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M2 Heme/Lymph > White Blood Cell Disorders - Olteanu & Atallah > Flashcards

White Blood Cell Disorders - Olteanu & Atallah Flashcards

1
Q

What is a leukemoid reaction?

A

A benign, exaggerated response to infection with leukecyte counts greater than 50,000/uL

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2
Q

What are some causes of leukemoid reactions? What types of cells are seen in these etiologies?

A

perforating appendicitis- neutrophils

Whooping cough- lymphocytes

nematode infection- eosinophils

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3
Q

What is leukoerythroblastotic reaction? How is it different from leukemoid reaction?

A

It is an expansion of young red and white blood cells in the peripheral blood This is different from leukemoid reactions because it has a variety of cells, and the cells are not mature.

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4
Q

What can cause leukoerythroblastotic reaction?

A

Crowding of cells out of the marrow by external growth (fibrosis, metastatic cancer)

Overgrowth of cells due to external stress (infection, growth factor abundance)

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5
Q

What is neutrophilia? What are GENERAL causes of it?

A

neutrophil counts over 7000/uL decreased extravasation from blood OR overproduction of neutrophils

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6
Q

What is neutropenia? What are GENERAL causes of it?

A

neutrophil counts under 1500/uL increased extravasation from the blood OR underproduction/ destruction

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7
Q

Primary Myelofibrosis (PMF)

  1. What category of WBC disorder is PMF?
  2. Describe the pathology generally.
A

Primary Myelofibrosis (PMF)

  1. PMF is a myeloproliferative neoplasm
  2. Pathology:
    • Rapid development of BM fibrosis
    • Extramedulary hematopoiesis (EMH) in the spleen, liver, & LNs
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8
Q

Primary Myelofibrosis (PMF)

  • What are clinical findings of PMF?
A

Primary Myelofibrosis (PMF)

  • Splenomegaly
  • Portal hypertension
  • Splenic infarcts
  • Left-sided pleural effusions
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9
Q

Primary Myelofibrosis (PMF)

  1. Mutations in what are associated with PMF?
    • Functionally, what type of mutation?
  2. Knowing this, what type of drug (recently developed!) might be useful in treating PMF?
A

Primary Myelofibrosis (PMF)

  1. JAK2 or mpl
    • JAK2 is part of the signalling cascade for the thrombopoietin receptor
    • mpl is the gene that encodes this receptor
  2. JAK inhibitors
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10
Q

Primary Myelofibrosis (PMF)

  • What laboratory findings are noted in PMF?
A

Primary Myelofibrosis (PMF)

  • BM fibrosis
  • Clusters of atypical megakaryocytes in BM
  • Peripheral blood leukocytosis (early stage)
  • Variable platelet count
  • Normochromic, normocytic anemia
    • Teardrop cells in peripheral blood
    • Leukoerythroblastic reaction
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11
Q

Primary Myelofibrosis (PMF)

  1. What is the median survival for PMF patients?
  2. What are the major causes of morbidity and mortality in PMF patients?
  3. What condition do up to 30% of PMF patients develop?
A

Primary Myelofibrosis (PMF)

  1. Survival:
    • 3-7 years in fibrotic stage
    • >10 years in prefibrotic stage (early)
  2. M&M causes:
    • BM failure (infection, hemorrhage)
    • Thromboembolic events
    • Portal HTN
    • Cardiac failure
    • AML
  3. AML!
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12
Q

Essential Thrombocythemia (ET)

  1. What category of WBC disorder is ET?
  2. Desribe the pathology generally.
  3. Knowing the pathology, what clinical symptoms do you expect to see in ET?
A

Essential Thrombocythemia (ET)

  1. ET is a myeloproliferative neoplasm
  2. Neoplastic stem cell disorder with proliferation of megakaryocytes
  3. Sxs:
    • Bleeding or thrombosis
      • Depending on whether platelets are functional
    • Splenomegaly
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13
Q

Essential Thrombocythemia (ET)

  • Mutations in what are associated with ET? How common is this mutation in ET?
A

Essential Thrombocythemia (ET)

  • JAK2, ~50% of pts
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14
Q

Essential Thrombocythemia (ET)

For a patient with ET, what findings might you expect to find with:

  1. CBC?
  2. Peripheral blood smear?
  3. BM aspirate?
A

Essential Thrombocythemia (ET)

  1. CBC:
    • Platelets > 450,000/µL
    • Mild neutrophilic leukocytosis
  2. PB:
    • Numerous platlets with abnormal morphology
      • large, hypogranular platelets
  3. BM:
    1. Numerous abnormal megakaryocytes
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15
Q

Essential Thrombocythemia (ET)

  1. What is the median survival time for a pt with ET?
  2. How is ET treated?
A

Essential Thrombocythemia (ET)

  1. 12-15 years
  2. Alkylating agents or other drugs that lower platelet count
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16
Q

Myelodysplastic Syndromes

  1. Like myeloproliferative neoplasms, MDS’s result from disorders of which cell type?
  2. Unlike proliferative neoplasms, MDS’s result in what major condition?
    • Why?
A

​Myelodysplastic Syndromes

  • Hematopoietic stem cells
  • Ineffective hematopoiesis & Cytopenias
    • Dysfunctional cells prone to apoptosis
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17
Q

Fill in the blanks: In a myelodysplastic syndrome, you might expect to see up to ___% blasts in the PB or BM. If the percent blasts is above that value, the patient’s condition now meets the diagnostic criteria for __________.

A
  • 20%
  • Acute Myeloid Leukemia
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18
Q

Describe the laboratory findings of a myelodysplastic syndrome.

A
  • Hypercellular BM
  • Cytopenias!
    • uni-, bi, or pancytopenia (more than one cell lineage may be affected)
  • Leukoerythroblastic reaction
    • Similar to a leukomoid reaction, with the addition of nucleated red blood cells
  • Increased myeloblasts & general dysplastic features
  • Ring sideroblasts
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19
Q

What is a ring sideroblast?

A

An abnormal nucleated RBC. Iron staining will show a blue ring of iron granules around the nucleus.

  • Ring does not have to completely encircle the nucleus to be counted as a ring sideroblast
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20
Q

Chromosomal abnormalities account for 50% of MDS cases. Which abnormalities, in **which three chromosomes, **are often responsible?

A
  • Chr 5: monosomy 5 or del5q
  • Chr 7: monosomy 7 of del7q
  • Chr 8: trisomy 8
21
Q

Myelodysplastic Syndromes

  1. In what age group are MDSs seen?
  2. What clinical symtoms do these pts present with?
A

Myelodysplastic Syndromes​

  1. Elderly (50-80 years)
  2. Sxs:
    • Weakness
    • Infections
    • Hemorrhage
    • Can be asymptomatic
22
Q

Myelodysplastic Syndromes​

  1. What is the median survival of MDS?
  2. What is t-MDS? What is its median survival?
  3. What are the major causes of mortality in MDS pts?
A

Myelodysplastic Syndromes​

  1. 9-29 months
  2. Therapy-induced MDS
    • Arises as a complication of cancer treatments due to stress on the BM
    • Median survival: 4-8 months
  3. Infection & bleeding secondary to cytopenias
23
Q

Myelodysplastic Syndromes

  • How is MDS treated?
  • Among the treatments, what special drug class is used?
A

Myelodysplastic Syndromes

  • Supportive
    • blood products
    • antibiotics
    • growth factors
  • Special: Hypomethylating agents (not curative)
    • Decitabine
    • Azacitidine
  • Allogenic stem cell transplant
24
Q

In what age group is AML typically seen?

ALL?

A

AML: Adults

ALL: Children

25
Q

Define an acute leukemia.

How does acute leukemia differ from chronic leukemia?

A

A neoplastic proliferation of immature cells (blasts) that recapitulates progenitor cells of the hematopoietic system.

Acute:

  • Immature cells
  • Untreated natural history of weeks to months

Chronic:

  • Mature cells
  • Untreated natural history of months to years
26
Q

How common is ALL as a cancer among children?

Does ALL show a gender bias?

A

MOST COMMON cancer in children!

M:F = 1.4:1

27
Q

Contrast the following cytologic features of the blasts in AML with those in ALL:

  1. Blast size
  2. Chromatin
  3. Nucleoli
  4. Cytoplasm
  5. Auer rods
  6. Myelodysplasia
A
  1. Blast size
    • AML: large & uniform
    • ALL: small to medium & variable
  2. Chromatin
    • AML: finely dispersed
    • ALL: coarse
  3. Nucleoli
    • AML: 1 to 4; often prominent
    • ALL: absent or 1 to 2; indistinct
  4. Cytoplasm
    • AML: moderately abundant; often granules
    • ALL: scant to moderate; lack granules
  5. Auer rods
    • AML: Present in 60-70%
    • ALL: Absent
  6. Myelodysplasia
    • AML: Often present
    • ALL: Absent
28
Q

Cytochemical stains exploit the presence of intracellular enzymes within certain cells types that will produce a colored product from the stain. Name two enzymes used in this way, and the blast types they are found in.

A
  1. MPO (myeloperoxidase): myeloblasts
  2. NSE (non-specific esterase): monocytic blasts
29
Q

What is minimal residual disease?

A

(Wiki)

  • Small numbers of leukemic cells that remain in the patient during treatment, or after treatment when the patient is in remission.
  • It is the major cause of relapse in cancer and leukaemia.
  • Monitoring MRD is important in the personalization of leukemia treatment.
30
Q
  1. What technique is useful for differentiating between AML and ALL, as well as between different subtypes of AML and ALL?
  2. What are the subtypes of ALL?
  3. What are the major subtypes of AML?
A
  1. Immunophenotyping
  2. ALL
    • B-ALL
    • T-ALL
  3. AML
    • Megakaryocytic
    • Monocytic
    • Erythroid
    • Myeloblastic (granular cells)
31
Q

KNOW THIS SLIDE

In general, what CD antigens are typically present in the following acute leukemias?

  1. T-ALL
  2. B-ALL
  3. (Generic) Myeloid
    • What specific CDs are found with megakaryocytic AML?
    • Monocytic AML?
A
  1. T-ALL = Single-digit CDs (1-9)
  2. B-ALL = CDs around/in the 20s (19, 20, 22)
  3. General Myeloid = CDs in the teens or >30 (13, 15, 33, 117, MPO)
    • Megakaryocytic = CD41 & CD61
    • Monocytic = CD14
32
Q

Name four markers of cellular immaturity found in acute leukemia and the type of acute leukemia where each is found.

A
  1. CD34 (AML and ALL)
  2. TdT (mostly ALL)
  3. CD117 (AML)
  4. CD1a (immature T cells only)
33
Q

Define AML.

A
  • Systemic neoplasms of myeloid progenitor cells, primarily involving the blood and bone marrow but possibly extrameduallary sites as well. A heterogeneous set of disorders is generally poor outcome (long term survival of ~25%)
34
Q
  1. Describe the common clinical symptoms of AML.
  2. What consequence of the disease are all of these symptoms due to?
  3. Name three less common AML findings.
A
  1. Sxs:
    • Weakness
    • Fatigue
    • Petechiae
    • Infections
  2. All related to cytopenias
  3. Less common:
    • Organomegaly
    • Lymphadenopathy
    • Extramedullary tissue infiltration
    • Coagulopathy (in specific variants)
35
Q

What is the major diagnostic criterion of AML?

A

>20% blasts in the PB or BM

36
Q

Is the BM typically hyper- or hypocellular in AML?

A

**Hypercellular. **However, the BM may eventually fibrose and become “spent” or “burned out”, leading to hypocellularity.

37
Q

What CBC findings would be noted in AML?

A
  • Can have severe leukopenia ranging to marked leukocytosis
  • Anemia & thrombocytopenia are the norm
38
Q

Name the four WHO categories of AML.

A
  1. AML w/ recurrent cytogenetic abnormalities
  2. AML w/ myelodysplasia-associated changes
  3. AML & MDS, therapy-related (t-AML)
  4. AML not otherwise categorized
39
Q

Describe the specific features of AML with recurrent cytogenetic abnormalities. How is its prognosis?

A
  • “de novo” AML
  • flat incidence rate of different age groups
  • generally reciprocal translocations
  • distinctive morphologic features
    • dysplasia of maturing lineages is not prominent
  • No antecedant MDS
  • generally, favorable prognosis
40
Q

Describe the specific features of AML with MDS-associated changes. How is its prognosis?

A
  • Likely biologically related to MDS
    • May be antecedent MDS
  • MDS-like cytogenetic abnormalities
    • Complex karyotypes
      • Partial or whole losses of chromosomes
  • Increasing incidence with age
  • Prominent multilineage dysplasia
  • Poor prognosis
41
Q

Name four recurrent cytogenetic abnormalities seen with AML and any associated fusion proteins due to those abnoramlities. Which of the four has an unfavorable prognosis?

A
  1. t(8;21): AML1/ETO fusion protein
  2. inv(16): CBFbeta/MYH11 fusion
  3. t(15;17): PML/RARalpha fusion
  4. 11q23 (MLL) rearrangements
    • unfavorable prognosis
42
Q
  1. AML featuring which cytogenetic abnormality can be treated with a form of vitamin A?
  2. What is this vitamin A drug?
  3. How does this drug treat the disease?
A
  1. t(15;17)
  2. all-trans retinoic acid (ATRA)
  3. the retinoic acid receptor, important for myeloid maturation, becomes blocked via abnormal fusion of the PML and RARalpha genes. ATRA overcomes this block and allows the cells to mature, correcting the disease.
43
Q

What is the prognosis for AML with t(15;17)? What is the main cause of mortality?

A

Majority of pts - Extremely good. Give ATRA and they do just fine.

Minority of patients - early mortality due to Disseminated Intravascular Coagulopathy.

44
Q
  1. What hematologic features typically are seen in AML with t(15,17)?
  2. In which variant of this disease would you not see some of these features?
A
  1. Typical:
    • Hypergranular blasts
    • Reniform (kidney-shaped) nuclei
    • Leukopenia
  2. Variant:
    • “Microgranular variant”
    • Leukocytosis
45
Q

Langerhans cell histiocytosis (histiocytic neoplasm)

  • Name two immunophenotypic markers
  • Name a key feature seen on EM
  • What gene is often mutated in this condition?
A
  • Markers:
    1. CD1a
    2. Langerin
  • EM: Birbeck granules (“tennis racket” appearance)
  • BRAF mutations
46
Q

What the $@*% is hemophagocytic lymphohistiocytosis (HLH)?

A

(Wiki)

  • HLH is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages
  • characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines.
  • It is classified as one of the cytokine storm syndromes.
47
Q
  1. Defects in what gene cause primary HLH?
  2. What are two secondary causes of HLH?
A

Primary: Perforin gene mutations

Secondary: EBV, lymphomas

48
Q

What are 4 notable lab findings in HLH?

A
  1. Markedly high ferritin
    1. Intracellular iron-storing protein
  2. Hypertriglyceridemia
  3. Hypofibrogenemia
  4. Hemophagocytosis
    • Histiocytes phagocytose RBCs, leukocytes, platelets, and their precursors in the BM and other tissues (Wiki)

M2 Heme/Lymph (32 decks)

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