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Block 8 - Autoimmunity, Neoplasia, and Hematologic Disease > ASF - White Blood Cells and Leukemias (Duong) > Flashcards

ASF - White Blood Cells and Leukemias (Duong) Flashcards

1
Q

A 60-year-old man has lymphadenopathy. Biopsy reveals effacement of the node by small CD5+ CD23- B-lymphocytes. The protein overexpressed is:

A. Bcl-2

B. Cyclin D1

C. Ig light chain

D. Myc

A

B. Cyclin D1

CD5+ and CD23- B-cells indicates mantle cell lymphoma.

Mantle cell lymphoma is caused by a t(11:14), which fuses cyclin D1 with the Ig heavy chain gene. This leads to proliferation of B-cells.

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2
Q

Reed-Sternberg cells are associated with which type of cancer and are oftentimes surrounded by what type of cells?

A

Hodgkin Lymphoma

Typically Reed-Sternberg cells are surrounded by granulocytes. This is because the RS cells secrete chemotactic factors that recruit granulocytes, leading to an enlarged lymph node.

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3
Q

A translocation between chromosome 11 and 14 involving the BCL-1 gene will induce which cancer?

A

Mantle Cell Lymphoma

t(11:14) fuses cyclin D1 with Ig heavy chain gene, producing the B-cell lymphoma 1 (BCL-1) gene.

CD5+, but CD23-

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4
Q

Translocation of genes PML/RARA between chromosomes 15 and 17 is indicative of what cancer?

A

Promyelocytic leukemia (AML type M3)

Translocation of promyelocytic gene with retinoic acid receptor gene prevents maturation of promyelocytes.

These cells will have lots of Auer rods, which causes clotting –> DIC

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5
Q

Polycythemia vera is associated with low levels of what hormone?

A

Erythropoietin (EPO)

Polycythemia vera is a proliferation of mature myeloid cells, in particular RBCs.

Erythropoietin is a hormone released by the kidneys that tells the bone marrow to make more RBCs.

Because the RBC count is so high already, there will be a negative feedback loop that inhibits EPO release.

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6
Q

An adolescent with an anterior medialstinal mass is likely to have what type of cancer?

A

T-cell acute lymphoblastic leukemia (T-ALL)

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7
Q

Letterer-Siwe disease is another term for what cancer? This condition occurs in what age patients?

A

Langerhans Cell Histiocytosis

Often seen in children under the age of 2

Presentation: skin rash and cystic skeletal defects in infants (< 2 years old)

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8
Q

What cancer is associated with a massive production of monoclonal IgM?

A

Waldenstrom Macroglobulinemia

WM is a form of plasma cell dyscrasia that causes an increase in monoclonal IgM antibodies.

Causes hyperviscosity of blood –> visual and neurologic defects

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9
Q

Below is an image of a histologic section of the skin that has been stained with a special dye that stains CD4+ T-cells dark purple. Which type of leukemia does this patient have? What is the name of the structure circled in red?

A

Mycosis Fungoides

MF is a chronic leukemia of CD4+ T-cells (mature, differentiated cells) that invade the skin and produce rash, plaques , and nodules.

Nodules in skin are called Pautrier Bodies (circled in red)

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10
Q

A 65-year-old develops pancytopenia and splenomegaly. Hairy cells are identified in the peripheral blood. He should expect:

A. Blast transformation in 3-5 years

B. CNS involvement

C. Development of lymphadenopathy

D. Durable responses to chemotherapy

A

D. Durable responses to chemotherapy

This patient has hairy cell leukemia, which is deadly unless treated with chemotherapy.

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11
Q

The acronym CRAB is associated with organ failure during what cancer?

A

Multiple Myeloma

  • hyperCalcemia - osteoclast activating factor induces breakdown of bone –> increased calcium
  • Renal insuccifiency - increased light chain production –> light chains deposited in renal tubules
  • Anemia
  • Bone lesions - from bone breakdown
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12
Q

Hashimoto’s Thyroiditis is induced in which type of cancer?

A

MALT lymphoma

MALT lymphoma is an extranodal marginal zone lymphoma.

Also associated with: Sjögren Syndrome and H. pylori infection

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13
Q

A patient with blood work has the following results. The tube on the left is normal, and the tube on the right is indicative of what cancer?

A

Acute Leukemia

Blood smear on the left shows immature lymphoblasts in the blood. Right: increased population of lymphoblasts in blood and increased SED rate.

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14
Q

A patient with CD5 upregulated and CD23 downregulation was which cancer?

A

Mantle Cell Lymphoma

Small B-cell lymphoma that expands the mantle zone of the follicle. Caused by t(11:14), which fuses cyclin D with Ig heavy chain gene.

B-cells in this class have CD5 (a normal T-cell marker) and low CD23.

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15
Q

In flow cytometry, what cellular quality is side scatter related to?

A

Granularity

Side scatter (y-axis) indicates increasing granularity.

Forward scatter (x-axis) indicates increasing size

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16
Q

True or False: Burkitt Lymphoma is CD10 positive.

A

True

CD10 is associated with B-cell tumors

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17
Q

What is the name of the cell that the arrow is pointing to? What disease is indicated by the histological slide?

A

Reed-Sternberg Cell

Hodgkin Lymphoma

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18
Q

A 10-year-old female develops bilateral ovarian masses. Histology shows sheets of lymphocytes with a “starry-sky” appearance. Which of the following is most likely to be present?

A. A chromosomal translocation juxtaposing c-myc against the Ig heavy chain promoter region

B. Characteristic ultrastructural changes

C. CD5+ B-cells

D. T-cell immunophenotype

A

A. A chromosomal translocation juxtaposing c-myc against the Ig heavy chain promoter region.

Starry-sky appearance on a histological section indicates Burkitt Lymphoma, which is caused by t(8:14).

t(8:14) causes c-myc to be fused with the Ig heavy chain gene, leading to massive B-cell proliferation (purple cells = sky)

Tingible body macrophages (“stars”) are present to eat up the B-cells that have undergone apoptosis.

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19
Q

In the flow cytometry reading, is the cell population that is analyzed monoclonal or polyclonal?

A

Monoclonal

Monoclonal populations will all have the same relative size and granularity, leading to a large spot.

Polyclonal populations would have multiple dots dispersed all across the plot.

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20
Q

In flow cytometry, what quality is forward scatter related to?

A

Size

Forward scatter indicates increasing size

Side scatter indicates increasing granularity

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21
Q

For patient who have B-cell acute lymphoblastic leukemia (B-ALL), how would a t(12:21) and a t(9:22) impact the prognosis of the patient?

A
  • t(12:21) - good prognosis
  • t(9:22) - worst prognosis
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22
Q

What is the difference between anisocytosis and poikilocytosis of RBCs?

A
  • Anisocytosis - different sizes
  • Poikilocytosis - different shapes
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23
Q

What is the normal kappa to lambda light chain ratio?

A

3:1

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24
Q

M5 Acute monocytic leukemia will involve what characteristic phenotype?

A

Gum hypertrophy

M5 AML is also known as acute monocytic leukemia. It is characterized by an infiltration of monocytes into the gums –> hypertrophy

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25
Q

Your patient’s lab results have come back and the report reads as follows:

CD19+, CD20+, CD5+, CD23+ with clonal SIg

That type of cancer is this?

A

Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Leukemia (SLL)

Markers:

  • CD19 and CD20 - B cell markers
  • CD5 - normal T-cell marker, abnormal B cell marker; indicative of CLL
  • CD23 - involved in B-cell activation and growth
  • Clonal surface Ig
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26
Q

Eradication of H. pylori in cases of gastric extranodal marginal zone (MALT) lymphoma often leads to:

A. Gastric adenocarcinoma

B. Regression of the tumor cells

C. Transformation to diffuse large-cell lymphoma

D. Transformation to mantle cell lymphoma

A

B. Regression of the tumor cells

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27
Q

What condition is indicated by the histologic section below and is it cancerous or benign?

A

Diffuse Large B-cell Lymphoma

Things to notice on histologic slide:

Large B-cells with lots of cytoplasm (not well differentiated)

Prominent nucleoli

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28
Q

Waldenstrom’s macroglobulinemia has what characteristic histological figure?

A

Dutcher Bodies

WM has dutcher bodies, which are cytoplasmic inclusions of IgM.

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29
Q

What is the difference between a leukemia and a lymphoma?

A

Leukemias are WBC cancers of the bone marrow and peripheral blood

Lymphomas are WBC cancers of the lymph nodes

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30
Q

DIC is commonly seen in patients with what type of cancer?

A

Promyelocytic leukemia (M3 AML)

Promyelocytes tend to have lots of myeloperoxidase (MPO) in their cytoplasm. This MPO clumps together and forms Auer rods.

MPO activates the coagulation cascade, which can use up clotting factors and lead to DIC

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31
Q

What cancer is associated with the histologic section below and what causes this appearance?

A

Burkitt Lymphoma

This is the typical “starry sky” appearance: lots of purple B-cells with interspersed tingible-body macrophages (clear looking cells) that eat up dead B-cells.

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32
Q

Chronic myelocytic leukemias are oftentimes associated with what genetic mutations?

A

t(9:22)

Fuses Bcr with Abl gene –> constitutively active tyrosine kinase

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33
Q

A translocation between chromosomes 14 and 18 that upregulates Bcl-2 production will cause which cancer?

A

Follicular lymphoma

t(14:18) brings the Ig heavy chain gene close to Bcl-2, an anti-apoptotic factor.

Bcl-2 prevents B-cell death, which leads to build up of B-cells in the follicles, which causes lymphadenopathy.

CD10+

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34
Q

A 60-year-old male has lymphadenopathy. Biopsy of the lymph node shows diffuse effacement of the architecture by large B-cells. The diagnosis is:

A. Chronic lymphocytic leukemia

B. Diffuse Large B-Cell Lymphoma

C. Hodgkin Lymphoma

D. Multiple Myeloma

A

B. Diffuse Large B-cell Lymphoma

Characterized by large, poorly differentiated B-cells

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35
Q

A 10-year-old boy develops gum hypertrophy and a high white blood cell count with numerous blasts. The blasts are positive for CD14 and non-specific esterase. The lineage of the blasts is:

A. Erythroid

B. Lymphoid

C. Megakaryocytic

D. Monocytoid

A

D. Monocytoid

Gum hypertrophy is associated with acute monocytic leukemia (M5 AML).

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36
Q

What is the most common leukemia of adults?

A

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

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37
Q

A 40-year-old male has splenomegaly and a WBC of 100,000 (very high). The differential count shows all stages of myeloid differfentiation with less than 5% blasts. Philadelphia chromosome t(9:22) is positive. This translocation results in:

A. An abnormal tyrosine kinase

B. Increased c-myc

C. Overexpression of an anti-apoptotic protein

D. Resistance to retinoic acid

A

A. An abnormal tyrosine kinase

t(9:22) causes chronic myelogenous leukemia by fusing the BCR and Abl genes to form a constitutively active tyrosine kinase.

B. describes Burkitt Lymphoma - t(8:14)

C. describes follicular cell lymphoma t(14:18)

D. describes acute promyelocytic leukemia t(15:17)

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38
Q

What type of cancer is associated with Dutcher Bodies?

A

Waldenstrom’s Macroglobulinemia

Dutcher bodies are cytoplasmic inclusions that are indicative of WM.

WM is a B-cell lymphoma that produces monoclonal IgM

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39
Q

Reed-Sternberg cells are associated with what type of cancer?

A

Hodgkin Lymphoma

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40
Q

What condition is associated with this histological stain?

A

Hodgkin’s Lymphoma

The cell circled in red is a Reed-Sternberg cell: a multinucleated cell with prominent nucleoli.

Reed-Sternberg cells recruit lymphocytes to the lymph node, causing the node to increase in size.

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41
Q

MALT lymphoma is associated with which bacterial strain?

A

H. pylori

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42
Q

What is the typical presentation of a precursor T-cell lymphoblastic leukemia/lymphoma?

A

Increased white count and a mediastinal mass

Mediastinal mass = T-cell involvement because of the location of the thymus

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43
Q

How is T-cell clonality determined?

A

PCR and southern blotting

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44
Q

Rouleaux formation, when RBCs stack on each other, is indicated in what cancer?

A

Multiple Myeloma

Multiple myeloma is a B-cell neoplasm that produces large numbers of monoclonal antibodies. Increased immunoglobulins in the blood decrease the zeta potential (negative charge) of the RBCs, causing them to stack.

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45
Q

What medication is ideally used in treatment of diffuse large B-cell lymphoma?

A

Rituximab

RItuximab is an anti-CD20 monoclonal antibody that binds to CD20 on all B-cells (except plasma cells) and causes them to be destroyed.

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46
Q

A 16-year-old male presents with a mediastinal mass and blasts in the peripheral blood. The cells are positive for CD7 and TdT. The best way to determine clonality is:

A. Cytochemistry

B. Serum Electrophoresis

C. Surface Immunoglobulin light chain restriction

D. T-cell receptor gene rearrangement studies by PCR or Southern blot

A

D. T-cell receptor gene rearrangement studies by PCR or Southern Blot

Mediastinal mass indicates a proliferation of cells in the thymus. CD7 is a marker for T-cells, and TdT is a marker for immature lymphoblasts.

Because this is a T-cell, it does not produce light chain and would not show any difference on serum electrophoresis. Cytochemistry would not show if this were monoclonal or polyclonal.

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47
Q

This patient’s retinal exam showed large, red blood vessels. After undergoing plasmapheresis, the vessels return to normal size. What is the name of the condition that this patient suffers from?

A

Waldenstrom’s Macroglobulinemia

WM is a plasma cell disorder in which excessive amounts of IgM are produced. Large amounts of IgM thicken the blood, which can lead to eye and neural damage.

Plasmapheresis is used to remove the excess IgM, thinning the blood back to normal viscosity.

48
Q

What is the classic technique for subtyping T-cells?

A

Southern Blotting

49
Q

B-raf gene leads to what type of cancer?

A

Hairy cell leukemia

50
Q

Patients with myelodysplastic syndromes express what type of phenotype?

A

Pancytopenia

51
Q

What drug is ideal in treating chronic myelocytic leukemia? What is the mechanism of action of this drug?

A

Imatinib

This drug will inhibit tyrosine kinase activity. Remember the chronic myelocytic leukemia (CML) is caused by a t(9:22), which fuses the BCR and Abl genes to produce a constitutively active tyrosine kinase.

52
Q

What cancer is highly responsive to all trans retinoic acid (ATRA)?

A

Promyelocytic leukemia (M3 AML)

t(15:17) causes a translocation between PML and retinoic acid receptor gene, which leads to a buildup of Auer Rods (composed of myeloperoxidase).

All-trans retinoic acid

53
Q

A 60-year-old male develops plethora. His hematocrit is 60% and erythropoietin level is low. He develops thrombotic complications. The most definitive diagnostic test would be:

A. Analysis of JAK2 mutation

B. LAP score

C. Search for Howell-Jolly bodies

D. TRAP stain

A

A. Analysis of JAK2 mutation

Plethora (increased RBC count) and low erythropoietin indicate polycythemia vera. This is a myelodysplastic disorder characterized by a mutation in JAK2.

54
Q

What CD marker is associated with B-cell monocytic lineage?

A

CD14

55
Q

What type of cancer is shown here? What special stain could be used to determine this type of cancer?

A

Hairy Cell Leukemia

Tartrate-resistant acid phosphatase (TRAP) stain

Notice the cytoplasmic projections on these large B-cells.

56
Q

There is a mutation of which gene in hairy cell leukemia?

A

B-raf

57
Q

What is the genetic translocation most commonly associated with acute promyelocytic leukemia (M3 AML)? What genes are targeted during this translocation?

A

t(15:17)

This targets the PML/RARA (retinoic acid receptor) gene.

58
Q

Analysis of your patient’s tumor with flow cytometry indicates that it is CD13+, CD33+, CD117(cKIT)+, CD15+

What type of cancer does your patient have?

A

Acute Myeloid Leukemia

CD13, CD14, and CD33 all indicate AML

CD117 (c-KIT) is a receptor tyrosine kinase that is involved in proliferation

59
Q

A 35-year-old male develops acute leukemia and disseminated intravascular coagulopathy. The blasts have numerous Auer rods. The chomosomal translocation in the blasts results in:

A. A tyrosine kinase which is coinstitutively activated

B. An anti-apoptotic protein

C. Fusion proteins which block myelocytic differentiation at the myelocytic stage

D. Increased production of c-myc

A

C. Fusion proteins which block myelocytic differentiation at the myelocytic stage

This stem describes a patient with acute promyelocytic leukemia (M3 AML).

t(15:17) fuses PML with retinoic acid receptor and prevents promyelocytes from differentiating into myelocytes.

DIC is caused by increased myeloperoxidase, which clumps together to form Auer rods.

60
Q

In what patient population are precursor T-cell lymphoblastic leukemias and lymphomas more common?

A

Adolescents

These patients tend to present with a mediastinal mass and an increased leukocyte count.

61
Q

A mutation of JAK2 tyrosine kinase is associated with which type of disorders?

A

Myelodysplastic Disorders

These disorders are characterized by increased proliferation of myeloid-derived cells (RBCs, monocytes, granulocytes, etc.).

62
Q

What is the most common form of non-Hodgkin’s lymphoma?

A

Diffuse Large B-cell Lymphoma

Remember that this type of lymphoma can be sporadic or can be caused by follicular lymphoma.

Patients in late adulthood will present with an enlarging lymph node or an extranodal mass.

63
Q

A deletion of chromosomes 5q and 7q are associated with what condition?

A

Myelodysplastic syndromes

64
Q

What CD marker is characteristic of follicular lymphoma?

A

CD10

Follicular lymphoma is caused by t(14:18), which fuses the Bcl-2 gene with the Ig heavy chain gene.

This leads to B-cells in the follicles that cannot undergo apoptosis.

65
Q

What are the proteins produced by plasma cell (multiple) myeloma that deposit into renal tubules and cause renal dysfunction?

A

Ig Light Chains

Remember that a multiple myeloma is generally a monoclonal antibody-producing plasma cell tumor. Light chains can aggregate and form clumps that get trapped in the renal tubules.

This patient would also have a large amount of light chains in his/her urine and an M spike on serum electrophoresis.

66
Q

Below is a histologic section of a liver biopsy. Which type of neoplasm does this section indicate?

A

Chronic lymphocytic leukemia/Small lymphocytic lymphoma

Remember that CLL can start in the blood, but can infiltrate the tissues. This causes the name to change to small lymphocytic lymphoma.

Leukemia = blood

Lymphoma = lymph nodes or organs

67
Q

Follicular lymphoma is a result of genetic translocation among which two chromosomes?

A

t(15:17)

This upregulates production of Bcl-2, which is an anti-apoptotic factor. This would cause an increase in size of the follicles of the lymph node.

68
Q

Mantle Cell Lymphoma is associated with which genetic translocation?

A

t(11:14)

Mantle cell lymphoma is characterized by growth of the area immediately surrounding the follicle. This is caused by fusion of cyclin D1 with Ig heavy chain, which allows the cells to move from G1 to S phase of cell cycle without restriction.

This would lead to the appearance of sheets of dark-purple B-cells with interspersed clear tingible body macrophages.

THIS IS A SIMILAR APPEARANCE TO BURKITT LYMPHOMA! PAY ATTENTION TO QUETION STEM

69
Q

What are the 4 main symptoms that are associated with multiple (plasma cell) myeloma?

A
  • HyperCalcemia
  • Renal insufficiency
  • Anemia
  • Bone lesions

Remember that CRABs have multiple legs. So multiple myeloma causes these 4 “crab” symptoms.

70
Q

What is the name of the structure that the blue arrow is pointing to and which type of blood cancer is it associated with?

A

Auer Rod

Associated with acute myeloid leukemia (AML)

Auer rods are made when myeloperoxidase (MPO) clumps together.

Remember: MYELOperoxidase is only in MYELOid cells

71
Q

What is another name for Hand-Schuller-Christian Disease and which ages does this typically occur in?

A

Langerhans Cell Histiocytosis

Typically occurs in people above the age of 3

Langerhans Cell Histiocytosis is divided into two types:

Letterer-Siwe: affects kids below age 2

Hand-Schuller-Christian Disease: affects people above age 3

Just look at the names. Letterer-Siwe has 2 names, so kids 2 and below get it. Hand-Schuller-Christian disease has 3 names, so people above age 3 get it.

72
Q

A 60-year-old male presents with retroperitoneal lymphadenopathy. A lymph node biopsy shows effacement of the normal architecture by a nodular infiltrate of CD10 positive clonal B-cells. The chromosomal translocation in this lymphoma results in:

A. Constitutive activation of tyrosine kinase

B. Increased cyclin D levels

C. Inhibition of apoptosis

D. Over expression of a growth hormone receptor

A

C. Inhibition of apoptosis

Things to focus on in the stem: nodular architecture, CD10+ clonal cells

These two clues indicate follicular lymphoma, a B-cell tumor that has a t(14:18), which fuses Bcl-2 with Ig heavy chain gene to inhibit apoptosis.

73
Q

Hairy cell leukemia is associated with which CD markers?

A
  • CD19 and CD20 (normal B-cell markers)
  • CD11c
  • CD103
  • Monoclonal surface Ig (SIg)
74
Q

An 80-year-old man has pancytopenia. A bone marrow biopsy is hypercellular. There is tri-lineage dysplasia and karyotypic analysis shows del 7. He has:

A. Chronic lymphocytic lymphoma

B. Chronic myelogenous leukemia

C. Hairy cell leukemia

D. Myelodysplasia

A

D. Myelodysplasia

Myelodysplastic disorders typically involve more than one cell type that is proliferating. These disorders are caused by JAK2 mutations, which arise from deletions of chromosome 5 (del 5) or 7 (del 7).

75
Q

Follicular lymphoma, SLL/CLL, marginal zone lymphoma, and Waldenstrom Macroglobulinemia are all considered to be indolent or aggressive types of B-cell non-Hodgkin lymphomas?

A

Indolent

This means that these lymphomas typically do not cause pain and gradually develop over time.

76
Q

Mutations or deletions in which chromosomes are responsible for myelodysplastic syndromes?

A

Chromosomes 5 and 7

These mutations/deletions cause an abnormal JAK2, which leads to increased proliferation of myeloid cells.

77
Q

In Hodgkin’s lymphoma, what do the subcategories of A and B indicate? Correlate these with survival rate.

A
  • A - no associated symptoms (better prognosis)
  • B - associated symptoms (worse prognosis)
78
Q

Mantle cell lymphoma has which CD marker upregulated? Which CD marker is absent in this condition that differentiates it from CLL?

A

CD5+ and CD23-

79
Q

What class of plasma cell tumors generally presents with bone masses and production of paraproteins?

A

Plasma cell dyscrasias

These include: multiple myeloma (most common), monoclonal gammopathy of undetermined significance (MGUS), and Waldenstrom Macroglobulinemia.

The para proteins in this case are increased immunoglobulins.

80
Q

Floret cells are most commonly seen in what type of cancer?

A

Human T-cell Lymphoma/Leukemia

Caused by Human T-lymphotropic virus 1 (HTLV-1)

Floret (flower) cells are CD4+ T-cells that contain lots of cytoplasm and have multi-lobulated nuclei that makes them look like a flower.

81
Q

A 23-year-old female is diagnosed with nodular sclerosing Hodgkin Disease. She has involvement of her mediastinal and axillary nodes. She has fevers and night sweats. Her stage is:

A. 1A

B. 1B

C. 2A

D. 2B

A

D. 2B

This is broken down into stage of lymph node involvement and presence of symptoms.

Stage 1 - one lymph node involved

Stage 2 - more than one lymph node involved on same side of diaphragm

A - no symptoms

B - presence of symptoms

82
Q

When testing for T-cell leukemia, what tests should be done? What about for B-cell leukemia?

A

T-cells: PCR and southern blot

B-cells: serum electrophoresis and flow cytometry

83
Q

A 60-year-old man develops lymphocytosis. He is asymptomatic. The best way to determine if the cells are neoplastic is:

A. Coomb’s test

B. Flow cytometry for B-cell surface Ig light chain restriction

C. Myeloperoxidase stain

D. TdT stain

A

B. Flow cytometry for B-cell surface Ig light chain restriction

If the flow cytometry shows a large concentration of cells within the same range of size and granularity, it is monoclonal. If it shows diffuse, scattered pattern, it is polyclonal. Large increase in monoclonal cells usually indicates neoplasia.

Coombs tests for autohemolytic anemia

MPO stain would be used for myeloid cells

TdT indicates that a cell is a blast (immature), but does not indicate type.

84
Q

What are the general features of lymphoid neoplasms?

A

Most will be made up of B-cells

All of them are clonal

85
Q

What makes a leukemia acute? What makes a leukemia chronic?

A

Acute leukemias consist of pre-mature (blast) cells that comprise more than 20% of the leukemic cells.

Chronic leukemias consist of mature cells.

86
Q

Using the flow cytometry data below, identify each of the following cell types (labelled A, B, and O).

A

A = lymphocytes (small and agranular)

B = monocytes (slightly larger, slightly more granular)

C = granulocytes (large and granular)

87
Q

Explain why individuals with HIV or who have just had a transplant are most likely to develop lymphomas.

A

These individuals are immunocompromised, which downregulates immune surveillance of tumor antigen.

88
Q

A patient with gum hypertrophy most likely has what type of cancer?

A

Acute monocytic leukemia (M5 AML)

89
Q

What is the most common malignancy of childhood?

A

B-cell acute lymphoblastic leukemia (B-ALL)

Can occur in all ages, but mainly in children.

> 80% of patients are cured with chemotherapy

90
Q

What cell type is characteristically seen in Hodgkin Lymphoma histology?

A

Reed-Sternberg cells

91
Q

In B-cell acute lymphoblastic lymphoma (B-ALL), what karyotypic qualities suggest good outcumes and poor outcomes?

A
  • Good outcomes - t(12:21)
  • Bad outcomes - t(9:22)
92
Q

What cell surface marker is present in precursor B-cell leukemia (B-ALL)?

A

CD-10

93
Q

True or False: Patients with precursor T-cell lymphoblsatic leukemia (T-ALL) will be CD3 negative.

A

True.

CD3 are normally located in the cytoplasm of T-cells when they are immature (blasts).

When T-cells mature, the CD3 is expressed on the cell surface.

94
Q

What type of cancer is associated with myasthenia gravis?

A

Thymoma

Also associated with SLE and RA

95
Q

What physical findings would be present in a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)?

A
  1. Lymphadenopathy
  2. Hepatosplenomegaly
  3. Shingles
96
Q

A thymoma is associated with what secondary autoimmune condition?

A

Myasthenia Gravis

97
Q

In what type of syndromes are mature myeloid cells not able to be produced?

A

Myelodysplastic syndromes

98
Q

Mantle cell cancer has expression of which cyclin molecule?

A

Cyclin D1

Mantle cell lymphoma is a t(11:14) that fuses cyclin D1 with the Ig heavy chain gene. Cyclin D1 causes cells to progress from G1 to S phase of cell cycle.

99
Q

What type of neoplasm is this slide suggestive of?

A

Chronic lymphocytic leukemia (CLL)

Two things to look for in this slide: smudge cells and lymphocytes with “soccer ball” nuclei.

If these were located in a lymph node or organ tissues, they would be classified as small lymphocytic lymphoma (SLL)

100
Q

A B-cell chronic lymphoid leukemia will have B-cells with what CD markers?

A

CD5, CD19, CD20, CD23, and surface Ig

101
Q

What age group is mostly affected by Hodgkin Lymphoma?

A

10-30

102
Q

Using the flow cytometry read out below, what cancer does this indicate?

A

B-cell acute lymphoblastic leukemia (B-ALL)

The readout shows CD19+ and CD10+ cells. These indicate immature (blast) B cells.

Remember that CD19 = immature B-cells and CD20 = mature B-cells

Most leukemias will have a mix, but CD19 and 20 indicate B-cells

103
Q

What is the prognosis of acute myeloid leukemia?

A

15-30% survival

104
Q

What cell surface marker typifies a lymphoid stem cell?

A

CD34

105
Q

A patient with T-cell acute lymphoid leukemia is likely to have mutations of which gene?

A

NOTCH-1

106
Q

Myeloperoxidase (p-ANCA) is positive for what type of cancer?

A

All acute myeloid leukemias

Acute promyelocytic leukemia (M3 AML) will have the highest concentration of myeloperodixase (MPO) on a slide shown as Auer Rods.

107
Q

A 4-year-old female presents abruptly with petechiae, bruising, fatigue, and bone pain. The white blood cell count is elevated with 60% blasts. By flow cytometry the blasts are positive for CD19 and CD10. The lineage of the blasts is:

A. Myeloid

B. Monocytic

C. Precursor B-cell

D. T-cell

A

C. Precursor B-cell

CD19 indicates an immature B-cell, so you can rule out all of the rest of the answers.

108
Q

A 4-year-old female presents abruptly with petechiae, bruising, fatigue, and bone pain. The white blood cell count is elevated with 60% blasts. By flow cytometry the blasts are positive for CD19 and CD10. The prognosis of the patient with current chemotherapy is:

A. Cure impossible, but long term survival

B. Death within 3 to 6 months is inevitable

C. High probability of cure with chemotherapy

A

C. High probability of cure with chemotherapy

109
Q

NOTCH1 mutations are associated with what type of cancer?

A

T-cell acute lymphoblastic leukemia (T-ALL)

These mutations are determined via PCR and southern blotting

110
Q

What cancer causes calvarial bone defects, diabetes insipidus, and exopthalmos?

A

Langerhans Cell Histiocytosis

111
Q

Why is it more difficult to subdifferentiate between T-cell types?

A

T-cells don’t have a light chain equivalent

112
Q

An M3 acute myeloid leukemia (AML) will result in which type of cancer?

A

Acute promyelocytic leukemia

This is characterized by a t(15:17) mutation causing PML to fuse with retinoic acid receptor. This prevents promyelocytes from maturing.

113
Q

A 6-year-old female has lytic calvarial lesions, diabetes insipidus, and exopthalmos. A biopsy of a lytic lesion shows numerous eosinophils and histiocytes. EM shows Birbeck granules. The neoplastic cells are:

A. Eosinophils

B. Langerhans cells

C. Osteoclasts

D. Plasma cells

A

B. Langerhans cells

Lytic calvarial lesions (pictured below), diabetes insipidus, exopthalmos, numerous histiocytes, and Birbeck granules all point toward Langerhans Cell Histiocytosis.

114
Q

Differentiate between type 1 and type 2 malignant thymomas.

A
  1. Type 1 - cytologically benign, but invasive
  2. Type 2 (thymic carcinoma) - cytologically and biologically malignant
115
Q

What are the three leukemias that induce splenomegaly?

A
  1. Chronic myeloid leukemia (CML)
  2. Chronic lymphoid leukemia (CLL)
  3. Hair cell leukemia
116
Q

What is the name of the structure circled in yellow and in which condition will these be seen?

A

Bribeck Granules

Langerhans cell histiocytosis

These are also called “zipper” and “tennis racket” granules

Block 8 - Autoimmunity, Neoplasia, and Hematologic Disease (20 decks)

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