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Review / Wrong Answers > DIT review - Heme 4 > Flashcards

DIT review - Heme 4 Flashcards

1
Q

What is the most common type of NHL

A

Diffuse large B-cell

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2
Q

What is the translocation in follicular lymphoma

A
  • t(14;18) = heavy chain Ig (14) and BCL-2 (18)
    • Overexpression of BCL-2 = decreased apoptosis
      • Recall that BCL2 stabilizes mitochondrial membranes, preventing leakage of cytochrome C, and thus preventing apoptosis
      • Follicle is where B-cells are produced, so lack of apoptosis = malignant B-cell proliferation
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3
Q

What is the translocation in Burkitt lymphoma

A
  • t(8;14) = c-myc (8) and heavy chain Ig (14)
    • Overexpression of c-myc oncogene
    • THINK: “B” in Burkitt = 8 and “tt” = 14
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4
Q

Histology of Burkitt lymphoma

A
  • “Starry sky” appearance – sheet of lymphocytes with interspersed “tingible body” macrophages
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5
Q

Presentations of Burkitt lymphoma

A
  • Different forms:
    • Endemic/African form = jaw
    • Sporadic form = abdomen
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6
Q

Translocation in mantle cell lymphoma

A
  • t(11;14) = cyclin D1 (11) and heavy chain Ig (14)
    • Overexpression of cyclin D1 = G1 to S transition
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7
Q

What is small lymphocytic lymphoma

A
  • Lymphoma equivalent of CLL
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8
Q

Translocation in marginal zone lymphoma

A
  • t(11;18)
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9
Q

What is a MALToma

A
  • Marginal zone lymphoma within lymphoma a of mucosal tissue
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10
Q

What diseases are associated with marginal zone lymphoma

A
  • Associated with chronic inflammatory states:
    • Sjogren syndrome
    • Hashimoto thyroiditis
    • H. pylori
  • The marginal zone is only produced when the cells in germinal center are activated (i.e. inflammation), so it makes sense that this lymphoma occurs in chronic inflammatory states
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11
Q

What are the T-cell NHL’s

A

Adult T-cell lymphoma

Mycosis fungoides

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12
Q

What disease is associated with adult T-cell lymphoma

A

HTLV

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13
Q

What is Sezary syndrome

A

If malignant T-cells of Mycosis fungoides leave the skin lesions and get into the blood it is called Sezary Syndrome

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14
Q

What cancer is associated with t(8;14), and what is the effect of the translocation

A
  • Burkitt lymphoma
  • C-myc activation
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15
Q

What cancer is associated with t(9;22), and what is the effect of the translocation

A
  • CML, rarely ALL
  • BCR-ABL oncogene (constitutively active tyrosine kinase)
  • Responds to Imatinib (tyrosine kinase inhibitor)
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16
Q

What cancer is associated with t(11;14), and what is the effect of the translocation

A
  • Mantle cell lymphoma
  • Cyclin D1 activation
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17
Q

What cancer is associated with t(14;18), and what is the effect of the translocation

A
  • Follicular lymphoma
  • BCL-2 activation
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18
Q

What cancer is associated with t(15;17), and what is the effect of the translocation

A
  • APL (subtype of AML)
  • Responds to all-trans retinoic acid
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19
Q

What is the most common leukemia in childre

A

ALL

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20
Q

What serum markers indicated ALL

A
  • TdT (+) and PAS (+)
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21
Q

What disorder is associated with ALL

A

Down syndrome

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22
Q

Presentation of T-ALL

A
  • May present as mediastinal/thymic mass
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23
Q

Marker indicative of B-ALL

A

CD10+

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24
Q

Translocation associated with B-ALL

A
  • t(12;21) = better prognosis
  • t(9;22) = poor prognosis
25
Markers and histology associated with AML
* MPO (+) and PAS (+) * Auer rods * Crystal aggregates of MPO * Especially seen in Acute promyelocytic leukemia, AML
26
Risk factors for AML
Chemotherapy, radiation, myelodysplastic syndrome, Down syndrome
27
Common presentation of AML
* DIC is a common presentation * Auer rods activate coagulation cascade
28
Treatment of AML
* Treatment: * Retinoic acid * Think: * Auer sounds like Fuer (Hitler) – Sound of music * I am 16 going on 17 (15;17) * Poke Hitler in the eye with a carrot – treat with retinoic acid
29
Common cell markers in CLL
* 95% have B-cell markers (rather than T-cell) -\> CD20+ and CD5+
30
Histology of CLL
Smudge cells * THINK: CLL = Crushed Little Lymphocytes = smudge cells
31
What is Richter transformation
Progression of CLL to aggressive lymphoma, usually diffuse large B-cell lymphoma
32
What cancer is associated with autoimmun hemolytic anemia and why/
* CLL (usually involves B-cells) may cause autoimmune hemolytic anemia (both warm of cold cell agglutinins) * If neoplastic B-cells do make Ig, it will be defective and can attack the bod
33
Common complication of CLL
* Hypogammaglobulinemia * Neoplastic B-cells don’t make Ig
34
What is the leukemic version of small cell lymphocytic lymphoma
CLL
35
What are the distinguishing features of hairy cell leukemia
* Neoplastic proliferation of mature B-cells with hairy cytoplasmic processes * Causes bone marrow fibrosis – dry tap on aspiration * Stains TRAP (+)
36
Treatment of hairy cell leukemai
* Treat with Cladribine (adenosine deaminase inhibitor)
37
What are the myeloproliferative disorders
Aka chronic leukemias of myeloid cells: - Chronic myeloid leukemia (CML) - Polycythemia vera - Essential thrombocytopenia - Myelofibrosis
38
Translocation associated with CML
* t(9;22) – Philedalphia chromosome * THINK: Carry Mai, Lord, because she is able * Mai’s birthday is 9/22 * BCR-ABL (because she is able)
39
What does CML progress to?
* May progress to AML (80%) or ALL (20%)
40
Treatment of CML
* Responds to Imatinib (bcr-able tyrosine kinase inhibitor)
41
Mutation in polycythemia vera
* JAK2 kinase mutation
42
Presentation of polycythemia vera
* Plethora (flushed face) * Blurred vision and HA (due to hyperviscosity) * Thrombosis (e.g Budd Chiari) * Itching after bathing * Erythromelalgia (severe, burning pain and red-blue coloration in the extremities)
43
Mutation in essential thrombocytopenia
* JAK2 kinase mutation
44
Presentation of essential thrombocytopenia
* Increased bleeding – too many platelets * Thrombosis – dysfunctional platelets
45
What is myelofibrosi
* Obliteration of bone marrow with fibrosis
46
Mutation in myelofibrosis
* JAK2 kinase mutation
47
Presentation of myelofibrosis
* “Teardrop” RBCs – due to marrow fibrosis * Splenomegaly – extra-medullary hematopoiesis
48
Describe the problem in myelodysplastic syndrome
* Defect in cell maturation of all non-lymphoid lineages * Stem-cell disorder and dysplasia involving ineffective hematopoiesis * Cause by de novo mutations or environmental exposures
49
Histology of myelodysplastic syndrome
* Pseudo-Pelger-Huet anomaly * Neutrophils with bilobed nuclei, connected by a thin strand * Typically seen after chemotherapy
50
Describe the classic presentation of multiple myeloma
* Anemia – plasma cells packed in bone marrow inhibit production of other cells * Renal insufficiency – excessive antibodies plug up kidney and form casts * Back pain – plasma cells stimulate osteoclasts * Hypercalcemia – plasma cells stimulate osteoclasts
51
What changes in immunoglobulin levels do you see in multiple myeloma
* Increased production of IgG and IgA * M spike of serum electrophoresis (M spike = increased gamma) * Increased risk of infection (monoclonal antibodies lack antigenic diversity)
52
What is the X-ray finding in multiple myeloma
* Lytic bone lesions * Plasma cells activate RANK on osteoclasts
53
Histology of multiple myeloma
* Rouleaux formation of RBCs * Increased serum protein leads to decreased charge between RBCs
54
What are some of the effects of increased immunoglobulin in multiple myeloma
* Primary AL amyloidosis * Ig light chains in urine (Bence Jones protein) * Do not show up on routine urinalysis * Shows up on urinary protein electrophoresis * Renal damage * Excessive antibodies plug up kidney and form casts
55
Findings in Waldenstrom macroglobulinemia
* M spike due to increased IgM (vs. Multiple myeloma with IgM and IgA) * Hyperviscosity: * Blurred vision * Raynoud phenomenon * Amyloidosis * No lytic bone lesions (vs. multiple myeloma)
56
Describe the presentation of monoclonal gammopathy of undetermined significance (MGUS)
* Monocloncal proliferation of plasma cells * Production of monoclonal immunoglobulins = M spike * No symptoms * 1-2% progress to multiple myeloma
57
Positive markers found in Langerhans cell histiocytosis
* S-100 (+) and CD1a (+)
58
Presentation of Langerhans cell histiocytosis
* In children * Lytic bone lesions * Skin rash * Recurrent otitis media

Review / Wrong Answers (140 decks)

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