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Heme/Lymph > Intro to WBC disorders > Flashcards

Intro to WBC disorders Flashcards

1
Q

Monocytes, neutrophils, eosinophils, basophils all come from what type of blast?

A

Myeloblast

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2
Q

What three cell lines result from myeloid blasts?

A

Eyrthroid precursor, megakaryocyte, myeloblast

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3
Q

What areas are white blood cells distributed in?

A

Bone Marrow, Peripheral blood (granulocytes/monocytes/lymphocytes)

Lymph nodes, thymus, spleen, tonsils, adenoids, Peyer Patches

MALT–in lung and GI tract

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4
Q

Benign Leukocyte disorders are NOT neoplastic thus not:

A

Clonal

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5
Q

In benign leukocyte disorders, we see Qualitative and Quantitative disorders…

What types of quantitative disorders do we see?

A

Increased: cytoses

Decrease: cytopenias

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6
Q

Normal range for WBCs

A

4,000 to 10,000

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7
Q

Reference range for :

Neutrophils

Lymphocytes

Monocytes

A

 Neutrophils = 1,500 – 6,000
 Lymphocytes = 1,000 – 3,500
 Monocytes = 100 – 600

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8
Q

Reference range for:

Eosinophils

Basophils

A

 Eosinophils = 0 – 450
 Basophils = 0 – 200

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9
Q

 NOT leukemia (benign, exaggeratedresponse to infection)
 Absolute leukocyte count > 50,000/mL
 May involve neutrophils, lymphocytes oreosinophils

A

Leukemoid reaction

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10
Q

Three causes of leukemoid reactions?

A

Perforating appendicitis (neutrophils)

Whooping cough (lymphocytes)

Cutaneous larva migrans (eosinophils)

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11
Q

Describe leukoerythroblastic reaction

A

immature bone marrow cells in the PB d/t BM infiltrative disease (such as fibrosis or metestatic breast cancer) or from severe BM stress (such as sepsis or Growth factor)

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12
Q

What defines neutrophilia and when do we see it?

A

absolute neutrophil count > 7,000

seen in sterile inflammation with necrosis (acute MI)

Infection (like acute appendicitis)

Drugs (steroids or catecholamines, lithium)

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13
Q

What is the pathogeneis of neutrophilia?

A

Increased production and decreased margination

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14
Q

Define Neutropenia:

Etiology:

Pathogenesis

A

Absolute neutrophil count: <1,500

Etiology of neutropenia: chemo, aplastic anemia, immune destruction, septic shock

Pathogeneis of neutrophenia: decreased production, increased destruction/margination

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15
Q

Describe Eosinophilia:

Etiotology

A

Absoulte eosino count >700

 Type I hypersensitivity (e.g., bronchialasthma, penicillin allergy, hay fever)
 Invasive helminths (e.g., strongiloidiasis, hookworm)
 Hypocortisolism (e.g., Addison’s disease)
 Neoplasms (e.g., Hodgkin lymphoma)

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16
Q

Pathogenesis of eosinophilia

A

Increased produciton (induced by interleukins)

Increased tissue rectruitement by chemotactic factors

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17
Q

Describe Basophilia:

Etiology of Basophilia:

A

absoulte basophil count of >200

 Chronic myelogenous leukemia or CML (and otherchronic myeloproliferative neoplasms)
 Chronic kidney disease

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18
Q

 Proliferation of neoplastic cells, primarily in BM and PB

A

Leukemia

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19
Q

Proliferation of neoplastic cells, primarily in LNs and extramedullary lymphoid tissue

A

Lymphoma

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20
Q
A
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21
Q
A
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22
Q

Myeloid neoplasms are neoplastic _______ disorders

A

stem cell

**can involve more then one cell lineage

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23
Q

What are the 4 key WHO classification criteria in Myeloid neoplasms?

A

Morphology

Immunophenotype

Genetic Features

Clinical features

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24
Q

What are the three types of myeloid neoplasms?

A

 Myeloproliferative neoplasms (MPN)
 Myelodysplastic syndromes (MDS)
 Acute myeloid leukemia (AML)

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25
Q

What are the four myeloproliferative disorders we need to know?

A

Chronic myelogenous leukemia: BCR ABLpositive (CML)

Polycythemia vera (PV)

Primary myelofibrosis (PMF)

Essential thrombocythemia (ET)

26
Q

Myeloproliferative neoplasms, General features:

______hematopoietic stem cell disorders

Proliferation of one or more of the follwing myeloid lineages:

A

Clonal hematopoeitic stem cell disorders

Lineages: Granulocytic/ Erythroid/ Megakaryocytic/ Mast cell

27
Q

MPN is common in what decade?

How common?

A

MPN seen in 5th - 7th decade

Incidence: 6-10/100,000/year

28
Q

MPN is:

Hyper or Hypocellular BM

with

effective or ineffective hematopoesis

A

Hypercellular BM with effective
hematopoiesis (increased PB counts =
cytoses) <= clonal abnormalities
increase cell proliferation

29
Q

MDS is:

Hyper or Hypocellular BM

with

effective or inneffective hematopoesis

A

Hypercellular BM with ineffective
hematopoiesis (decreased PB counts =
cytopenias) <= clonal abnormalities
promote cell death

30
Q

Chronic Myelogenous Leukemia (type of myloproliferative disease)

Epidemiology:
Pathogenesis:

A

peak at 40-60 years

Pathogenesis:

  1. Neoplastic expansion of the pluripotentialstem cell
  2. BCR-ABL fusion gene (produces protein with tyrosine kinase activity)
31
Q

What 5 key clincal findings do we see in CML?

A

Hepatosplenomegaly, fatigue, weight loss, weakness, anorexia

32
Q

Lab findings show Leukocytosis with immature myeloid cells, few myeloblasts (2-3%) and basophilia… what would we expect to find on FISH or RT-PCR?

A

BCR-ABL fusion gene~~ common in CML

33
Q

expected lab findings for CML

A

Leukocytosis with immature myeloid cells
 Few myeloblasts (2-3%)
 Basophilia
 Thrombocytosis (40-50% cases) or thrombocytopenia
 Hypercellular BM (~100%) with granulocytic hyperplasia

34
Q

Chromosome and gene responsible for CML

A

Philadeplphia Chromosome: t(9;22)

BCR-ABL fusion gene (FISH or RT-PCR)

35
Q

What should a normal bone marrow core biopsy look like?

A

100-age rule

ex: if you are 30 yo you should have 70% cellularity and there should be adipocytes in there

36
Q

What are the three stages seen in CML if left untreated?

A

Chronic phase~ 3 years

Accelerated phase~ 1 year

Blast phase = acute leukemia (meloid or lymphoblastic)

*all end up in blast phase if left untreated

37
Q

What is the ideal type of treatement for CML?

A

BCR-ABL tyrosine kinase inhibitors

  • Gleevec
  • Dasatinib
  • Nilotinib

*can’t cure CML but staying on this therapy you can see 95% event free survival

38
Q

Pathogenesis:
 Neoplastic expansion of the pluripotential
stem cell
 Increase in RBCs, granulocytes and
platelets
 Janus 2 kinase gene (JAK2) mutation in
virtually all cases

A

Polcythemia Vera

39
Q

We see expansion of ___, _____ and _____ in polycythemia Vera

A

increase in RBC, granulocytic, and platelets

40
Q

What mutation is present in virtually all cases of polycythemia vera?

A

Janus 2 kinase gene (JAK2)

–constitutional activation of JAK/STAT will cause activation of genes important in proliferation and survival~ in this case constant proliferation of red cells

41
Q

PV – Clinical findings

A

 Splenomegaly
 Thrombotic events due to hyperviscosity (e.g. hepatic vein thrombosis)
 Gout (increased uric acid due toincreased cell breakdown)
 Signs of increased histamine (releasedfrom mast cells in the skin)
 Ruddy face
 Pruritus after bathing
 Peptic ulcer disease

42
Q

Your patient comes in with splenomegaly, is itchy after they bathe and was recently diagnosed with peptic ulcer disease. The Lab shows they ahve increased RBC mass. What would you suspect of their EPO and SaO2 levels

A

This is describing polycythemia vera

EPO is decreased

Normal oxygen saturation

43
Q

In most polycythemias, there is an increase in EPO, why is polycythemia vera different?

A

In the other polycythemias, the increased EPO is driving the overproduciton of RBCs

In PV, it is the JAK/STAT2 mutation that is upregulating the transcription of RBCs, NOT the EPO

44
Q

What are the major criteria for polycythemia vera?

A

Hemoglobin > 18.5 g/dL in men, 16.5 g/dL in
women or other evidence of increased RBC volume
Presence of JAK2 mutation

45
Q
A
46
Q

PV – Clinical course
 Conservative treatment (e.g., phlebotomy); median survival of:
 Most patients die of:

A

> 10 years

thrombosis or hemorrhage

47
Q

In PV, 15-20% of pts evolve to what phase?

2-3% of PV pts develope what diseases?

A

evolved to ‘spent phase’

devo MDS or AML

48
Q

Rapid development of BM fibrosis and extramedulary hematopoiesis (EMH) in the spleen, liver and lymph nodes

A

Primary myelofibrosis (PMF)

~ type of myeloproliferative neoplasm

49
Q

What are the clinical findings seen in: Primary myelofibrosis (PMF)

A

Splenomegaly with portal hypertension
Splenic infarcts with left-sided pleural effusions

50
Q

What mutation is found 50% of the time in PMF?

A

JAK2 seen in 50% cases

51
Q

What do we see on a blood smear in a patient with Primary myelofibrosis?

A

BM fibrosis and clusters of atypical megakaryocytes
 Peripheral blood leukocytosis (early stage)
 Variable platelet count
 Normochromic, normocytic anemia
*Teardrop cells
*Leukoerythroblastic reaction

52
Q
A
53
Q

Median Survival for PMF:

A

3-7 years in fibrotic stage

>10 years in early (prefibrotic) stage

54
Q

5-30% of patients with PMF will develop:

A

AML

55
Q

Major causes of morbidity and mortality in PMF:

A

 BM failure (infection, hemorrhage)
 Thromboembolic events
 Portal hypertension
 Cardiac failure
 AML

56
Q

Essential thrombocythemia (ET):

Neoplastic stem cell disorder with proliferation of__________

A

megakaryocytes

57
Q

Platelet count in Essential throbmocythemia is:

A

> 450,000/mL

58
Q

In this disease we see atypical platelet morphology

Mild neutrophilic leukocytosis

Hypercellular BM with abnormal megakaryocytes

A

Essential Thrombocytopenia

59
Q

Describe what you see in Essential Thrombocythemia on HE

A

more platelets that are larger in size (they shouldn’t be larger then RBCs)

See large/giant, hypogranular platelets

60
Q

Clinical findings of essential thrombocythemia

A

bleeding or throbmosis

splenomegaly

Jak2 mutation in 50% pts

61
Q

Common mutation in ET pts?

Median survival:

Tx for ET

A

JAK2 in 50% pts

median survival: 12-15 years

Tx: alkylating agents or simular drugs to lower platelet count

Heme/Lymph (10 decks)

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