

Heavy-chain Disease

43
Q

In MM, Myeloma cells release ___

A

interleukin 1 (osteoclast activating factor)

44
Q

SS:

The two class IV lymphoid neoplasms that we covered?

A

Adult T cell leukemia/lymphoma
&
Mycosis Fungoides

45
Q

SS: What gene is involved in Adult T cell leukemia/lymphoma?

A

TAX promotes secretion of pro-inflamatory NF-kB. Messes with TP53 (makes it ineffective)

46
Q

Neoplastic proliferation of mature CD4 T cells associated with HTLV-1.

MC seen in Japan and Caribbeans.

A

Adult T cell leukemia/lymphoma

47
Q

SS. Disorder?

Hematologic findings:

Normocytic anemia with rouleaux formation 
Thrombocytopenia
Increased sed rate
Prolonged bleeding time 
Due to a defect in platelet aggregation
A

rouleaux formation and increased sed rate bc of increased serum protein (IgM elevated)

MM is associated with platelet problems.

48
Q

Tests to dx MM?

A

Diagnosis:
BM
SPE and UPE
Xray

49
Q

Plasma cell disorder where:

Recurrent infections is a common cause of death
Sepsis due to Hemophilus influenzae, Streptococcus pneumoniae

A

MM

50
Q

What lab results will you find with someone who has Adult T-Cell Leukemia/lymphoma?

A

TdT negative!! Because it’s a marker for pre-B/T cells!! (these are more mature T-cells: lymphoblasts!)

CD4+ lymphoblasts (Tdt negative)

Leukemia: So NN anemia & thrombocytopenia

WBC: normal to elevated

51
Q

What is Mycosis Fungoides?

skin involvement progresses through three stages –> rash to ____ to ___ masses
spreads to other sites (MC _____)

A

Neoplastis proliferation of mature CD4+ cells that inflitrate the skin.

rash to plaque to nodular

spread MC to lymph nodes

52
Q

Patient from Japan presents with:

Hepatosplenomegaly
Generalized lymphadenopathy
Skin infiltration (rash)
Lytic bone lesions with hypercalcemia

A

Adult T-Cell Leukemia/lymphoma

53
Q

Neoplastic proliferation of _______ cells which are classically positive for CD15 and CD30.

A

Reed-sternberg (Hodgkin Lymphoma): Class V

54
Q

Disorder:

Involve neoplastic peripheral CD4 Th cells
Adults 40-60 years (SS)
MF involves the skin and other extra-cutaneous sites

Skin involvement presents with a rash secondary to a T cell infiltration of the epidermis and formation of Pautrier’s abscesses

A

Mycosis Fungoides

55
Q

Four classical subtypes of HL? From MC to LC.

A

Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte poor

56
Q

____ association in over 70% of cases of mixed cellularity

A

EBV

57
Q

HL is more common in M/F?
Whites or blacks?
Age distribution of HL?

A

Males
Whites
Bimodal: most common in young adults 15 to 34 years of age and in patients older than 50

58
Q

Pathogenesis of HL?

A

Unknown factors (? EBV, retrovirus) cause B and/or T cells to become neoplastic Reed-Sternberg cells

59
Q

Clinical findings:

Describe lymph nodes in HL?

A

Involves localized groups of nodes and has contiguous spread
Often involves cervical or supra-clavicular nodes
Cut section has a bulging “fish-flesh” appearance

60
Q

In HL, unlike NHL, the lymphocytes in the lymph nodes are __ _______

A

not neoplastic

61
Q

T/F Reed-Sternberg cells have CD45 markers.

A

False . CD15 and CD30

62
Q

Lacunar cells have to do with which subtype of HL?

A

NS

63
Q

HL vs NHL:

A

Age: HL is more younger

Fever: HL more associated with fevers. Pel Ebstein’s fever.

Nodes: HL is more unilateral, while NHL is more likely to be bilateral.

Rarely involves Waldeyer’s ring (oropharyngeal lymphoid tissue), skin or gastrointestinal tract

64
Q

most common type of HL?

A

NS

65
Q

Which subtype of HL?

female predominant disease
most commonly involves the anterior mediastinum and neck nodes.

70% 5 year survival

A

NS

66
Q
A

Lymphocyte depleted

67
Q

presents in males under 40 years of age
enlarged, painless cervical or supraclavicular nodes
Classic RS cells are extremely difficult to find

90% survival 5 years

A

Lymphocyte predominant (not classical 4)

68
Q

common in men greater than age 50
RS cells are very easy to find interspersed among plasma cells and eosinophils
Strong EBV relationship
50% 5 year survival

A

MC

69
Q

T/F: Clinical stage more important than type of HL

A

True!

70
Q

Describe AML

A

Neoplastic accumulation of immature myeloid cells (greater than 20%) in BM.

71
Q

High yield subtypes of AML:

M2-

A

M2- Most common 30-40%.

AML w/maturation

72
Q

AML:

M3-

A

Acute promyelocytic leukemia:

t(15;17) Primary granules = increased risk for DIC
myeloblasts characterized by + cytoplasmic staining for MPO. Crystal aggregates of MPO = AUER RODS!

73
Q

AML:

M5-

A

Acute monocytic leukemia:

Proliferation of monoblasts; usually lacks MPO
Characteristically infiltrates gums

74
Q

AML: M7-

A

Acute megakaryoblastic leukemia

Proliferation of megakaryoblasts; lacks MPO
associated with down syndrome (before age of 5)

75
Q

Myelodystplastic Syndromes

Age bracket?

(RR pg 329/Robins: 462):

A

50-80 y/o

76
Q

Pathogenesis of myelodysplastic syndrome?

A

5q;7q deletions
or
trisomy 8

Myeloblasts (neutrophil pre-cursors)

77
Q

How do we know if myelodysplastic syndrome has progressed to AML?

A

If myeloblasts are >20% in BM.

78
Q

What disorder is this:

Group of acquired clonal disorders that affects stem cells.
RBC’s are (mostly) macrocytic or microcytic

Characterstically have cytopenias in peripheral blood and normocellular/hypercellular bone marrows.

Frequently progresses to AML

A

Myelodysplastic syndromes

79
Q

What are myeloproliferative disorders?

What types are there?

A

Neoplastic proliferation of MATURE myeloid cells.
(Retinas capacity for terminal differentiation)
Average age: 50-60

Can differentiate (mature) so cells of ALL myeloid lineages are increased; classified based on the dominant myeloid cell increased.

CML- increased granulocytes (basophils)
PV- increased RBCs
ET- increased megakaryocytes/platelets
Myelofibrosis

80
Q

CML: Neoplastic proliferation of ____ myeloid cells, characteristically ____ are increased.

A

mature

basophils

81
Q

SS.

Two outside factors that can cause CML?

A

Ionizing radiation or benzene.

82
Q

Genetics of CML?

A

t(9:22) BCR-ABL proto-oncogene which activates tyrosine kinase activity!
Philadelphia chromosome (Ph)
PH 95%, +BCR-ABL 100%

83
Q

CML is distinguished from a leukemoid reactions by?

A

In the neoplasm:
LAP is negative
Increased basophils
t(9;22)

84
Q

CML can be converted to ALL or AML if:

A

AML- if >20% myeloblasts

ALL- if >20% lymphoblasts

85
Q

Tx of CML?

A

Imatinib: blocks tyrosine kinase

86
Q

Pathogenesis of PV?

A

Jak2 mutation.

Increases in all myeloid cell lines but especially RBCs!

87
Q

Clinical findings in PV?

A

Splenomegaly- Increased RBC’s increases sequestration

Thrombotic events- Increased viscosity

Impaired CNS circulation- same

Increased histamine release- increased # of mast cells

Gout- bc increased turnover of cells (nucleus is degraded and end product of purine degradation - uric acid crystals)

88
Q

PV increased/decreased:

Hct
RBC mass/Plasma volume

Leukocytosis/penia
Thrombocytosis/penia

EPO?
ABG values?

BM hypercellular or hypo?

A

Hct, RBC mass, Plasma Volume INCREASED

Leukocytosis
Thrombocytosis

Decreased EPO (have enough RBC's - feedback) 
Normal ABG

BM hypercellular –> fibrosis

89
Q

ET pathogenesis?

A

Like PV:

Jak2 mutation.

90
Q

ET symptoms?

A

increased risk of bleeding (platelets not working) or thrombosis (over active)

RBCs and granulocytes are increased

91
Q

Pathogenesis of myelofibrosis?

A

Like PV, and ET:

Jak2 mutation

92
Q

In myelofibrosis: what is increased?

A

Mature myeloid cells; especially megakaryocytes

93
Q

Describe pathophys of meylofibrosis

A

Megakaryocytes produce excess PDGF causing marrow fibrosis.

Causing:
Extramedullary hematopoiesis.
- this causes leukoerythroblastic smear: increased leukocytes, increased erythrocytes, increased immature cells bc no reticulin gates in spleen (unlike BM)

Splenomegaly
-above reason

Tear drop RBC’s
- bc of marrow fibrosis

Thrombosis
-increased platelets