WBC Disorder Associations Flashcards

1
Q

What are the two driving forces for acute inflammation?

A

1) Bacterial infection

2) Tissue necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Which leukopenia/leukocytosis is associated with bacterial infections? What is one exception of bacterial species?

A

NEUTROPHIL LEUKOCYTOSIS - Increased neutrophils (left-shift)
Exception = BORDATELLA PERTUSSIS - Results in lymphocyte leukocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Which leukopenia/leukocytosis is associated with chronic myeloid leukemia (CML)?

A

BASOPHILIA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Which leukocytosis is Hodgkin lymphoma associated with?

A

EOSINOPHILIA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What marker is decreased in neutrophil leukocytosis?

A

CD16 (i.e. Decreased Fc receptor expression from LEFT SHIFT - Increase in release of immature neutrophils from the bone marrow as a response to bacterial infection or tissue necrosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

High cortisol state (e.g. exogenous cortisol or Cushing’s Syndrome) is associated with which 2 leukopenia/leukocytosis?

A

1) LYMPHOPENIA - Due to induction of lymphocyte apoptosis by cortisol
2) NEUTROPHIL LEUKOCYTOSIS - Due to cortisol-mediated impairment of neutrophil adhesion to the endothelial lining of vasculature (i.e. marginated pool) -> Release of marginated neutrophils into the serum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Which leukopenia/leukocytosis is associated with infectious mononucleosis (mono)? What is the most common viral agent of mono, what is the less common viral agent?

A
LYMPHOCYTE LEUKOCYTOSIS (reactive CD8+ CTL) 
Most common = EBV
Less common = CMV
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

EBV infection increases the risk of which neoplastic condition? Particularly in which pt population?

A

B CELL LYMPHOMA, particularly in IMMUNOCOMPROMISED pt population (e.g. HIV pts)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the main difference between ACUTE leukemia and CHRONIC leukemia?

A

ACUTE Leukemia - Neoplastic proliferation of IMMATURE BLASTS in the BONE MARROW
CHRONIC Leukemia - Neoplastic proliferation of MATURE, CIRCULATING lymphocytes in the BLOOD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the most common type of acute lymphoblastic leukemia (ALL)?

A

B-ALL: B-lymphoblast acute lymphoblastic leukemia

Accumulation of >20% B-lymphoblasts in the bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the surface markers of B-ALL(leukemia)?

A

CD34+ (HSC)/ tDt+ (Lymphoblast)/ CD10,CD19,CD20 (B-lymphoblast)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the surface markers of T-ALL(lymphoma)? Which surface marker do these T-lymphoblasts NOT express?

A

CD34 (HSC)/ tDt (Lymphoblast)/ CD2-CD8 (T-Lymphoblast)

Do NOT express CD10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which age population is acute lymphoblastic leukemia most prevalent in?

A

Children, associated with Down’s syndrome AFTER age 5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the prognosis of B-ALL based on? What are the two important prognoses (1 good, 1 bad) based on?

A

Cytogenetic abnormalities -
1) GOOD PROGNOSIS - t(12;21) in children
2) BAD PROGNOSIS - t(9;22) in adults - Philadelphia chromosome
(Ph+ ALL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the difference between lymphoma and leukemia? Which ALL is associated with which?

A

LYMPHOMA - Malignant cells form a MASS = T-ALL (T-lymphobast acute lymphoma)
LEUKEMIA - Malignant cells float around in BLOOD = B-ALL (B-lymphoblast acute leukemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which pt population is acute myeloid leukemia most prevalent in?

A

Older age pt population - avg age 55yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are the 4 main sub-classes of acute myeloblastic leukemia (AML)

A

1) ACUTE PROMYELOCYTIC LEUKEMIA (APL)
2) ACUTE MONOBLASTIC LEUKEMIA
3) ACUTE MEGAKARYOBLASTIC LEUKEMIA
4) AML that has arisen from a background of pre-existing dysplasia (MYELODYSPLASTIC SYNDROME)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Why is APL considered a medical emergency? What is the treatment of APL?

A

Risk of DIC

Translocation t(15;17) -> Disrupted retinoic acid receptor expression -> Inhibits maturation of promyelocytes -> HIGH [promyelocytes] -> HIGH [Auer rods inside promyelocytes] -> Activation of coagulation cascade -> DIC

Tx = ATRA (all-trans-retinoic acid)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Which leukemia is associated with infiltration and swelling of the gums?

A

ACUTE MONOCYTIC/MONOBLASTIC LEUKEMIA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Which pt population does chronic leukemia affect the most?

A

Older adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Which leukemia can transform to diffuse large B-cell lymphoma (called RICHTER TRANSFORMATION - marked as enlarging lymph node or spleen)?

A

CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) - Neoplastic proliferation of NAIVE B cells in the blood -> Migrates to lymph nodes -> SMALL LYMPHOCYTIC LYMPHOMA

Lymphoma cells pick up additional mutations -> RICHTER Transformation -> Rapid growth/hypertrophy of lymph node/spleen = DIFFUSE LARGE B CELL LYMPHOMA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Which chronic leukemia is an exception of the general rule that LAD and splenomegaly generally result after a neoplastic proliferation of mature circulating lymphocytes?

A

HAIRY CELL LEUKEMIA (HCL) - TRAP pneumonic

TRAP+, Trapped in splenic RED pulp and bone marrow so can not distribute into lymph nodes (Absent LAD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Which virus is ACUTE T-CELL LEUKEMIA LYMPHOMA strongly associated with? Where is this virus most commonly found?

A

HTLV1

Japan + Caribbean

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Which cancer is associated with LYTIC BONE LESIONS + HYPERCALCEMIA?

A

MULTIPLE MYELOMA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Which cancer is associated with LYTIC BONE LESIONS + HYPERCALCEMIA + RASH?

A

ADULT T-CELL LEUKEMIA LYMPHOMA (ATLL)

*Remember that T-cell leukemias like to migrate to the epidermis and skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Which chronic leukemia is associated with Pautrier microabscesses (aggregates of neoplastic CD4+ T cells in the skin)? What syndrome is it now called if these neoplastic cells migrate into the blood?

A
MYCOSIS FUNGOIDES 
SEZARY SYNDROME (Contains **cerebriform nuclei**)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What are the surface markers of chronic lymphocytic leukemia (CLL) naive B cells?

A

CD5 and CD20

*Aberrant expression of CD5 because it’s NORMALLY expressed on T cell surfaces NOT B cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the most common leukemia overall?

A

Chronic lymphocytic leukemia (CLL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the most common cause of death in chronic lymphocytic leukemia?

A

INFECTION due to the complication of HYPOGAMMAGLOBULINEMIA (Low Ig production) due to neoplastic naive B cells “crowding out” normal Ab production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What are the 3 general complications of myeloproliferative disorders with the exception of ESSENTIAL THROMBOCYTHEMIA?

A
  1. Increased risk of HYPERURICEMIA/GOUT - Due to high turnover of nuclei -> Hyper-activation of purine degradation pathway -> INCREASED URIC ACID
  2. Progression to MARROW FIBROSIS
  3. Transformation to ACUTE LEUKEMIA (AML 2/3 or ALL 1/3)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What needs to be ruled out when looking at a blood smear of CML? How do you rule that disease process out?

A

REACTIVE LEUKEMOID REACTION (High WBC count reactive/secondary to an acute infection/inflammatory process)

  1. CML - LAP NEGATIVE (leukocyte alkaline phosphatase)
  2. CML - t(9;22)
  3. CML - BASOPHILIA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Which myeloproliferative disorders are driven by a Jak2 kinase mutation? Which one is driven by a Tyr kinase mutation in the HSC?

A

JAK2 MUT- Polycythemia vera, Essential thrombocythemia, Myelofibrosis
TYR K. MUT - Chronic myeloid leukemia (CML)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is the classic example of venous thrombosis related to hepatic infarction? What is the MOST COMMON CAUSE of this syndrome?

A

BUDD-CHIARI SYNDROME: Hepatic vein thrombosis -> Hepatic infarction
Most common cause = POLYCYTHEMIA VERA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What condition needs to be differentiated from POLYCYTHEMIA VERA? How do you rule it out?

A

REACTIVE POLYCYTHEMIA due to
1. Lung disease -> Hypoxia (Low SaO2, Increased EPO)
2. Renal cell carcinoma -> Ectopic EPO production (Normal SaO2, Increased EPO
PV: DECREASED EPO (due to negative feedback), NORMAL SaO2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Which tumor is most commonly associated with ECTOPIC ERYTHROPOIETIN (EPO) production?

A

RENAL CELL CARCINOMA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Which two conditions need to be on my differential if I see INCREASED PLATELETS in a blood smear?

A

ESSENTIAL THROMBOCYTHEMIA

Fe-DEFICIENY ANEMIA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Which myeloproliferative disorder is associated with tear-drop RBC production?

A

MYELOFIBROSIS - Tear-shaped due to RBC-production within a fibrotic bone marrow and “squeezed” exit out to the blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

t(9;22) cytogenetic abnormality is a defining feature of which neoplastic leukemia? Which leukemia constitutes a small subset of this translocation pt pool?

A

CHRONIC MYELOID LEUKEMIA

Small subset - Acute lymphoblastic leukemia (mostly B-ALL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Ddx of INCREASED granulocytes and their pre-cursors on blood smear

A
  1. LEUKEMOID REACTION (Increased WBC secondary/reactive to a primary inflammatory/infectious process)
  2. CML (Chronic myeloid leukemia) - Especially BASOPHILIA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Ddx of INCREASED platelets on blood smear

A
  1. ESSENTIAL THROMBOCYTHEMIA

2. Secondary Reactive Thrombocytosis: FE-DEFICIENCY ANEMIA + SPLENECTOMY/AUTOSPLENECTOMY

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Ddx of PAINLESS LAD (3)

A
  1. CHRONIC INFLAMMATION (e.g. autoimmune diseases)
  2. METASTATIC CARCINOMA (e.g. axillary lymph node enlargement from breast cancer metastasis)
  3. LYMPHOMA (eg. Follicular Lymphoma)
42
Q

Which region of the lymph node is expanded (resulting in reactive LAD) for rheumatoid arthritis and early HIV?

A

CORTEX - specifically follicular dendritic cells of the cortex

43
Q

Which region of the lymph node is expanded (resulting in reactive LAD) with a viral infection (e.g. EBV causing infectious mononucleosis)?

A

PARACORTEX

44
Q

Which region of the lymph node is expanded (resulting in reactive LAD) with lymph node drainage of an area of cancer?

A

MEDULLA - Specifically the sinus histiocytes of the medulla (HISTIOCYTOSIS)

45
Q

Are NON-HODGKIN LYMPHOMA neoplastic cells mostly B cells or T cells?

A

B CELLS

46
Q

Which lymphomas (4) constitute a neoplastic proliferation of SMALL B Cells?

A
  1. FOLLICULAR LYMPHOMA
  2. MANTLE CELL LYMPHOMA
  3. MARGINAL ZONE LYMPHOMA
  4. SMALL LYMPHOCYTIC LYMPHOMA - CLL cells that spread to the lymph node
47
Q

Which lymphoma is a neoplastic proliferation of INTERMEDIATE B cells?

A

BURKITT LYMPHOMA

48
Q

Which lymphoma is a neoplastic proliferation of LARGE B cells?

A

DIFFUSE LARGE B CELL LYMPHOMA

49
Q

Diffuse large B-cell lymphoma can be a complication of which original lymphomas?

A

SMALL LYMPHOCYTIC LYMPHOMA - CLL Naive B cells
FOLLICULAR LYMPHOMA - Small B cells

Generalized LAD -> These cells acquire additional mutations -> 1 of the lymph nodes FURTHER ENLARGES -> DIFFUSE LARGE B CELL LYMPHOMA

50
Q

Which cytogenetic abnormality constitutes FOLLICULAR LYMPHOMA?

A

t(14;18) - BCL2 on Chrom 18 translocates to IgH on Chrom 14
OVEREXPRESSION of BCL2 -> EXCESSIVE ANTI-APOPTOSIS post-somatic hypermutation (B cells that SHOULD be apoptosed after SH do NOT die) -> LYMPHOMA

51
Q

Which lymphomas present in LATE ADULTHOOD + manifest with PAINLESS LAD?

A
  1. FOLLICULAR LYMPHOMA (SMALL B CELL)

2. MANTLE CELL LYMPHOMA (SMALL B CELL)

52
Q

MANTLE CELL LYMPHOMA is driven by which chromosomal translocation? And what is the end result in terms of expression?

A

t(11;14) - Cyclin D1 overexpression by its translocation from Chrom 11 to IgH locus on Chrom 14

53
Q

MARGINAL ZONE LYMPHOMA is associated with which general conditions?

A

CHRONIC INFLAMMATORY CONDITIONS (Eg. autoimmune)

  1. HASHIMOTO THYROIDITIS
  2. SJOGREN SYNDROME
  3. H.PYLORI GASTRITIS
54
Q

BURKITT LYMPHOMA is driven by which chromosomal translocation? What does it result in with regard to expression?

A

t(8;14) - C-myc overexpression due to its translocation from Chrom 8 to the IgH locus on Chrom 14

55
Q

Starry sky appearance histological appearance is associated with which lymphoma?

A

BURKITT LYMPHOMA - Due to very high mitotic index (lots of blue tumor nuclei in “sky” and tingible body macrophages present due to dying cells from rapid overgrowth “starry”)

56
Q

What is the MOST COMMON form of Non-Hodgkins Lymphoma?

A

DIFFUSE LARGE B CELL LYMPHOMA

57
Q

What is the main difference between Hodgkins lymphoma and Non-Hodgkins lymphoma?

A

NHL - ENTIRE mass is made up of malignant cells
HL - Mass = Rare neoplastic Reed Sternberg cells (Large B cells) + Reactive inflammatory cells (lymphocytes, eosinophils, plasma cells, macrophages - **Bulk of the mass)

58
Q

What are the cell surface markers of neoplastic Reed Sternberg cells of HODGKINS LYMPHOMA?

A

CD15+ and CD30+

59
Q

Which form of HL has the BEST prognosis and which form of HL has the WORST prognosis?

A

BEST - Lymphocyte-rich HL

WORST - Lymphocyte-depleted HL

60
Q

What is the most common malignancy of bone? What is the most common PRIMARY malignancy of bone?

A

MOST COMMON - Metastatic carcionma

MOST COMMON PRIMARY MALIGNANCY - Multiple myeloma

61
Q

What is the most common mAb overproduced resulting in the SPEP M-Spike by neoplastic plasma cells in MULTIPLE MYELOMA?

A

IgG or IGA

62
Q

What is the most common cause of death in multiple myeloma pts? Why?

A

INFECTION
Due to LOSS of antigenic diversity - neoplastic plasma cell resulting in a MONOCLONAL proliferation of ONLY either IgG or IgA

63
Q

Which serum cytokine is elevated in the serum that drives the overproduction of plasma cells in MULTIPLE MYELOMA?

A

IL-6

64
Q

What are two diagnostic findings of MULTIPLE MYELOMA?

A
  1. SPEP - M SPIKE
  2. BLOOD SMEAR - Rouleaux formation of RBC
    Increased serum protein -> Decrease in charge between RBC -> RBC no longer equally distributed -> RBC pile on top of each other like poker chips
65
Q

What are the 4 complications of MULTIPLE MYELOMA?

A
  1. Increased risk of infection - Due to monoclonal proliferation and loss of Ag diversity **Most common cause of death
  2. PRIMARY AMYLOIDOSIS - Due to overproduction of light chain and deposits in the blood as amyloid protein
  3. PROTEINURIA (specifically bence jone proteins) - Excretion of light chain in the urine
  4. MYELOMA KIDNEY (renal failure) - Deposits of light chain in kidney tubules
66
Q

Differential diagnosis of M-SPIKE shown on SPEP:

A
  1. MULTIPLE MYELOMA (IgG or IgA)
  2. MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE (MGUS - IgG or IgA)
  3. WALDENSTROM MACROGLOBULINEMIA (IgM
67
Q

BIRBECK (Tennis racket) Granules associated with ___?

A

LANGERHANS CELL HISTIOCYTOSIS

68
Q

What are the immunohistochemistry markers seen in LANGERHANS CELL HISTIOCYTOSIS?

A

LANGERHANS CELLS - CD1a+ and S100+

69
Q

Which lymphoma is associated with EBV?

A

BURKITT LYMPHOMA

70
Q

Ddx of an adolescent having a pathologic fracture

A
  1. OSTEOSARCOMA (tumor of the bone)

2. EOSINOPHILIC GRANULOMA (Langerhans cell histiocytosis)

71
Q

Down’s syndrome increases the risk of leukemia. Which leukemia BEFORE age 5 and which one AFTER age 5?

A

AFTER age 5: B-ALL - B-lymphoblastic acute leukemia

BEFORE age 5: ACUTE MEGAKARYOBLASTIC LEUKEMIA

72
Q

Which chromosomal translocation is DIFFUSE LARGE B CELL LYMPHOMA associated with?

A

3;14 - BCL-6 (3) translocation onto IgH (14)

73
Q

Which leukemia is B-RAF mutation associated with?

A

HAIRY CELL LEUKEMIA

74
Q

RUDDY COMPLEXION of the face (flushed face) + ERYTHROMELALGIA (burning in palms/soles of feet) + ITCHING after hot bath are pathognomonic for ___

A

POLYCYTHEMIA VERA

75
Q

ELEVATED HCT + MICROCYTIC ANEMIA are lab values pathognomonic of ___?

A

POLYCYTHEMIA VERA

76
Q

What are 4 physical findings of secondary neoplastic proliferation more prevalent in ACUTE MYELOID LEUKEMIA versus ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)?

A
  1. HEPATOSPLENOMEGALY, SPLENOMEGALY - Actually see this in ALL as well
  2. GUMS - ACUTE MONOCYTIC/MONOBLASTIC LEUKEMIA
  3. INFILTRATION TO SKIN - Although more common in T cell leukemias
  4. ALTERED MENTAL STATUS - Due to EXTREMELY HIGH WBC
77
Q

What clinical physical Sx (2) is most specific to ACUTE LYMPHOBLASTIC LEUKEMIA?

A
  1. BONE PAIN due to Bone marrow expansion (>20% blasts in the bone marrow)
  2. TESTICULAR ENLARGEMENT
78
Q

What clinical Sx is most specific to ACUTE MYELOID LEUKEMIA?

A

Gum swelling + Altered mental status changes (high WBC and vascular sludging in the CNS)

79
Q

Which staining detects MONOCYTES?

A

Non-specific ESTERASE

80
Q

What is the most common INDOLENT form of NON HODGKINS LYMPHOMA? What is the most common form of NON HODGKINS LYMPHOMA?

A

Most common indolent = FOLLICULAR LYMPHOMA

Most common = DIFFUSE LARGE B CELL LYMPHOMA

81
Q

What is the most CONSISTENT structural/numerical/chromosomal change seen ACUTE LYMPHOBLASTIC LEUKEMIA?

A

HYPERPLOIDY

82
Q

What are good prognosis factors for ACUTE LYMPHOBLASTIC LEUKEMIA? What are bad prognosis factors?

A

GOOD: t(12;21) - CHILDREN (age: 2-10), LOW WBC, HYPERDIPLOIDY

BAD: t(9;22) - ADULTS or INFANTS (

83
Q

Which staining detects MYELOBLASTS?

A

MPO

84
Q

Which leukemia is associated with STAT3 signaling mutations?

A

CHRONIC MYELOID LEUKEMIA (CML) STAT3 dysregulation associated with BCR-ABL fusion protein

85
Q

What is the most appropriate course of action for a pt with ACUTE MYELOID LEUKEMIA presenting thrombocytopenia and bleeding everywhere?

A

Bring pt to ER!

Especially if pt has APL, pt is at risk of DIC

86
Q

What are the two biggest risk factors (BAD PROGNOSIS factors) for ACUTE MYELOID LEUKEMIA?

A
  1. Prior CHEMO therapy (alkylating agents) or radiotherapy:
    Pre-existing dysplasia - Cells don’t form properly, can’t exit the bone marrow or enter the blood -> Accumulate blasts
  2. Previous lymphoma (MYELODYSPLASTIC SYNDROME) with subsequent irradiation therapy
87
Q

What are good prognosis factors of ACUTE MYELOID LEUKEMIA?

A
  1. Age
88
Q

What is a general important distinction in terms of overall CBC count with chronic conditions - CHRONIC LEUKEMIA vs MYELOPROLIFERATIVE DISORDERS?

A
  1. CHRONIC LEUKEMIA (e.g. HAIRY CELL LEUKEMIA) - will have ELEVATED WBC, but pancytopenia elsewise (like Acute leukemia)
  2. MYELOPROLIFERATIVE DISORDERS (e.g. CHRONIC MYELOID LEUKEMIA) - Will have PREDOMINANTLY ELEVATED WBC, and an elevated count across all cells in the myeloid lineage
89
Q

What cell markers are expressed in NORMAL Follicular cells? Which marker is additionally expressed in neoplastic follicular cells in FOLLICULAR LYMPHOMA?

A

NORMAL: CD19, CD20, IgM, BCL6

FOLLICULAR LYMPHOMA: BCL2 in addition to CD19, CD20, IgM, BCL6

90
Q

What is the normal bone marrow cellularity of a growing child? What is the normal bone marrow cellularity of an adult?

A

CHILD: 60-70%
ADULT: 40-50%

91
Q

Nearly all cells show proliferation Ag Ki-67 + starry sky appearance.
What lymphoma am I?

A

BURKITT LYMPHOMA

92
Q

Is BURKITT LYMPHOMA responsive or unresponsive to chemotherapy?

A

RESPONSIVE

93
Q

Which virus is most commonly associated with ENDEMIC BURKITT LYMPHOMA? Which virus is commonly associated with SPONTANEOUS BURKITT and DIFFUSE LARGE B CELL LYMPHOMA?

A

Endemic Burkitt - EBV

Spontaneous Burkitt and DLBCL - HIV but need to be very deficient in T cells, generally more EBV as well now

94
Q

Which type of HODGKIN LYMPHOMAS contains EBV viral DNA?

A
  1. LYMPHOCYTE-DEPLETED: Most aggressive, >90% RS cells + EBV viral DNA
  2. MIXED CELLULARITY: 70% of RS cells + EBV viral DNA
95
Q

Which type of HODGKINS LYMPHOMA has the best prognosis?

A

LYMPHOCYTE RICH HL - Contains atypical REED STERNBERG CELLS - Do express B cell markers (CD20 and BCL-6) + No association with EBV at all

96
Q

CHEMOTHERAPY is most associated with which leukopenia OR leukocytosis?

A

NEUTROPENIA - chemotherapy with alklylating agents -> Damage to stem cells -> Decreased WBC production

97
Q

SEVERE Gm- SEPSIS is most likely associated with which leukopenia or leukocytosis?

A

Mild/moderate Gm-sepsis: Associated with INCREASED NEUTROPHILS
But SEVERE Gm- sepsis: Associated with NEUTROPENIA as circulating neutrophils are out of blood (decreased) and in tissue

98
Q

What is the Tx of NEUTROPENIA associated with chemotherapy and severe Gm- bacteria sepsis?

A

GM-CSF, G-CSF

GM-CSF (Granulocyte/Monocyte colony stimulating factor)
G-CSF (Granulocyte colony stimulating factor)

99
Q

What immune cell is most sensitive to whole body radiation? Thus, what is the earliest change?

A

LYMPHOCYTES

Earliest change = LYMPHOPENIA

100
Q

What other pathologies are associated with LYMPHOPENIA? [Hint: Think 1 congenital, 1 acquired, 1 endocrine, 1 autoimmune, 1 iatrogenic]

A

IMMUNODEFICIENCY (DiGeorge, HIV)
HIGH CORTISOL STATE (e.g. Cushing’s or exogenous corticosteroids)
SLE = Type II HSR against WBC (as well as RBC, PLT)
Whole body radiation

101
Q

What is the Tx of HAIRY CELL LEUKEMIA? What is its mechanism?

A

2-CDA (CLADRIBINE) = ADENOSINE DEAMINASE INHIBITOR
Adenosine deaminase = Purine degradation enzyme

Hairy cell leukemia -> Neoplastic proliferation of MATURE B CELLS -> Cladribine ACCUMULATES ADENOSINE -> Toxic to cells -> Kills of mature B cells