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Pathology Pt. 4 > Hematopoietic and Lymphoid 2 > Flashcards

Hematopoietic and Lymphoid 2 Flashcards

1
Q

What is polycythemia (erythrocytosis)? What are normal and abnormal hematocrit and Hb levels?

A

It is abnormally high red cell count.

Normal HCT in males is 42-52% and in females in 37-47%.

Polycythemia is when HCT is above 52% in males and above 47% in females.

Normal Hb levels are 13.2-16.7 g/dL in males and 11.9-15.0 in females.

Polycythemia is when Hb is above 18.5 in males and above 16.5 in females.

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2
Q

What is the difference between relative polycythemia and absolute polycythemia?

A

Relative is when there is decreased plasma volume (from dehydration or diarrhea, for example) with normal red cell mass, so red cell density is increased.

Absolute in an increase in the total red cell mass.

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3
Q

What is the difference between primary and secondary absolute polycythemia?

A

Primary is when EPO is normal or low, secondary is when EPO levels are high.

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4
Q

What disease is associated with autonomous, erythropoietin-independent growth of red cell progenitors?

A

Polycythemia vera, a myeloproliferative disorder (a type of primary absolute polycythemia)

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5
Q

How does primary familial polycythemia cause polycythemia?

A

Rare EPO receptor mutation –> receptor activation without binding EPO

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6
Q

Name three conditions that can induce secondary polycythemia.

A
  1. Lung disease
  2. High-altitude living
  3. Cyanotic heart disease

All cause a compensatory increase in EPO

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7
Q

Renal cell carcinomas, hepatocellular carcinomas, and cerebral hemangioblastomas are three examples of cancers that can cause paraneoplastic syndrome, secreting _______.

A

EPO –> secondary polycythemia

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8
Q

Name three genetic mutations that cause secondary polycythemia.

A
  1. Hb mutations that cause high Hb O2 affinity.
    Inherited defects that increase the stability of HIF-1alpha, leading to increased EPO. These include:
  2. Chuvash polycythemia (homozygous VHL mutations)
  3. Prolyl hydroxylase mutations
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9
Q

What are three clinical manifestations of polycythemia?

A
  1. Viscous blood
  2. Cardiac function and peripheral blood flow impairment
  3. Skin and mucosae appear dark red
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10
Q

What white cell count qualifies as leukopenia?

A

less than 4300 per uL

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11
Q

What neutrophil count qualifies as neutropenia/agranulocytosis?

A

less than 1600 per uL

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12
Q

When absolute neutrophil count drops below 1000 per uL, risk for _______ infections is high, and an absolute count below ______ per uL puts the person at serious risk for infection.

A

microbial infections below 1000

500 = serious risk

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13
Q

Can neutropenia/agranulocytosis be caused by decreased or ineffective neutrophil production from radiation, drugs, viral infections, congenital stuff, megaloblastic anemia, or myelodysplastic syndrome?

A

Yeah

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14
Q

Contrast the causes for neutropenia with bone marrow hypocellularity vs neutropenia with bone marrow hypercellularity.

A

Hypocellularity in the marrow would be caused by drugs that suppress granulocytopoiesis, whereas hypercellular marrow would occur when there is increased neutrophil degradation and the body is trying to compensate. Hypercellular also occurs when there is ineffective granulocytopoiesis.

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15
Q

Describe the clinical manifestations of neutropenia (6).

A
  1. Malaise
  2. Fever
  3. Marked weakness
  4. Fatigue
  5. Ulcerating, necrotizing lesions on mucous membranes
  6. Infections with microorganism growth.

Easy: feel like shit overall, ulcers on mucous membranes with microorganisms

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16
Q

How is someone with infections and neutropenia treated?

A

With granulocyte colony-stimulating factor

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17
Q

What is leukocytosis?

A

Non-neoplastic increase in blood WBCs

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18
Q

What four things does the severity of leukocytosis depend on?

A
  1. Size of myeloid and lymphoid precursor pools in marrow, thymus, circulation, and tissues.
  2. Rate of release from pools.
  3. The marginal pool of cells adhering to vessel walls.
  4. Rate of extravasation of cells from blood into the tissues.
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19
Q

Four histologic subtypes of leukocytosis are neutrophilic, eosinophilic, monocytic and lymphocytic. Name what causes each.

A

Neutrophilic: acute bacterial infections.
Eosinophilic: allergic disorders, parasitic infections.
Monocytic: chronic infections, bacterial endocarditis.
Lymphocytic: chronic immune stimulation as seen in TB, EBV, CMV, infectious mono.

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20
Q

Name three histologic characteristics of neutrophils from a peripheral blood smear that would be indicative of leukocytosis.

A
  1. Purple cytoplasmic granules (toxic granulations).
  2. Blue cytoplasmic patches of dilated ER.
  3. Nuclei looks like an elongated blob - indicates immaturity of neutrophil and increased rate of release.
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21
Q

What is lymphadenitis?

A

Non-neoplastic inflammatory proliferation of the lymph nodes.

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22
Q

What gross morphological changes are seen in the case of lymphadenitis? What microscopic changes are seen?

A

Nodes are swollen, gray-red, and engorged.

Microscopic: large germinal centers with lots of mitotic figures (nonspecific)

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23
Q

What cell type can be found in lymph nodes in the case of infection by pyogenic organisms?

A

Neutrophils

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24
Q

In what type of lymphadenitis would you find abcesses in the center of follicles of lymph nodes?

A

Acute nonspecific lymphadenitis

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25
Q

Compare follicular hyperplasia and paracortical hyperplasia. What type of lymphadenitis are these an example of?

A

These are types of chronic nonspecific lymphadenitis.

Follicular hyperplasia is associated with B cell activation –> germinal center (follicular) reaction

Paracortical hyperplasia is associated with T cell activation

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26
Q

Name three stimuli that can cause follicular hyperplasia and three stimuli that can cause paracortical hyperplasia.

A

Follicular (B cell expansion): RA, toxoplasmosis, early HIV

Paracortical (T cells): Viral infections like EBV, some vaccinations (small pox), certain drugs (phenytoin, dilantin for seizures)

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27
Q

What is the difference between a lymphoma and leukemia?

A

The site of the tumor. Leukemias primarily involve the bone marrow with spillage of neoplastic cells into the blood. Lymphomas are tumors in the lymph nodes, spleen, or extranodal tissues.

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28
Q

Name the four types of lymphoid neoplasms.

A
  1. Hodgkin lymphoma
  2. Non-Hodgkin lymphoma
  3. Lymphocytic leukemia
  4. Plasma cell dyscrasias
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29
Q

What is a myeloid neoplasm? Name three examples.

A

Neoplasms arising from stem cells that give rise to blood cells (granulocytes, red cells, or platelets). Monoclonal proliferation crowds out other cells.

Examples

  1. Acute myeloblastic leukemia
  2. Chronic myeloproliferative disorders.
  3. Myelodisplastic syndromes
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30
Q

What is acute myelogenous leukemia (AML)?

A

Clonal proliferation of immature myeloid cells (pre-RBCs or pre-granulocytes like neutrophils) that don’t terminally differentiate (they are stuck as immature cells). The proliferation happens in bone marrow and they end up in blood and tissues

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31
Q

What is the most common leukemia type in adults (80%)? What is the median age of onset for this disease?

A

AML, median onset age is 60

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32
Q

Which disease is associated with a mutation in inhibitory transcription factor, leading to excess proliferation of immature myeloid cells?

A

Acute myelogenous leukemia (AML)

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33
Q

Describe the symptoms, prognosis, and common treatment associated with AML.

A

Symptoms: fatigue, pallor, fever, infections, abnormal bleeding.

Prognosis: chemo –> remission in 50% but 5-year survival is only 30%.

Tx: marrow transplant

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34
Q

Name the disease: occurs due to t(15;17) translocation. Leads to the formation of an abnormal PML/RARA fusion protein that blocks myeloid differentiation at the promyelocytic stage

A

Acute promyelocytic leukemia (a type of acute myelogenous leukemia)

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35
Q

What is acute promyelocytic leukemia associated with (4)?

A
  1. Radiation
  2. Chemo
  3. Benzene exposure
  4. Smoking doubles risk
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36
Q

How is acute promyelocytic leukemia treated?

A

trans retinoic acid (allows cells to undergo terminal differentiation) + chemo –> good prognosis

37
Q

What is the main difference between chronic myelogenous leukemia and acute myelogenous leukemia?

A

In AML, cells CAN’T differentiate but in CML, cells RETAIN the capacity for terminal differentiation

38
Q

Describe the subcellular mechanisms involved in the development of CML.

A

BCR-ABL fusion –> constitutively active BCR-ABL tyrosine kinase –> constitutive hematopoietic growth signals –> proliferation of MATURE myeloid cells (particularly granulocytes and platelets)

39
Q

What genetic markers are needed to establish the diagnosis of chronic myelogenous leukemia?

A

The presence of the Philadelphia chromosome, or molecular demonstration of the BCR/ABL fusion gene

40
Q

CML accounts for _____ to _____% of all leukemia cases and is mostly seen in adults between _____ and _____ years old.

A

15-20%, between 25 and 60 years old

41
Q

How can CML be cured? Name one other treatment and state how it works.

A

Allogeneic bone marrow transplant early in the disease can cure.

Other tx is imantinib mesylate (Gleevec), which blocks the ATP binding site on the tyrosine kinase.

42
Q

What happens if CML goes untreated?

A

It inevitably progresses to a BLAST CRISIS that can resemble either AML or lymphoblastic leukemia

43
Q

Describe the major histologic characteristics of CML.

A

Hypercellular bone marrow due to an increase in granulocytes that are in VARIOUS STAGES of development and also the presence of MEGAKARYOCYTES

44
Q

What is the most common childhood leukemia? What percentage of cases in children under six does it make up?

A

B-Acute Lymphoblastic Leukemia/Lymphoma - 75% of cases in children under 6

45
Q

What is the difference between B-lymphoblastic leukemia and B-lymphoblastic lymphoma?

A

Leukemias involve the bone marrow and lymphomas involve the nodes. Remember!?

46
Q

What mutations are associated with B-lymphoblastic leukemia?

A

Philadelphia chromosome and other numerous chromosomal translocations

47
Q

Describe the histologic characteristics of B-Acute Lymphoblastic Leukemia/Lymphoma (6).

A
  1. Lots of one kind of cell that crowds out others.
  2. Increased nuclear to cytoplasmic ratio.
  3. Small to medium sized cells
  4. Delicate/condensed chromatin
  5. Small, inconspicuous nucleoli
  6. Scant, agranular cytoplasm
48
Q

What are the clinical features of B-Acute Lymphoblastic Leukemia/Lymphoma (2)?

A
  1. Anemia/thrombocytopenia/neutrophilia

2. Tumors in bone marrow cause mass effect –> bone pain and arthralgias (joint pain)

49
Q

How is B-Acute Lymphoblastic Leukemia/Lymphoma treated and what is the prognosis?

A

chemo, >90% complete remission rate

50
Q

What type of lymphoma is the most common type in the Western world?

A

Mature (peripheral) B cell lymphomas

51
Q

What are the two most common types of mature B cell lymphomas?

A
  1. Diffuse large cell lymphomas (30%)

2. Follicular lymphomas (22%)

52
Q

At what ages do mature B cell lymphomas most often occur? Which types can possibly be seen in children?

A

6th and 7th decades. The ones that can be seen in children are Burkitt lymphoma and large B cell lymphoma. Burkitt lymphoma is mostly seen in children.

53
Q

Which lymphoma is the most important type of lymphoma in ADULTS, accounting for 50% of non-Hodgkin lymphomas? Describe it.

A

Diffuse large B cell lymphoma - rapidly evolving, multifocal and multinodal B cell neoplasm

54
Q

What are the morphological characteristics of a diffuse large B cell lymphoma (4)?

A
  1. Neoplastic cells look like immunoblasts
  2. They exhibit various B cell antigens
  3. Cells are large (3-4x normal)
  4. Large nuclei with open chromatin and prominent central nucleoli
55
Q

Name the disease: BCL6 and BCL2 rearrangements often seen, asociated with immunodeficiency (like HIV) and is usually positive for EBV.

A

Diffuse large B cell lymphoma

56
Q

What is the treatment and prognosis for diffuse large B cell lymphoma?

A

Combination of chemo drugs –> good results (60-80% remission, 50% of those remain disease free for years)

57
Q

Which disease involves a chromosomal translocation of 8q24 (where the MYC oncogene is)?

A

Burkitt lymphoma

58
Q

What is the most common childhood malignancy in Africa, with a peak incidence at ages 3 to 7?

A

Burkitt lymphoma

59
Q

How aggressive is Burkitt lymphoma?

A

It is one of the most rapidly growing malignancies.

60
Q

____ is present in nearly all cases of endemic Burkitt lymphoma but it is seen in less than _____% of the sporadic types.

A

EBV, 30%

61
Q

Burkitt lymphoma produces ______ tumors and there is a high risk for ______ involvement.

A

extranodal, high risk for CNS involvement

62
Q

What is the classical presentation of Burkitt lymphoma?

A

Destructive tumor in the jaws or other facial bones

63
Q

Patients with sporadic Burkitt lymphoma typically present with ______ _____.

A

abdominal masses

64
Q

What is the prognosis for Burkitt lymphoma?

A

Both endemic and sporadic are curable in up to 90% of patients

65
Q

Starry sky macrophages

A

Burkitt lymphoma

66
Q

Name two histologic characteristics of Burkitt lymphoma.

A
  1. Lots of mitotic figures

2. Starry sky macrophages (macrophages with apoptotic tumor cells)

67
Q

What the hell is the difference between Hodgkin and non-Hodgkin lymphomas?

A

The presence or absence of Reed Sternberg cells. If these are present = Hodgkin.

Reed Sternberg cells (aka lacunar histiocytes) are giant cells usually derived from B lymphocytes, classically considered crippled germinal center B cells, meaning they have not undergone hypermutation to express their antibody.

68
Q

What is the most common malignancy of Americans between the ages of 10 and 30?

A

Hodgkin lymphoma

69
Q

Name the disease: Young person with a lymphoma without abdominal or cranial masses.

A

Probably Hodgkin lymphoma

70
Q

The prevalence of Hodgkin lymphoma is ________ with a peak in the late 20s and a gradual increase after age of 50.

A

bimodal

71
Q

Hodgkin lymphomas arise in a single node or a chain of nodes, therefore _______ is important in making the prognosis. ____ is also another important prognostic factor.

A

staging, age is also important

72
Q

Describe the histologic characteristics of Reed Sternberg cells.

A

Binucleate with large, inclusion-like nucleoli and abundant cytoplasm. OWL-EYE APPEARANCE: surrounded by lymphocytes, macrophages, and eosinophils.

73
Q

What are the clinical features of Hodgkin lymphoma?

A
  1. Lymphadenopathy with CERVICAL AND MEDIASTINAL nodes affected in more than half of the cases.
  2. Involves lymph nodes, spleen (1/3 of cases), liver (2/3), lungs (1/2) and rarely bone marrow
  3. Epidural spread is a frequent neuro complication
74
Q

Multiple myeloma is a neoplasm of ________ cells, a type of ________ _______ B cell.

A

plasma cell, a type of terminally differentiated B cell

75
Q

What genetic changes are associated with multiple myeloma?

A

Dysregulation of D cyclin and chromosomal translocations

76
Q

Name four risk factors for multiple myeloma.

A
  1. Genetics: first degree relatives, higher (2x) frequency in blacks.
  2. Ionizing radiation
  3. Chronic antigenic stimulation
  4. Age (increases with age - mean age at Dx is 65)
77
Q

Describe the morphological and histological changes seen in multiple myeloma (3).

A
  1. Multifocal infiltration of the marrow by malignant plasma cells with multiple destructive (lytic) lesions or diffuse demineralization of bone.
  2. Punched-out lesions of the skull
  3. Plasma cells in marrow aspirate that have multiple nuelci, prominent nucleoli, light spots on the golgi, and cytoplasmic droplets containing Ig.
78
Q

Punched out lesions of the skull

A

Multiple myeloma

79
Q

In most cases of multiple myeloma, tumor cells secrete a single, homogenous, complete or partial _______, a _______, or most commonly, _____ or _____ light chains (AKA ______ ______ protein).

A

secrete homogenous complete or partial antibodies, M-component or paraprotein, or most commonly IgG or IgA light chains (Bence Jones protein)

80
Q

Describe the effects of multiple myeloma on bone tissue.

A

Tumors can secrete osteoclast-activating factor –> vertebral compression fractures, fractures of other bones, chronic pain, hypercalcemia.

81
Q

Can multiple myeloma cause anemia? How?

A

Yeah! The monoclonal plasma cell crowds out other cells.

82
Q

In which blood cancer is light-chain (AL) amyloidosis seen?

A

Multiple myeloma

83
Q

What is the prognosis of multiple myeloma?

A

Incurable - mean survival 6 months (untreated) and 3 years with treatment. Death usually due to infection or renal failure.

84
Q

Infections, immunologic inflammatory disorders like SLE and amyloidosis, hemolytic anemias, immune thrombocytopenia, splenic vein hypertension from right side heart failure, primary or metastatic neoplasms, and storage diseases like Gaucher’s can all cause ________.

A

splenomegaly

85
Q

What is a thymoma? Are they most often malignant? What age do they occur?

A

Neoplasm of thymic cortical or medullary epithelial cells. Most often benign (80%). Occur in adulthood.

86
Q

Describe a typical thymoma.

A

Encapsulated, irregularly shaped mass, firm, gray-to-yellow. Divided into lobules by fibrous septa

87
Q

Thymomas are often associated with __________ syndromes (myasthenia gravis, Graves, Cushing).

A

paraneoplastic

88
Q

What is the difference between acute myeloid leukemia and acute promyelocytic leukemia?

A

Acute promyelocytic leukemias are classified as such because they respond to trans-retinoic acid treatment.

Pathology Pt. 4 (10 decks)

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