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SBL Test 1 > Review: Lymphoid Leukemias > Flashcards

Review: Lymphoid Leukemias Flashcards

1
Q

____ = marker that is ONLY found on pre-B cells. If present, what does it mean?

A

CD10+

Acute process = MC in children

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2
Q

What marker can we use to differentiate a acute myeloid vs lymphoblastic leukemia?

A

TdT+ = only found on pre B/T cells (lymphoid cells)

NOT found in myeloid cells.

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3
Q

General differences between acute and chronic leukemias?

A

Acute = rapid onset of symptoms, involve -blasts, MC in children

Chronic = slower onset of symptoms, involve -cytes, MC in adults

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4
Q

MC leukemia in childhood?

A

Acute lymphoblastic leukemia

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5
Q

All children with thrombocytopenia have ________, until proven otherwise

A

ALL (Acute lymphoblastic leukemia)

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6
Q

ALL (Acute lymphoblastic leukemia) =

A

Malignant proliferation of lymphoblasts in the BM (either T-cells or B-cell)

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7
Q

Majority of ALLs are caused a proliferation of what cells?

A

B-cells (70%)

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8
Q

ALL (Acute lymphoblastic leukemia)

MC in =
Genetics =
RF

A

MC = children. If adults = worse prognosis

90% have hyperploidy (>50 Chr)
RF = Hispanic, M, Down Syndrome

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9
Q

ALL histology

A

Lympoblasts in the peripheral blood (large cells with large nucleus)

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10
Q

Acute Lymphoblastic Leukemia Symptoms

A

Rapid onset of symptoms (days - weeks)
Symptoms are due BM suppression:

  1. INC risk of infections and fever (neutropenia)
  2. Bleeding/hemorrhaging (thrombocytopenia)
  3. Anemia (fatigue, weak, pallor)

Mass effects (MC than in AML)

  1. Bone pains due to BM expansion
  2. Generalized LAD and Hepatosplenomegaly (cells go from blood to LN, spleen and liver)
  3. Big testicles
  4. CNS: spreads to menininges: HA/nerve palsies
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11
Q

Acute lymphoblastic Leukemia: Bad prognosis factors

A
  1. Under 2 or adolescence/young adult
  2. t(9:22): Philadelphia Chr
  3. > 100,000 -blasts in blood
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12
Q

Acute lymphoblastic Leukemia: Good prognosis factors

A
  1. 2-10 YO
  2. Hyperploidy
  3. Low WBC counts
  4. Trisomy 4, 7, 10
  5. t(12;22) Chr
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13
Q

Treatment of ALL

A

Chemo (85% cure rate); relapse can occur in 2 santuary sites (CNS and testes)

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14
Q

B-cell ALL

Markers
Genetics

A
  1. CD10+
  2. TdT
  3. t(12:22) => TEL-AML1 fusion gene (RUNX1 and ETV6 proteins): impairs differentiation of blasts (MC in children)
  4. t(9:22) => BCL-ABL fusion gene => 20-30% of ALL in adults (worse prognosis)
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15
Q

T-cell ALL differences from B-cell ALL

Markers
Genetics

Other:

A
  1. CD2-8, except CD6+.
  2. NOT CD10+

GOF NOTCH1 mutation (in 70% of all)

MC in adolescent males (10-20s)

Presents as a mediastinal mass (thymic lymphomas) with pleural effusion, and can compress structures:
can cause superior vena cava syndrome (SVC) and compress trachea (trouble breathing)

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16
Q

What is the MC leukemia of adults in the Western World?

A

Chronic Lymphocytic Leukemia

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17
Q

What leukemia is MC in adults in Japan, West Africa and Carribean?

A

Adult T-cell leukemia/lymphoma

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18
Q

Adult T-cell leukemia/lymphoma =

A

Malignant proliferation of CD4+ T-cells due to HTLV-1 infection.

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19
Q

Adult T-cell leukemia/lymphoma:

Pathogenesis
Clinical presentation

A

HTLV-1 (RNA virus) infects CD4 T-cells: produces Tax protein => + NF-kB => too many CD4+ T-cells

Rapidly progressive symptoms => fatal in months, even with aggressive chemo

  1. Skin lesions (ulcers, nodules, popular rash)***
  2. Lytic bone lesions  Hypercalcemia (Don’t confuse with MM*)
  3. Lymphocytosis
  4. Lymphadenopathy
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20
Q

Hairy cell leukemia =

Mc in?

A

Rare malignant proliferation of mature B cells, with filamentous hair-like projection.

Middle-aged white men (55YO)

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21
Q

Hairy cell leukemia markers

A
  1. CD103 **
  2. CD11c **
  3. CD25
  4. CD19/20/22
  5. TRAP (+) = Tartrate-resistant acid phosphatase
  6. Annexin A1 (1+)
  7. Surface IgG
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22
Q

Hairy cell leukemia

Genetics and pathogenesis

A

BRAF (serine/threonine kinase) activating point mutation: valine => glutamate at residue 600 (same as _____)

INC RAS-MAPK pathway

23
Q

Hairy cell leukemia histology

A
  1. Hairy cells: cells have hair-like cytoplasmic projections
  2. In stained smears = round/folded nuclei with pale amounts blue granular cytoplasm
24
Q

Hairy cell leukemia unique features

A
  1. Massive splenomegaly = red pulp becomes engorged, causing atrophy and obliteration of white pulp
  2. Dry tap on BM biopsy = hairy cells cause marrow fibrosis
  3. TART (+)
25
Q

Hairy cell leukemia clinical presentation

and prognosis

A

Middle Aged White M (55YO) presents with abdominal pain due to splenomegaly and pancytopenia (due to infiltration of BM and spleen)

  1. . Fatigue/weakness
  2. . Bone marrow suppression (anemia and decreased platelets)
  3. Peripheral LAD is rare

Excellent: Indolent and exceptionally sensitive to “gentle” chemotherap

26
Q

Hairy cell leukemia complications

A

↑↑↑ susceptibility to atypical mycobacterial infections

27
Q

What are plasma cell neoplasia (dyscrasias)?

A

Mature B-cells gain mutations => differentiate into plasma cell that ALWAYS release clusters of monoclonal Ig Ab or Ig fragment from bone marrow.

28
Q

Most common plasma cell dyscrasia

A

MGUS (Monoclonal Gammopathy of Uncertain Significance)

29
Q

Most common plasma cell malignancy

A

Multiple Myeloma

30
Q

Name 3 plasma cell dycrasias

A
  1. Multiple Myeloma (Plasma cell myeloma)
  2. MGUS (Monoclonal Gammopathy of Undetermined Significance)
  3. Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma)
31
Q

Multiple Myeloma (Plasma cell myeloma) =

A

Malignant proliferation of monoclonal plasma cells (all same type) in BM, producing excess M-proteins, that often produce lytic bone lesions.

32
Q

What Ig’s do plasma cells in Multiple Myeloma make?

A
  1. IgG (55%)
  2. IgA (25)
  3. Bence Jones proteins (light chains only
33
Q

Multiple Myeloma is MC in who?

A

65-70 YO African M

34
Q

Multiple Myeloma genetics

A

Gene ABNLity dictates prognosis

  1. IgH rearrangements
  2. 13q /17q deletions
  3. t(4:14)
  4. Overexpression of cyclin-D
35
Q

Pathogenesis of Multiple Myeloma

A

Myeloma cells are dependent on IL-6 for proliferation. Without IL-6, plasma cells will not proliferate. Thus, patients with MM will have high levels of IL-6.

Clinical features are due to:

  1. Effects of growth of plasma cell in tissues (particularly bone),
  2. Production of excess Ig
  3. Suppression of NL humoral immunity.

Excess growth of plasma cells and release of cytokines from myeloma cells causes osteoclast -mediated bone resorption => hypercalcemia, pathological fractures (especially in vertebral column), back pain => lytic bone lesions => hypercalcemia,
Hypercalcemia => Neurological signs and symptoms (confusion, weakness, lethargy, kidney stones + short QT)

If excess light chains = renal failure and amyloidosis:
Excess production of light chains exceed capacity of proximal tubule (where they are typically broken down and reabsorbed). Thus, they reach the distal tubule, combine with Tamm-Horsfall mucoproteins (THP), forming obstructing cats => light chains in urine (Bence Jones proteins). Hypercalcemia also impairs kidneys ability to concentrate urine (polyuria => volume contraction) decreased GFR)

36
Q

Subtypes of myeloma

A
  1. Solitary myeloma (plasmacytoma)
    • Extramedullary solitary mass in bone of ST, with no/little BM involvement
    • Aggressive progression overtime
  2. Smoldering myeloma (middle ground between MM and MGUMS)
    • Asymptomatic + 10-30% plasma cells in
    marrow + high plasma M component (> 3gm/dL) and NO lytic bone lesions
    • 75% progress to MM in 15 years
37
Q

Complication of Multiple Myeloma

A
  1. Recurrent bacterial infections : due to ↓ production of NL immunoglobulins = MCC of death
    - Step pneuo
    - Staph Aureus
    - E. coli
  2. Renal insufficiency
  3. AL amyloidosis
  4. Hyperviscosity (MC in lymphoplasmacytic lymphoma) = INC Ig in blood increase viscosity => decrease BF and sludging => CNS problems (HA, dizziness, coma, visual impairment).
38
Q

Clinical Presentation of MM

A

Patients often present with pathologic fracture and pain (due to plasma cell growth in tissues, particularly bone).

CRAB

  1. HyperCalcemia: due to bone resorption => neurological signs and symptoms (confusion, weakness, lethargy, kidney stones + short QT)
  2. Renal failure: due to formation of Bence Jones proteins and hypercalcemia
  3. Anemia/AL amyloidosis
  4. Bone lytic lesions/ Bone fractures (esp in vertebral column)/ Back pain
  5. AL amyloidosis
  6. Recurrent bacterial infections: due to ↓ production of NL immunoglobulins = MCC of death.
  7. 7% develop hype viscosity symptoms.
39
Q

Diagnosis of Multiple Myeloma

A
  1. UPEP (urine protein electrophoresis) = detects light chains (Bence Jones proteins); urine dipstick will be (-) because only detects albumin

Need both:

  1. SPEP (Serum protein electrophoresis): M-spike on gamma fraction => detects excess Ig
  2. BM biopsy: (needed for definited dx):
    • >30% plasma cells with atypia.
    • >3gm/dL of Ig (M protein) and/or >6mg/dL or urine Bence-Jones protein.
40
Q

Good and bad prognosis indicators of Multiple Myeloma

A
  1. Poorer prognosis =
    - 13q/17q deletion,
    - high levels of IL-6,
    - t(4:14)
  2. Good prognosis =
    Overexpression of cyclin-D
41
Q

What do you see on XR in Multiple Myeloma

A

Lytic bone lesions that causes “moth eaten skeleton”.

42
Q

What do you see on histology in Multiple Myeloma

A
  1. Rouleaux formation: RBC form linear array due to elevated M proteins in plasma. This finding is seen in any case where Ig levels are high.
  2. Mott Cells = multiple grape-like cytoplasmic droplets
43
Q

Markers for Multiple Myeloma

A
  1. High levels of IL-6
  2. CD138 (Syndecan-1)*
  3. CD56
44
Q

What is MGUS?

A

Asymptomatic proliferation of plasma cells in BM, producing excess M proteins, but not enough to qualify as MM.
• Serum M proteins is LESS than 3 gm/dL
• BM has <10% monoclonal plasma cells

May lead to MM.

45
Q

How do we determine if MGUS is progressing to Multiple Myeloma?

A

Periodically assess serum M levels and Bence Jones proteinuria

46
Q

Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma)

A

Malignant B-cell lymphoma, where B-cells differentiate into plasma cells and produce excessive amounts of IgM Ab, leading to hyperviscosity symptoms

47
Q

Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) is MC in who?

A

60-70YO older adults

48
Q

In Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma), why does IgM Ab cause hyperviscosity?

What other disease does it resemble?

A

IgM Ab are pentamer, a large MACROglobulin, causing hyperviscosity

Resembles CLL/SLL, but differs in that majority of tumor cells differentiate into plasma cells

49
Q

Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) symptoms?

A
  1. Non-specific symptoms: weakness, fatigue, WL
  2. 50% of patients = LAD, hepatosplenomegaly
  3. Symptoms of hyperviscosity:
  4. CNS symptoms: HA, dizziness, coma
  5. Retinopathy (visual impairment)
  6. Bleeding
  7. Cryoglobulinemia => Raynaud’s and cold urticaria
50
Q

How is Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) different from MM?

A
  1. NO complications from free light chains: renal failure and amyloidosis
  2. NO osteolytic bone lesions
51
Q

How do we diagnose Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) ?

A

SPEP: M-spike due to IgM

52
Q

Prognosis of Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) ?

A

Incurable, but symptoms can be alleviated with plasmaphoresis to remove IgM Ab

53
Q

Histology of Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma) ?

A
  1. Marrow infiltrate of lymphocytes, plasma cells, and plasmacytoid lymphocytes
  2. Russel bodies: cytoplasm
  3. Dutcher bodies: nucleus

SBL Test 1 (15 decks)

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