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Heme/Lymph > Intro to White Blood Cell Disorders > Flashcards

Intro to White Blood Cell Disorders Flashcards

1
Q

Where are WBCs found?

A
  • Bone marrow
  • Peripheral blood
  • Lymph nodes, thymus, spleen, tonsils, adenoids, Peyer patches
  • Mucosa-associated lymphoid tissue (MALT)
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2
Q

What is cytoses?

What is cytopenia?

A

Increased leukocytes

Decreased leukocytes

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3
Q

What is the normal reference range for WBCs?

A

4000 - 10,000/uL

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4
Q

What is a leukemoid reaction and what cells may be involved?

A

NOT leukemia

A benign, exaggerated response to infection with an absolute leukocyte count > 50,000

May involve neutrophils, lymphocytes or eosinophils

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5
Q

What are potential causes of a leukemoid reaction?

A
  • Perforating appendicitis - neutrophils
  • Whooping cough (Bordetella pertussis) - lymphocytes
  • Cutaneous larva migrans - Eosinophils
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6
Q

What is a leukoerythroblastic reaction?

What can cause it?

A

Immature bone marrow cells in the peripheral blood

Can be due to a BM infiltrative disease (fibrosis or breast cancer metastasis) or severe BM stress (sepsis or growth factor)

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7
Q

Neutrophilia is defined as an absolute neutrophil greater than ________

A

7,000/uL

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8
Q

What are some causes of neutrophilia?

A
  • Infection
  • Sterile inflammation with necrosis (acute MI)
  • Drugs (steroids, catecholamines, lithium)
  • Increased production and decreased margination (extravasation)
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9
Q

Neutropenia is an absolute neutrophil count less than ______

A

1500/uL

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10
Q

What are some possible causes of neutropenia?

A
  • Chemotherapy
  • Aplastic anemia
  • Immune destruction
  • Septic shock
  • Decreased production and/or increased destruction or margination
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11
Q

Eosinophilia is an absolute eosinophil count greater than _____ and is caused by…(4)

A

700/uL

  • Type I hypersensitivity (allergies)
  • Invasive helminths (*hookworm) *
  • Hypocortisolism (Addison’s disease)
  • Neoplasms (Hodgkin lymphoma)
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12
Q

Basophilia is a basophil count greater than ____ and can be caused by… (2)

A

200/uL

  • **Chronic myelogenous leukemia **(and other chronic myeloproliferative neoplasms)
  • Chronic kidney disease
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13
Q

What do leukemia and lymphoma have in common?

What is the difference?

A

Both are a proliferation of neoplastic cells

Leukemia: Primarily in BM and PB (peripheral blood)

Lymphoma: Primarily in lymph nodes and extramedullary lymphoid tissue

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14
Q

What four criteria are used to classify Myeloid neoplasms (neoplastic stem cell disorders)

A
  • Morphology
  • Immunophenotype
  • Genetic features
  • Clinical features
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15
Q

What are the 3 categories of myeloid neoplasms?

A
  1. Myeloproliferative neoplasms (MPN)
  2. Myelodysplastic syndromes (MDS)
  3. Acute myeloid leukemia (AML)
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16
Q

What are four types of myeloproliferative neoplasms?

A
  1. Chronic myelogenous leukemia, BCR-ABL positive (CML)
  2. Polycythemia vera (PV)
  3. Primary myelofibrosis (PMF)
  4. Essential thrombocythemia (ET)
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17
Q

MPN is a proliferation of…

A

One or more of the myeloid lineages

  • Granulocytic
  • Erythroid
  • Megakaryocytic
  • Mast cells
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18
Q

MPN general features

  • Age group:
  • BM appearance:
  • Effect on other organs:
  • Possible complications:
A
  • Age group: Common in adults (5th - 7th decade)
  • BM appearance: Hypercellular BM with effective hematopoiesis
  • Effect on other organs: Splenomegaly or hepatomegaly
  • Possible complications: Potential for progression - BM fibrosis or acute leukemia
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19
Q

What are the differences between myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS)?

A
  • MPN: Hypercellular BM with effective hematopoiesis (cytoses, increased PB counts, cell proliferation)
  • MDS: Hypercellular BM with ineffective hematopoies-is (cytopenias, decreased PB counts, cell death)
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20
Q

Chronic myelogenous leukemia (CML)

  • Age group:
  • Cellular mechanism:
  • Genetic defect:
  • Clinical findings:
A
  • Age group: peak at 40-60 years
  • Cellular mechanism: Neoplastic expansion of pluripotential stem cell
  • Genetic defect: BCR-ABL fusion gene
  • Clinical findings: Hepatosplenomegaly; weight loss, fatigue, weakness, anorexia
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21
Q

What are the laboratory findings in CML?

A
  • **Leukocytosis **with immature myeloid cells
  • Basophilia
  • Philadelphia chromosome: t(9;22)
  • BCR-ABL** fusion gene**
  • Few myeloblasts
  • Thrombocytosis (45-50%) or thrombocytopenia
  • Hypercellular BM (granulocytic hyperplasia)
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22
Q

What are the 3 stages in the course of CML?

A
  1. Chronic phase (3 years)
  2. Accelerated phase (1 year)
  3. Blast phase = acute leukemia (myeloid or lymphoblastic)
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23
Q

What therapy is done for CML?

A
  • Allogeneic stem cell transplant
  • BCR-ABL tyrosine inase inhibitors
    • Gleevec
    • Dasatinib
    • Nilotinib
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24
Q

Polycythemia vera (PV)

  • Increase in…
  • Genetic defect:
A
  • Increase in **RBCs, **granulocytes and platelets
  • Genetic defect: Janus 2 kinase (JAK2) mutation in virtually all cases
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25
Q

Clinical findings in PV

A
  • Splenomegaly
  • Thrombotic events (due to hyper viscosity)
  • Gout (increased cell breakdown)
  • Increased histamine (from mast cell sin the skin)
    • Ruddy face, pruritus after bathing, peptic ulcer
26
Q

What are the laboratory findings in PV?

A
  • **Increased RBC mass **(increased hemoglobin)
  • Decreased EPO (dysfunctional Hb feedback loop)
  • Leukocytosis
  • Thrombocytosis
  • Normal oxygen saturation
  • Hypercellular BM with fibrosis (in later stages)
27
Q

EPO increases in all polycythemias EXCEPT…

A

Polycythemia vera

28
Q

With conservative treatment of PV, median survival is greater than ___

Most patients die of _____ or ______

A

Median survival is greater than 10 years

Most patients die of thrombosis or hemmorhage

29
Q

Primary myelofibrosis (PMF) involves rapid development of __ _______ and _______ _______ in the spleen, liver and lymph nodes

A

BM fibrosis; Extramedulary hematopoiesis (EMH)

30
Q

What are the clinical findings in PMF? (2)

A
  • Splenomegaly (with portal hypertension)
  • Splenic infarcts with left sided pleural effusions
31
Q

Laboratory findings in PMF?

A
  • BM fibrosis and clusters of atypical megakaryotcytes
  • **JAK2 (or MPL) **mutation in 50%
  • Peripheral blood leukocytosis (early)
  • Normochromic, normocytic anemia
    • Teardrop cells
    • Leukoerythroblastic reaction
32
Q

What is the median survival of PMF?

A

3-7 years in fibrotic stage

>10 years in early (prefibrotic) stage

33
Q

What are the major causes of morbidity and mortality in PMF?

A
  • BM failure
  • Thromboembolic events
  • Portal hypertension
  • Cardiac failure
  • AML
34
Q

Essential thrombocythemia involves a proliferation of…

A

Megakaryocytes

35
Q

What cellular abnormalities are associated with Essential Thrombocythemia (ET)?

A
  • Platelet count > 450,000/uL
  • Mild neutrophilic leukocytosis
  • Hypercellular BM with abnormal megakaryocytes
36
Q

General ET characteristics

  • Clinical findings:
  • Genetic mutation:
  • Median survival:
  • Treatment:
A
  • Clinical findings: Bleeding, splenomegaly
  • Genetic mutation: JAK2 mutation (50%)
  • Median survival: 12-15 years
  • Treatment: Alkylating agents, lower platelet count
37
Q

Myelodysplastic syndromes are characterized by… (3)

A
  • Cytopenias (enhanced degree of apoptosis)
  • Dysplasia (one or more myeloid cell lineages)
  • Ineffective hematopoiesis (myeloblasts < 20%)
38
Q

What are some laboratory findings in MDS?

A
  • Cytopenias
  • Leukoerythroblastic reaction
  • Dysplastic features
  • Hypercellular BM
  • Ring sideroblasts
  • Increased myeloblasts
39
Q

What chromosomal abnormalities are associated with MDS?

A

Chromosomal abnormalities in 50%

del5q, +8, or del7q

40
Q

MDS clinical course

  • Age group:
  • Clinical signs:
  • Median survival:
  • Progression to ___ in 30% of cases
A
  • Age group: elderly (50-80 years)
  • Clinical signs: weakness, infections, hemmorhage
  • Median survival: 9-29 months (t-MDS: 4-8 months)
  • Progression to AML in 30% of cases
41
Q

What is the treament for MDS?

A
  • Supportive: blood products, antibiotics, growth factors
  • Hypomethylating agents: not curative
    • Decitabine
    • Azacitidine
  • Allogenetic stem cell transplant
42
Q

What is the definition of Acute leukemia and what are the two main categories?

A

Neoplastic proliferation of immature cells (blasts) recapitulatin progenitor cells of the hematopoietic system

  • Myeloblastic (myeloid) - AML
  • Lymphoblastic (lymphoid) - ALL
43
Q

Compare acute vs. chronic leukemia

A
  • Acute leukemia
    • Immature cells
    • Untreated natural history of weeks to months
  • Chronic leukemia
    • Mature cells
    • Untreated natural history of months to years
44
Q
A
45
Q

What are some differences between AML and ALL in terms of age group, male/female ratio and incidence?

A
  • AML
    • Primarily **Adults **(Median age = 60)
    • M:F = 1:1
    • 3 cases/100,000 pop./year
  • ALL
    • Most common in **Children **(75% under age 18)
    • M:F = 1.4:1
    • 1.4 cases/100,000 pop./year
46
Q

What are some cytologic differences in Blasts in AML and ALL?

A

AML has larger cells, finely dispersed chromatin, granular cytoplasm and auer rods

ALL has smaller (variable) cells, coarse chromatin, no granules and no auer rods

47
Q

What are the most useful cytochemical stains in identifying AML and ALL?

A

Myeloperoxidase (MPO): myeloblasts

Non-specific esterase (NSE): monocytic blasts in AMLs with monocytic differentiation

48
Q

What is the purpose of immunophenotyping in acute leukemia?

A
  • Distinguishes ALL from AML
  • Distinguishes B-ALL from T-ALL
  • Identifies subtypes
  • Identifies treatment and prognostic group
  • Fingerprint for minimal residual disease assessment
49
Q

Sorry about this slide but…

What are some examples of lymphoid antigens in…

T lineage:

B lineage:

AND what are some examples of myeloid antigens

Generic:

Sub lineage associated:

A
  • T lineage: CD1a, CD2, CD3, CD4, CD5, CD7, CD8
  • B lineage: CD19, CD20, CD22
  • Generic:** CD13, CD15, CD33, CD117, MPO**
  • Sub lineage associated: CD41 and CD61 (megakaryocytes); CD14 (monocytes)
50
Q

What are markers of immaturity in immunophenotyping for acute leukemia?

A
  • CD34 (AML and ALL)
  • TdT (mostly seen in ALL)
  • CD117 (AML)
  • CD1a (restricted to immature T cells)
51
Q

What is the prognosis in AML?

What is the cellular neoplasm in AML?

A
  • Poor outcome: long term survival = 25%
  • Systemic neoplasms of myeloid progenitor cells
    • Primarily involves blood and bone marrow
52
Q

Clinical features of AML?

A
  • Related to cytopenias - Weakness, fatigue, infections
  • Less common findings - organomegaly, lymphadenopathy
  • Coagulopathy in specific variants
53
Q

What is the diagnostic criterion for AML?

A

Greater than 20% myeloid blasts in blood or marrow

Maturation may be towards any one of the myeloid lineages (**Granulocytes, monocytes, **megakaryocytes, erythroid precursors)

54
Q

What are the hematologic features of AML?

A
  • Severe leukopenia to marked leukocytosis
  • Anemia and thrombocytopenia are the norm
  • Maturing/mature cells of all myeloid lineages may be dysplastic
55
Q

What are the different types of AML?

A
  • AML with recurrent cytogenetic abnormalities
  • AML with myelodysplasia-associated changes
  • AML and MDS, therapy related
  • AMLy not otherwise categorized
56
Q

AML with recurrent cytogenetic abnormalities

  • Incidence rate by age group:
  • Prognosis:
  • Antecedent myelodysplastic syndrome?:
A
  • Incidence rate by age group: flat incidence rate over different age groups
  • Prognosis: generally favorable
  • Antecedent myelodysplastic syndrome?: no
57
Q

AML with myelodysplasia-associated changes

  • Incidence rate by age group:
  • Prognosis:
  • Antecedent myelodysplastic syndrome?:
A
  • Incidence rate by age group: Increasing incidence with age
  • Prognosis: Poor
  • Antecedent myelodysplastic syndrome?: likely
58
Q

What genetic defects in AML are associated with favorable outcomes? unfavorable?

A
  • Favorable
    • t(8;21): AML1/ETO
    • inv(16): CBFß/MYH11
    • t(15;17): PML/RAR alpha
  • Unfavorable
    • 11q23 (MLL) rearrangements
59
Q

Describe AML with t(15;17)

A
  • Acute promyelocytic leukemia
  • Single cells with multiple auer rods (faggot cells)
  • Responds to all-trans retinoic acid (ATRA) - unique to this variant
  • Usually leukopenic
60
Q

Histiocytic conditions: Langerhans cell histiocytosis

  • Mutations:
  • Appearance on EM:
  • Cluster of differentiation:
A
  • Mutations: BRAF mutations
  • Appearance on EM: “tennis racket” - Birbeck granules
  • Cluster of differentiation: CD1a, langerin
61
Q

Histiocytic conditions: Hemophagocytic lymphohistiocytosis

Primary (defect):
Secondary HLH:
Distinguishing feature:

A
  • Primary (defect): defects in perforin gene
  • Secondary HLH: EBV, lymphomas
  • Distinguishing feature: very high ferritin

Heme/Lymph (18 decks)

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