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Heme and Lymph > Path intro and WBC 1 > Flashcards

Path intro and WBC 1 Flashcards

1
Q

how many units of blood do we have?

A

10 . . 1 unit=1 pint

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2
Q

How long do RBCs last?
Platelets?
Fresh frozen plasma?

A

42 days
5 days
1 year

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3
Q

what % of total blood is plasma?

cellular components?

A

55%

45%

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4
Q

When do Blood cell progenitors first appear? and where?

A

third week of emyryonic development in the yolk sack

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5
Q

Cells derived from the yolk sac are the source of what?

A

long lived tissue macrophages such as microglial cells in the brain and Kupffer cells in the liver

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6
Q

Where do definitive HSCs arise before they migrate to the liver during the third month

A

mesoderm of intraembryonic aorta/gonad/mesonephros region

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7
Q

What is the chief site of blood cell formation until shortly before birth?

A

liver

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8
Q

When do HSCs shift location and take residence in the bone marrow?

A

4th month

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9
Q

Until puberty, hematopoietically active marrow is found where?
it then becomes restricted to where?

A

throughout the skeleton

axial skeleton

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10
Q

a HSC loses it’s ability to self renew once it is past what stage?

A

multipotent progenitor

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11
Q

Processes that distort the marrow architecture, such as deposition of metastatic cancer or granulomatous disorders, can cause abnormal release of immature precursors into the peripheral blood, a finding referred to as what?

A

leukoerythroblastosis

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12
Q

What provides the best assessment of the morphology of hematopoietic cells?

A

Marrow aspirate smears

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13
Q

in normal adults, the ratio of fat cells to hematopoietic elements is about what?

  • hypoplastic states? (aplastic anemia)
  • hematopoietic tumors or in diseases characterized by compensatory hyperplasias (hemolytic anemias) and neoplastic proliferations such as leukemias?
A
  • 1:1
  • proportion of fat increased
  • fat cells often disappear
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14
Q

What type of disorders often induce local marrow fibrosis?

How are the lesions best seen?

A

metastatic cancers and granulomatous diseases

-inaspirable so best seen in biopsies

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15
Q

Abnormally Low WBC count

A

leukopenia

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16
Q

reduction in number of neutrophils in blood

A

neutropenia

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17
Q

clinically significant reduction of neutrophils, making individuals susceptible to bacterial and fungal infections

A

Agranulocytosis

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18
Q

What is the most common cause of agranulocytosis?

A

drugs toxicity

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19
Q

Another name for a Natural Killer cell

A

Large granular lymphocyte

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20
Q

If we see extra medullary hematopoiesis after delivery, such as in the liver, what does this indicate?

A

a stressed infant

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21
Q

Where do we normally get Bone marrow from an Adult?

Pediatrics?

A

PSIS

sternum

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22
Q

What are the two cells that RESIDE in the peripheral blood?
where do leukocytes (monocytes, Granulocytes, and lymphocytes) reside?
Why are they seen in peripheral blood?

A
  • Platelets and RBCs
  • Tissue
  • Peripheral blood is a transport medium
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23
Q

lifespan of circulating platelets if no significant clotting occurs

A

7-10 days

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24
Q

Senescent platelets are eliminated how?

A

phagocytosis in the spleen

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25
Q

Are nucleated red cells normal in peripheral blood?

A

No

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26
Q

At a Hb level about what, is EPO released at a constant rate?

A

About 10

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27
Q

As Hemoglobin levels fall below 10, plasma EPO does what?

A

increases LOGARITHMAICALLY in response to increasing severity of anemia

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28
Q

A peripheral blood “immature” RBC

A

reticulocyte

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29
Q

What % of red cell population should reticulocytes be

A

2%

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30
Q

describe a reticulocyte compared to a mature RBC

A
  • contain remnant endoplasmic reticulum and ribosomes (rough ER) that forms a reticulum within the cytoplasm
  • 20-30% larger than mature RBC
  • circulate for 2-3 days before all remnants of ribosomal endoplasmic structures ae extruded
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31
Q

What may be used to possibly determine if we have an increase reticulocyte count?
what condition may possibly show this?

A
  • RDW: red cell distribution Width

- Anemias where RBCs are being produced

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32
Q

once a monocyte exits the intravascular space, it differentiates into what?

A

macrophage

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33
Q

Meloblast lineage leads to what cells

A

granulocytes

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34
Q

What cells of the myeloblast lineage should not be seen in peripheral blood

A
  • Myeloblast
  • promyelocyte
  • myelocyte
35
Q

What does a myeloblast lack

A

granules

36
Q

What is a juvenile granulocyte called?

what is a major feature?

A

Metamyelocyte

-indented nucleus

37
Q

in what circumstances could you see metamyelocytes in the peripheral blood

A

left shift (acute pyogenic infections)

38
Q

What Granulocytes can normally be seen In peripheral blood?

A
  • metamyelocyte
  • Band (horseshoe nucleus) - both of these in left shift
  • segmented polymorphonuclear leukocytes: neutrophils: PMNs or polys
39
Q

What do band forms not have?

A

filaments

40
Q

describe a mature neutrophil

A

lobation: thin thread-like chromatin filaments join 2-5 lobes

41
Q

life span of B lymphocytes?

T?

A

hours to days

days to years

42
Q

most (around 80%) of circulating lymphocytes in the peripheral blood are what

A

T cells

43
Q

% of plasma that is water

A

91%

44
Q

relative measure of Red cell mass or the RBCs as a % of blood volume

A

hematocrit

45
Q

plasma with diminished fibrinogen and other clotting factors

A

serum

46
Q

Ulcerating necrotizing lesions of the gingiva, floor of the mouth, buccal mucosa, pharynx, or elsewhere in oral cavity are quite characteristic of what?
Describe the lesions

A

Agranulocytosis

-Deep, undermined, and covered by gray to green/black necrotic membranes

47
Q

Severe neutropenia that occurs in association with monoclonal proliferations of large granular lymphocytes

A

LGL leukemia

48
Q

With excessive destruction of neutrophils in the periphery, the marrow is usually what?
What else causes this feature?

A

hypercellular due to a compensatory increase in granulocytic precursors
-neutropenias caused by ineffective granulopoiesis (megaloblastic enemias and myelodysplastic syndromes)

49
Q

Agranulocytosis caused by agents that suppress or destroy granulocytic precursors shows what in the marrow?

A

Hypocellularity

50
Q

Serious infections from agranulocytosis are most likely when the neutrophil count falls below what level?

A

500 per mm^3

51
Q

in some instances, such as following myelosuppressive chemotherapy, neutropenia is treated with what?

A

G-CSF

52
Q

In acute infections, there is a rapid increase in the egress of mature granulocytes from the bone marrow pool, an alteration that may be mediated through the effects of what?

A

TNF and IL-1

53
Q

What growth factor stimulates production of eosinophils?

Neutrophils?

A

IL-5

G-CSF

54
Q

In sepsis, or severe inflammatory disorders (Kawasaki disease), leukocytosis is often accompanied by morphologic changes in the neutrophils such as what?

A
  • Toxic granulations
  • Dohle bodies
  • Cytoplasmic vacuoles
55
Q

patches of dilated endoplasmic reticulum that appear as sky blue cytoplasmic “puddles”

A

Dohle bodies

56
Q

Acute lymphadenitis in the cervical region is most often due to drainage of microbes or microbial products from infections where?
Axillary or inguinal?
Mesenteric?

A

teeth or tonsils
extremities
acute appendicitis

57
Q

Describe the nodes of acute lymphadenitis

A

enlarged and painful

58
Q

Tingible body macrophages

A

follicular hyperplasia . . (chronic nonspecific lymphadenitis)

59
Q

Causes of follicular hyperplasia?

A
  • RA
  • Toxoplasmosis
  • early stages of infection with HIV
60
Q

Features favoring a reactive nonneoplastic follicular hyperplasia

A
  • Preservation of the lymph node architecture
  • marked variation in the shape and size of the follicles
  • The presence of frequent mitotic figures, phagocytic macrophages, and recognizable light and dark zones
61
Q

What causes paracortical hyperplasia?

A

stimuli that trigger T cell mediated immune responses such as acute viral infections (infectious mononucleosis)

62
Q

increase in the number and size of the cells that line lymphatic sinusoids

A

sinus histiocytosis (also called reticular hyperplasia)

63
Q

Sinus histocytosis is particularly prominent in lymph nodes draining what

A

cancers such as carcinoma of the breast

64
Q

Characteristically, lymph nodes in chronic reactions are what?
What locations?

A

nontender, as nodal enlargement occurs slowly over time and acute inflammation with associated tissue damage is absent
-inguinal and axillary nodes

65
Q

reactive condition marked by cytopenias and signs and symptoms of systemic inflammation related to macrophage activation

A 
Hemophagocytic Lymphohistiocytosis (HLH)
-Also referred to as macrophage activation syndrome
66
Q

The common feature of all forms of HLH is what?

A

systemic activation of macrophages and CD8+ cytotoxic T cells

  • the activated macrophages phagocytose blood cell progenitors in the marrow and formed elements in the peripheral tissue
  • Macrophages also release a cytokine stew or storm . . shock like picture
67
Q

What are familial forms of HLH associated with?

A

NK to properly form of deploy cytotoxic granules

68
Q

What is the most common trigger of HLH

A

infection, particularly EBV

69
Q

clinical features of HLH

A

-present with an acute febrile illness associated with splenomegaly and hepatomegaly

70
Q

What is usually seen on bone marrow exam with HLH

A

hemophagocytosis

71
Q

What do Lab studies reveal in HLH?

A
  • anemia, thrombocytopenia, and very high levels of plasma ferritin and soluble IL-2 receptor (inflammation)
  • elevated liver function tests and triglyceride levels (hepatitis)
72
Q

coagulation studies of HLH

A
  • evidence of DIC

- if untreated can progress rapidly to multiorgan failure, shock, and death

73
Q

prognosis of HLH without treatment?

A

Grim . .less than 2 months

74
Q

with prompt treatment of HLH about half patients survive but with what sequelae

A
  • renal damage in adults

- growth and mental retardation in children

75
Q

What are the 3 types of myeloid neoplasia and describe them?

A
  • Acute myeloid leukemia (AML): immature progenitor cells accumulate in the bone marrow
  • Myelodysplastic syndromes: associated with ineffective hematopoiesis and resultant peripheral blood cytopenias
  • Chronic myeloproliferative disorders: increased production of one or more terminally differentiated myeloid elements usually leads to elevated peripheral blood counts
76
Q

Dendritic cell proliferations . . .Langerhans cells

A

Histiocytoses

77
Q

what protoocogene is activated in germinal center B cell lymphomas by TRANSLOCATIONS to the transcriptionally active Ig locus

A

MYC

78
Q

What proto-oncogenes has a role in many B cell malignancies and is activated by POINT MUTATIONS

A

BCL6

79
Q

What inherited genetic diseases are at increased risk for acute leukemia?
childhood leukemia?

A
  • Bloom syndrome, Fanconi anemia, and ataxia Telangiectasia

- Down syndrome and Type I neurofibromatosis

80
Q

Virus and Malignancy: HTLV-1?

  • EBV
  • KSHV
A
  • T-cell leukemia/lymphoma
  • Burkitt lymphoma, 30-40% of HL, and many B cell lymphomas in the setting of T cell immunodeficiency
  • Malignancy effusion often in pleural cavity
81
Q

Chronic inflammation and malignancy: H. pylori?

HIV?

A
  • Gastric B-cell lymphomas and intestinal T cell lymphomas

- B-cell lymphomas within any organ. systemic hyperplasia of GERMINAL CENTER

82
Q

Smoking increases risk of what malignancy

A

Acute myeloid leukemia in adults

83
Q

Blasts in peripheral blood?

A

ALWAYS abnorma

Heme and Lymph (27 decks)

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