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CMBM Exam 5 > Leukemias > Flashcards

Leukemias Flashcards

1
Q

Blood Cell Lineages Chart

A
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2
Q

Normal Bone Marrow Cellularity

A
  • 100 minus the pts. age
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3
Q

Causes of Leukemia Chart

A
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4
Q

Causes of Leukemia

A
  • Oncoproteins cause an arrest in maturation
  • Mutations in transcription factors that give tumor cells a growth advantage
  • Genetic diseases that promote genomic instability have greater likelihood of developing leukemia
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5
Q

Leukemia

A
  • CBC almost always abnormal
  • Abnormalities in at least 2 major lineages
  • Anemia usually normocytic, normochromic
  • WBC elevated in >50% of cases due to circulating blasts
  • 20% pts. will have WBC >100 x 10^9/L
  • Rare cases are leukopenic
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6
Q

Monoclonal Antibody Markers

A
  • Major lymphoid markers: CD3 (T-cells), CD20 (B-cells)
  • General blast marker: CD34
  • Lymphoblast marker: TdT (terminal deoxytransferase)
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7
Q

Acute vs. Chronic

A
  • Acute refers to a process involving immature cells (notably blasts) i.e.:

*acute myeloid leukemia

*acute lymphoblastic leukemia

  • Chronic refers to a process involving mature cells i.e.:

*chronic lymphocytic leukemia (B-cells)

*Note: chronic myelogenous leukemia is classified as a myeloproliferative neoplasm

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8
Q

AML Epidemiology

A
  • 70% of all acute leukemia
  • Most cases seen in adults
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9
Q

AML Clinical Presentation Chart

A
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10
Q

Acute Myeloid Leukemia

A
  • Defined as finding >20% myeloid blasts in bone marrow, although certain genetic findings define the diagnosis even w/ <20% blasts
  • Auer rods; specific for blasts of myeloid lineages, most commonly seen in acute promyelocytic leukemia
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11
Q

AML WHO Classification

A
  • AML w/ recurrent chromosomal rearrangements

*t(8;21); CBFalpha/ETO fusion gene

*inv(16); CBFbeta/MYH11 fusion gene

*t(15;17); RARalpha/PML fusion gene (one of the most important ones to recognize b/c it can be deadly within a short amount of time)

*t(11a23;v); diverse MLL fusion genes

  • Defines leukemia even if <20% blasts are present
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12
Q

Acute Promyelocytic Leukemia (APML)

A
  • t(15;17) PML-RARalpha disrupts terminal differentiation of granulocytes
  • Toxic enzymes from Auer rods = disseminated intravascular coagulation
  • Must treat w/ all-trans-retinoic acid (ATRA)
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13
Q

Acute Megakaryocytic Leukemia

A
  • Megakaryoblasts predominate
  • Huge blasts w/ cytoplasmic blebs
  • Can be seen in Down Syndrome
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14
Q

Acute Lymphoblastic Leukemia (ALL)

A
  • Cell of origin is usually B-cell precursor
  • Less cytoplasm than AML, no Auer rods
  • More dense chromatin, fewer nucleoli, usually lack cytoplasmic granules
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15
Q

ALL Clinical Presentation

A
  • Present more acutely
  • Most common leukemia in children
  • Symptoms of bone marrow suppression (low counts)
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16
Q

Immunoprofile of ALL

A
  • TdT+
  • CD19+ early B-cell differentiation
  • CD10+ (marker of early pre-B-cell differentiation aka CALLA)
17
Q

ALL Prognosis

A
18
Q

Chronic Leukemias

A
  • B-cell chronic lymphocytic leukemias

*chronic lymphocytic leukemia (CLL)

*B-cell prolymphocytic leukemia (PLL)

*Hairy cell leukemia (HCL)

  • T-cell chronic lymphoid leukemia

*T-cell prolymphocytic leukemia (T-PLL)

*T-cell large granular lymphocyte leukemia

*Adult T-cell leukemia/lymphoma (ATLL)

*Sezary syndrome

  • Mature clonal disorders

*involved blood and bone marrow

*may infiltrate other organs ie.e lymph nodes and spleen

  • Usually affect older adults
  • Indolent, gnerally better prognosis
19
Q

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

A
  • Most common leukemia of adults
  • May transform to diffuse large B-cel lymphoma (Richter transformation)
  • Profound lymphocytosis
20
Q

CLL vs. Reactive Lymphocytosis

A
  • Reactive lymphocytosis:

*infectious mononucleosis (EBV)

  • Mature, non-reactive lymphocytosis:

*pertussis

21
Q

Richter Transformation

A
  • Progression of CLL/SLL to diffuse large B-cell lymphoma
22
Q

Hairy Cell Leukemia

A
  • Rare, B-cell leukemia- 2% of all lymphoid neoplasms
  • Middle aged- elderly adults, indolent
  • Tumor cells predominantly in bone marrow and spleen, may present w/ splenomegaly and pancytopenia
  • Neoplastic cells w/ hair-like cytoplasmic projections
  • Tartrate resistant acid phosphatase (TRAP), CD11c, CD25, CD103, and Annexin A1 are positive (specific markers expressed by these cells)
23
Q

T-cell Prolymphocytic Leukemia (T-PLL)

A
  • Rare
  • Average 70 y/o, men>women
  • Presenting sx: splenomegaly, hepatomegaly, lymphadenopathy, skin lesions
  • Prolymphocytes usually >90% of all lymphocytes
  • Lymphocytes express CD3
24
Q

Adult T-cell Leukemia/Lymphoma

A
  • Neoplasm of CD4+ T-cell infected by HTLV-1 (Human T-cell leukemia virus)
  • Endemic in Japan and Caribbean
  • Skin lesions, lymphadenopathy, peripheral blood lymphocytosis, hypercalcemia
  • Clover leaf and flower cells
25
Q

Sezary Syndrome

A
  • Leukemia form of mycosis fungoides, a mature T-cell lymphoma of CD4+ cells that presents in the skin

CMBM Exam 5 (11 decks)

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