Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

HMS Homeostasis I > Non-Malignant Leukocyte Disorders > Flashcards

Non-Malignant Leukocyte Disorders Flashcards

1
Q

Organization of bone marrow

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Indications for performing a bone marrow examination

A
  • Unexplained decrease or increase in blood cell counts beyond the normal range
  • Presence of abnormal cells in the blood, such as immature leukocytes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
A

Normal bone marrow aspirate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

“Dry tap”

A

When you tap someone’s bone marrow but you don’t get anything.

Indicates bone marrow fibrosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Bone marrow aspirate vs bone marrow biopsy

A

Bone marrow aspirate: Great for cytology, but doesn’t give much information about organization or cellularity of marrow biopsy, and may come back dry.

Bone marrow biopsy: Gives an idea of cellularity and organization of marrow, but not nearly as useful for cytological purposes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
A

Normal bone marrow biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Normal vs aplastic anemia bone marrow biopsy

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Where does marrow aplasia come from?

A

Marrow aplasia is thought to arise from genetic alterations in hematopoietic stem cells, either spontaneous mutations or mutations induced by an extrinsic insult such as a drug, toxin, or viral infection.

May be due to impaired capacity for proliferation, or due to acquired neoantigen and subsequent attack by CD8 T cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The most frequent cause of marrow aplasia

A

Iatrogenesis

Due either to drugs or radiation therapy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Chloramphenicol

A

An antibiotic

For whatever reason, can induce irreversible aplastic anemia in an idiosyncratic manner, affecting only an extremely small fraction of exposed individuals.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Evaulating a patient with aplastic anemia

A

Elicit a thorough history of exposure to toxic chemicals, particularly hydrocarbons and other industrial solvents.

The most frequent offender is benzene. Viral infections, particularly non-A, non-B, and non-C hepatitis, can occasionally cause severe, irreversible marrow aplasia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Idiopathic aplastic anemia

A

Patients with marrow aplasia not due to drugs, toxins, or viral infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Clinical presentation of aplastic anemia

A
  • Progressive fatigue
  • Pallor
  • Acute infection symptoms (if associated neutropenia)
  • Petechiae and ecchymoses (if associated thrombocytopenia)
  • Lack of lymphadenopathy
  • Severe anemia on CBC with high retic count and therefore high MCV
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pancytopenia CBC

A
  • Low erythrocytes
  • Low thrombocytes
  • Low neutrophils
  • Low monocytes
  • Often higher lymphocytes due to higher lifespans
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Normal vs aplastic marrow density (%s)

A

30-50% is normal

aplastic is usually <10%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Therapy for aplastic anemia

A
  • Discontinue myelosuppressive drug or toxin exposure
  • Transfusion if needed
  • Hematopoietic stem cell transplant
  • Immunosuppression if CD8-mediated autoimmune process
  • If HSC transplant not possible in severe cases, calcineurin inhibitors (cyclosporine) and antithymocyte/antilymphocyte globulin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Pure red cell aplasia

A

Severe anemia but normal white cells and platelets.

May be immune mediated, as suggested by presence of thymoma (tumor of thymic epithelial cells), lymphoma, or autoimmune disorder.

May be due to acquired anti-EPO in patients on EPO supplementation.

May be due to parvovirus B19.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Myelophthisis

A

Crowding out of bone marrow, most often by metastatic cancer.

Some causes, such as metastatic cancer, will also cause fibrosis of surrounding marrow, worsening the anemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q
A

Smear indicative of myelophthisic anemia with myelofibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Neutrophil storage pool

A

Mature neutrophils that reside in the bone marrow, waiting for infection before springing out.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Neutrophil circulating pool

A

Neutrophils outside of the bone marrow and in the bloodstream circulating at any given moment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Neutrophil marginal pool

A

Neutrophils sequestered on the walls of blood vessels at any given moment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Normally, ___ of the neutrophils in the body are in the circulating pool

A

Normally, <5% of the neutrophils in the body are in the circulating pool

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Myelopoiesis

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

“Left shift”

A

Release of band cells into circulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Leukemoid reaction

A

Extreme leukocytosis due to left shift that mimicks leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Leukoerythroblastosis

A

Inflammatory process involving the marrow itself that produce marrow fibrosis and distortion, leading not only to the release of immature myeloid elements but also nucleated erythroid progenitors and misshapen tear-drop red cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Hereditary neutrophilia

A

Rare form of chronic granulocytosis that usually exhibits an autosomal dominant pattern of inheritance.

Often gain-of-function mutations in the granulocyte colony-stimulating factor (G-CSF) receptor that lead to marrow hyperstimulation and increased neutrophil production.

29
Q

Down syndrome myeloproliferative disorder

A

About 10% of infants with Down syndrome develop a transient myeloproliferative disorder characterized by granulocytosis, circulating immature myeloid precursors, and hepatosplenomegaly.

This disorder appears to be caused by acquired mutations in the transcription factor GATA-1 that result in deregulated growth of myeloid progenitors. Resolves spontaneously in 75% of patients, but 25% develop full-blown acute myeloid leukemia over a period of months to several years.

30
Q

Leukocyte adhesion deficiency

A

Rare autosomal recessive disorder. Deleterious effects on the function of β2 integrin (LAD I) or the selectin ligand PSGL-1 (LAD II).

Elevated neutrophil counts, particularly in the face of infection, due to an inability of neutrophils to adhere to endothelium and migrate into infected tissues.

31
Q

Secondary causes of granulocytosis

A
  • Stress (catecholamines and glucocorticoids cause demargination)
  • Infection
  • Inflammation of noninfectious etiology
  • Drug reactions
  • Marrow hyperstimulation (like in megaloblastic anemia or ITP)
32
Q

Döhle bodies and toxic granulations

A

Both seen in neutrophils in acute infection.

Toxic granulation: Persistence of neutrophil granules.

Döhle bodies: pale blue cytoplasmic aggregates of endoplasmic reticulum

33
Q

The second most common form of leukocytosis is ___

A

The second most common form of leukocytosis is eosinophilia

34
Q

Causes of eosinophilia

A
  • Th2 reaction
  • Worm infection
  • SLE or vasculitis (rheumatic disease)
  • Certain myeloproliferative neoplasms
  • Reaction to Hodgkin’s lymphoma
35
Q

Basophilia

A
  • Uncommon, if found usually in association w/ eosinophilia
  • Sometimes seen in asthma or atopy
  • Occurs in some myeloid neoplasms like CML
36
Q

Monocytosis

A

Occasionally seen in association with tuberculosis or myeloid neoplasms, particularly those myeloproliferative neoplasms in which the monocytes and their precursors are a component of the malignancy.

37
Q

Lymphocytosis

A
  • Most commonly due to gamma group Herpesvirus infection
  • Bordetella pertussis releases a toxin that inhibits the migration of lymphocytes out of the blood into lymph nodes, causing lymphocytosis
38
Q

Activated CD8 T cells

A

Characteristically have abundant cytoplasm containing a few azurophilic granules and are sticky, often adhering to surrounding red cells

39
Q
A

Mononucleosis

40
Q

Evaluating neutropenia

A
  • Helpful to obtain serial neutrophil counts
  • Careful history can determine whether the neutropenia is likely to be acute or chronic
  • Examine oropharynx, lymph nodes, spleen, and perianal region for signs of infection
41
Q

Treatment of neutropenia

A

Initial treatment: Manage complications!!! If the patient has a fever, give them broad spectrum antibiotics immediately, even if cultures are negative.

Long-term treatment: Restore neutrophil count. Stop nonessential drugs (resolves 7-10 days), G-CSF administration, HSC transplant. Autoimmune may be managed with glucocorticoids. LGL responds well to glucocorticoids and low-dose methotrexate,

42
Q

Common causes of lymphopenia

A
  • HIV
  • Chronic corticosteroid treatment
  • DiGeorge syndrome (thymic aplasia due to germline deletions involving chromosome 22q11)
  • SCID (RAG1/RAG2 mutations)
43
Q

Qualitative neutrophil dysfunction

A
  • Lack of adhesion
  • Lack of chemotaxis
  • Lack of pathogen recognition and phagocytosis
  • Lack of granule fusion machinery (inability to degranulate)
  • Lack of pathogen-killing equipment (especially Phox)
44
Q

Chédiak-Higashi syndrome

A

Disorder affecting neutrophil granules. Abnormal granules in this disorder result in impaired bacterial killing.

45
Q

Hemophagocytic lymphohistiocytosis

A

Variety of settings and has a number of triggers. Common feature in all is the activation of macrophages throughout the body due to overstimulation by factors released by effector T cells. Activated macrophages phagocytose hematopoietic cells and release additional cytokines and chemokines, producing a systemic inflammatory state associated with cytopenias.

46
Q

What’s wrong with this bone marrow aspirate?

A

Hemophagocytic histiocytosis

47
Q

Treating HLH

A
  • Primary: Responds to chemotherapy and immunosuppressive agents but inevitably recur unless hematopoietic stem cell transplantation is performed.
  • Secondary: Treat with therapy for the underlying illness, but also require HLH management with chemotherapy and immunosuppressive agents. Etoposide particularly effective.
48
Q

Etoposide

A

Topoisomerase II inhibitor, chemotherapeutic

49
Q

Spicule

A

Mix of fat and bone bits. Come through on bone marrow aspirates. If you are looking at what you are told is a marrow aspirate and you don’t see this, you have been horribly deceived, this is not a bone marrow aspirate.

This may occur with “hemodilute” aspirates, i.e. aspirates that are really more blood than bone marrow.

50
Q

Normocellular marrow

A

Equally cells and fat

Kids may have less fat, geriatrics may have more

51
Q

Bone marrow biopsy or aspirate precursor lineages

A
52
Q

Granulopoiesis summary

A
53
Q

“Leukemoid reaction” is really two things together

A

Leukocytosis + left shift

54
Q

Leukoerythroblastic response is really 3 things:

A

Left shift, leukocytosis, erythrocytosis

Overproduction and “left shift” of all lineages

55
Q

___ antibiotics may trigger leukocytosis.

A

-cycline antibiotics may trigger leukocytosis.

56
Q

Elevation of platelet count in leukemoid reaction is probably ___.

A

Elevation of platelet count in leukemoid reaction is probably a physiologic response.

Slgiht thrombocytopenia is often part of the acute phase response and is non-malignant.

57
Q

You are only really worried about neutropenias that are under ___.

A

You are only really worried about neutropenias that are under 500 per unit vol.

58
Q

Benign neutropenia of childhood

A

Presents in some infants (6-12 months)

Do get some prophylactic antibiotics, but almost always resolves by end of early childhood.

59
Q

Neutropenia may result following ___ infection.

A

Neutropenia may result following viral infection.

60
Q

Managing a patient with neutropenia

A

Preventive: Neutropenic precautions, isloation, + pressure

Supportive: Aggressive antibiotics during infection

Therapeutic:

  • Immune etiology: steroids, IVIG
  • Neutropenia of immunodeficiency: methotrexate
  • Congenital neutropenias and drug-induced neutropenias: G-CSF
  • Severe congenital neutropenias: HSC transplant
61
Q

Lovitch Leukocytosis Flowchart

A
62
Q

Lovitch Neutropenia Flowchart

A
63
Q

Lovitch Pancytopenia Flowchart

A
64
Q

One platelet on a 40x field is roughly ___ in circulation.

A

One platelet on a 40x field is roughly 10,000 per unit in circulation.

65
Q

EDTA artifacts on smear

A

EDTA in blood preparation may result in some burr/spur cells as an artifact. This is not abnormal.

66
Q

What are the major sources of G-CSF?

A
  • Endothelial cells
  • Fibroblasts
  • Macrophages
67
Q

Common causes of drug-induced leukocytosis

A
  • Catecholamines (demargination)
  • Beta adrenergics (demargination)
  • Corticosteroids
68
Q

General approach to leukopenia/leukocytosis

A

HMS Homeostasis I (62 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions