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RHS > L26- WBC Pathology IV > Flashcards

L26- WBC Pathology IV Flashcards

1
Q

lymphoid neoplasms include (1)

myeloid neoplasms include (2)

A

1:

  • Non-Hodgkin Lymphoma / Leukemia (including plasma cell neoplasms)
  • Hodgkin Lymphoma

2:

  • acute myeloid leukemia (AML)
  • chronic myeloproliferative neoplasms (MPN)
  • myelodysplastic syndrome (MDS)
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2
Q

Myeloid neoplasms arise from (1) cells in the (2) primarily. (3) and (4) can sometimes also be affected secondarily. (5)- list the categories of myeloid neoplasms.

A

1- myeloid hematopoietic progenitors cells (erythroid, granulocytic, megakaryocytic lineages)
2- bone marrow
3- peripheral blood
4- hematopoietic organs: spleen, liver, LNs

5- AML, MPN, MDS

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3
Q

AML most affects (1) age group and usually block (2) development. (3) will then accumulate in bone marrow and peripheral blood.

A

1- adults, inc incidence with inc age; median age = 50 y/o
2- early stage of myeloid development
3- blasts / immature myeloid cells

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4
Q

describe the forms of AML (by WHO) in terms of molecular features and outcome

A

(formerly M0-M7)
-AML w/ recurrent chromosomal translocations

  • AML w/ multilineage dysplasia
  • AML therapy related
  • AML not otherwise specified
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5
Q

give the name for AML type M0-M7 (former WHO classification)

A 
(degree of maturation in granulocytic lineages)
M0- minimally differentiated leukemia
*M1- AML w/o maturation
**M2- AML w/ some maturation
M3- acute promyelocytic leukemia
(presence of additional lineages of blast cells)
*M4- acute myelomonocytic leukemia
M5- acute monocytic leukemia
M6- acute erythroleukemia
M7- acute megakaryocytic leukemia
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6
Q

list the technique used to distinguish between Myeloblasts

A

(many AML type –> many myeloblasts)

  • morphology
  • special histochemical stains (traditional)
  • immunophenotyping (flow cytometry, IHC)
  • cytogenetics (occasionally very useful)
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7
Q

AML pathogenesis:

  • (1) will disrupt genes encoding for (2)
  • mutations will produce (3) genes and (4) proteins leading to (5)
  • additionally (6) is also present to stimulate excess proliferation
  • presence of (7) will cause (8)
A

1- translocations
2- Transcription Factors for normal differentiation
3- chimeric genes
4- abnormal fusion proteins
5- blocks terminal differentiation
6- TK mutations
7- accumulation of proliferating neoplastic precursors
8- suppress normal hematopoietic progenitors

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8
Q

AML has a (slow/rapid) onset (include time frame). (2) is the most apparent symptom. Infiltration of (3) is often popular, and infiltration forming (4) is uncommon. (5) is a dangerous complication caused by (6) mutation.

A 
1- rapid, wks-mos
2- cytopenias: either anemia, leukopenia / neutropenia, thrombocytopenia OR pancytopenia
3- mucosa, skin, soft tissue
4- extramedullary masses
5- DIC
6- t(15;17)
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9
Q

DIC can be seen in type (1) AML because of (2)

A

1- M3, acute promyelocytic leukemia

2- t(15;17)

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10
Q

AML-M3 = (1):

  • (2) and (3) are present within neoplastic promyelocytes
  • release of (4) from (3) can cause (5) due to (6) genetically
A 
1- acute promyelocytic leukemia
2- Auer Rods
3- course azurophilic granules
4- myeloperoxidase
5- DIC
6- t(15;17)
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11
Q

AML-M5 = (1):

  • (2) is difficult to determine on peripheral blood smear (include features)
  • (3) is the most useful in diagnosis, particularly (4)
A

1- acute monocytic leukemia
2- monocyte differentiation: presence of folded nuclear membrane in monoblast
3- skin, mucosal deposition (b/c resident macrophages go to these places)
4- gingival hyperplasia

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12
Q

AML-M1 = (1):

-(2) are present in myeloblasts on peripheral blood smear

A

1- acute myeloid leukemia w/o maturation

2- azurophilic granules in cytoplasm

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13
Q

list the techniques for diagnosis of AML, indicate the gold standard

A
  • CBC: high or low WBC count
  • Peripheral Blood: circulating blast cells
  • ***BM aspirate / biopsy: >20% blasts
  • Flow Cytometry (surface markers)
  • Cytochemical staining: M1-M3 are MPO, sudan black positive, M4-M5 are estarase positive
  • Cytogenetics
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14
Q

(1) is the gold standard for diagnosis of AML, where (2) must be present for a positive diagnosis

A

1- bone marrow aspirate / biopsy

2- >20% blast cells

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15
Q

AML Flow Cytometry:

  • CD(1) for myeloid stem cells
  • CD(2) marker of immature blast cells
  • CD(3) marker for subset of more mature lineages of AMLs
A

1- CD34
2- CD33
3- CD15

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16
Q

AML cytogenetics:

  • (1) are the common translocations. The more translocations indicates a (2) prognosis.
  • (3) of chromosome (4) are common in (5) patients
A

1- (younger Pts) t(15;17), t(18;21), t(16;16)
2- better prognosis (unless involving chr.11)

3- deletion / monosomy
4- chr.5/7
5- adults with AML, MDS, or post-chemo/radiotherapy

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17
Q

list the diagnostic methods for AML that help distinguish it from ALL (hint- 4)

A

Morphology: granules, Auer rods (AML)

Histochemical Stains:

  • AML: MPO, sudan black, esterase stains
  • ALL: PAS in some

Immunophenotyping: used to differentiate myeloid from lymphoid markers

**Cytogenetics

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18
Q

AML treatment:
-most are treated with (1)

  • M3 / AML with (2) is treated with (3) to induce (4) and eventually relapse
  • (5) is a possible Tx for high risk AML or relapsed AML
A

1- combination chemotherapy

2- AML w/ t(15;17) [chimeric RARα-PML blocks differentiation]
3- ATRA (all trans retinoic acid) / vitA derivative
4- differentiation into neutrophil
(note- chemotherapy must also be done after ATRA Tx)

5- bone marrow transplant (AML still may recur)

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19
Q

AML prognosis, is indicated by its (1):

-(2) is good prognosis, (3) has intermediate prognosis, (4) has poor prognosis

A

1- cytogenetics
2- t(8;21), inv16
3- t(15;17)
4- del. chr.5/7

20
Q

AML Tx, (1) achieve remission, but (2)% of those will relapse within 5 years

A

1- >60%

2- >50%

21
Q

list the myeloproliferative neoplasms

A
  • CML, chronic myeloid leukemia
  • PV, polycythemia vera
  • ET, essential thrombocytopenia
  • PMF, primary myelofibrosis
22
Q

General features of myeloproliferative neoplasms:

  • (1) growth is evident as it fills up (2) and prevents (3)
  • because of (3), (4) will occur causing (5)
A

1- neoplastic clone growth reaching terminal differentiation (inc / dysregulated growth)
2- bone marrow
3- (suppresses) normal hematopoiesis
4- seeding to secondary hematopoietic organs: spleen, liver
5- organomegaly

23
Q

Myeloproliferative neoplasms:

  • ‘spent phase’ causes (1) in (2) types
  • ‘blast crisis’ is the other complication seen in (3) type, converting it to (4)
A

1- BM fibrosis + cytopenias
2- PV, ET, PMF

3- CML
4- acute leukemia

24
Q

Myeloproliferative neoplasms:

most simply explained by the abundance of (1) and the absence of (2) seen in other leukemias

A

1- one of the WBCs, RBCs, or megakaryocytes

2- blast cells (no arrest or accumulation)

25
Q

Myeloproliferative neoplasms mostly involve a mutated (1) enzyme causing (constitutional/defective) activity. Although (3) is circumvented, (4) is importantly not impaired.

A

1- TK
2- constitutional activity
3- normal growth control (via GFs) => GF-indep. prolif. + survival of marrow precursors
4- differentiation

26
Q

list the common genetic TK mutation for the following Myeloproliferative neoplasms:

(1) CML
(2) PV
(3) ET
(4) PM

A

1- t(9;22) / Philadelphia chr. (100%) [inc ABL activity]
2- JAK2 point mutation (95%)
3- JAK2 pt mut. (50-60%), MPL pt. mut.(5-10%)
4- JAK2 pt mut. (50-60%), MPL pt. mut.(5-10%)

27
Q

CML is related to the uncontrolled production of (1) due to a defect in (2). (1) will appear like (3) cells, but without (4).

A

1- mature granulocytes with normal differentiation
2- pluripotent stem cell for myeloid / lymphoid lineages (morphologically only affect granulocytes)

3- neutrophils (in abundance)
4- no functionality

28
Q

CML is associated with (1) genetic mutation in which (2) process results.

A

1- t(9;22) / Philadelphia chromosome

2- BCR gene on chr.22 is fused with ABL1 gene on chr.9 –> constitutional expression of fused protein => kinase activity and unchecked proliferation (via RAS, STAT, AKT)

29
Q

CML clinical presentation:

  • affects (1- age, sex) populations most
  • presents with gradual onset of (2) sxs
  • (3) may be present if (4) organ is involved
  • (5) is used to differentiate CML from leukemoid reaction (via severe bacterial infection)
A 
1- adults (25-60 y/o), M>F
2- fatigue, weakness, loss of weight and appetite (due to suppression of normal BM)
3- abdominal discomfort
4- spleen / splenomegaly
5- peripheral blood smear
30
Q

CML labs:

  • (1) appearance on PB
  • (2) appearance on BM
  • (3) cytogenetic results
A

1- ‘left-shift’ (inc young cells) leukocytosis of mostly neutrophils and precursors + inc eosinophils, basophils /// <10% blast, possible thrombocytosis

2- 100% cellular almost (hypercellularity), mainly inc granulocytic precursors + megakaryocytes (no inc in blast%)

3- t(9;22) via PB/BM

31
Q

describe the course / progression of CML

A

(triphasic)
1) Stable phase (chronic phase): 2-5 yrs w/o effective Tx

2) Accelerated phase: after 2-5 yrs 50% of Pts progress here –> inc blast%, BM fibrosis, thrombocytopenia, extra cytogenetic abnormalities
3) Blast crisis: progression to acute leukemia w/in 1 yr; 75% myeloid, 25% lymphoid

32
Q

CML Tx: list the 4 therapies

A

1) Imatinib: TK inhibitor (BCR-ABL kinase) /// slows disease progression, but doesn’t destroy abnormal clone
2) IFN-α: slows disease progression
3) Hydroxyurea: ‘gentle’ chemotherapy
4) allogenic BM transplant: younger Pts, 75% cure rate

33
Q

describe the classification of Polycytothemia

A

Reactive: due to reduced plasma volume (secondary to dehydration)

Absolute:

1) primary = polycytothemia vera (PV), associated with splenomegaly and low EPO
2) secondary: appropriately (lung disease, cyanotic HD, high altitude) OR inappropriately high EPO (EPO tumors: renal, cerebellar, hepatic tumors)

34
Q

PV = (1):

  • characterized by (2) and (3) levels in CBC
  • most patients present with (4)
A

1- polycythemia vera
2- abnormal RBC count elevation
3- abnormal elevated Hb levels
4- asymptomatic

35
Q

In advanced or symptomatic PV, list the symptoms and complications that may develop

A
  • hyperviscosity Sxs: HA, dizziness, visual disturbances
  • pruritus, erythromelalgia
  • early satiety due to splenomegaly

-Polycythemia complications: thrombosis or bleeding

36
Q

list the WHO criteria for PV diagnosis

A

Major criteria:

1) Hb >16.5 g/dL in men, >16 g/dL in women OR >48-49% Hc OR inc RBC mass
2) BM biopsy with hypercellularity, prominent proliferation of all cells
3) JAK2 mutation (98% cases)

Minor criteria:
-low EPO levels (subnormal serum EPO)

Need all 3 majors OR 2 major + minor

37
Q

Primary Myelofibrosis is a neoplasm of (1) cells. The release of (2) CKs will cause the characteristic (3) in BM.

A

1- megakaryocytes
2- PDGF, TGF-β
3- obliterative bone marrow fibrosis

38
Q

Primary Myelofibrosis:

  • (1) common genetic mutations
  • (2) age group is most affected
  • patient commonly present with (3) and (4)
  • complications include (5)
A

1- JAK2 (–> activates JAK-STAT)
2- >60 y/o
3- progressive anemia (obliterative BM fibrosis)
4- hepatosplenomegaly (extramedullary hematopoiesis)
5- infections, bleeding disorder, transformation into AML

39
Q

Primary Myelofibrosis Dx:

  • (1) on PB
  • (2) on BM
  • (3) spleen appearance
A

1- leukoerythroblastosis (extramedullary hematopoiesis) + immature appearing RBCs/WBCs (RBCs with tear drop appearance / poikilocytes)

2- hypocellular, diffuse fibrosis w/ entrapped megakaryocytes

3- massive (splenomegaly), subcapsular infarcts

40
Q

ET = (1):

  • (2) are the common mutations
  • (3) is the characteristic feature or definition
A

1- essential thrombocytopenia
2- JAK2, MPL mutations
3- inc / abundance of abnormal platelets (neoplastic megakaryocytes)

41
Q

ET = (1):

  • mostly affects (2) age group
  • (slow/rapid) onset
  • occasionally (4) is a major complication
A

1- essential thrombocytopenia
2- >60 y/o
3- slow, indolent growth
4- thrombosis: DVT, portal vein / hepatic vein thrombosis, erythromelalgia (hand/foot burning due to arteriolar occluasion)

42
Q

ET appearance on:

  • (1) PB
  • (2) BM
A

1- abnormally large platelets

2- inc megakaryocytes w/o fibrosis (vs PMF)

43
Q

MDS = (1):

  • (2) is definition
  • (3) is the main cause affecting (4) age group with (slow/rapid) onset
  • (6) is the other cause
A 
1- myelodysplastic syndrome
2- clonal maturation defects in stem cells --> ineffective hematopoiesis / abnormal differentiation --> cytopenia
3- idiopathic/primary
4- >50 y/o
5- slow, gradual
6- 2-8 yrs post-chemo/radio-therapy
44
Q

MDS = (1):

  • many cases transform into (2)
  • deaths are related to (3)
  • median survival rate is (4)
A

1- myelodysplastic syndrome
2- AML (10-40% cases)
3- cytopenia complications
4- 9-30 mos (only 2-4 mos in therapy related MDS)

45
Q

MDA appearance on:

  • (1) PB
  • (2) BM
A

1- macrocytic anemia, cytopenia +/- blast cells

2- RBC morphological abnormality +/- granulocytic precursors, +/- megakaryocytes

46
Q

list the diagnostic techniques for MDS

A

1) CBC, PB: various cytopenias (partial or full pancytopenia)
2) BM morphology: hypercellular, disorganized hematopoiesis
3) cytogenetic abnormalities: chr.5/7 deletion, trisomy 8
4) flow cytometry

47
Q

MDS Tx

A
  • allogenic bone marrow transplant (younger patients)

- supportive Tx (older patients)

RHS (35 decks)

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