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Blood and Lymph > Unit 1 > Flashcards

Unit 1 Flashcards

1
Q

The cellular component of the blood makes up about ______% of its volume

A

40-45%

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2
Q

Serum

A

When clotted blood is centrifuged, the clear fluid that collect at the top of the tube. Lacks clotting factors which have been consumed to make the clot

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3
Q

Plasma contains:

Ends up where in a centrifuge tube?

A

Proteins, lipids, salts, carbohydrates. Sits at the top of the centrifuge tube on top of the buffy coat layer

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4
Q

Buffy coat layer contains:

Ends up where in a centrifuge tube?

A

White blood cells and platelets. Sits below the plasma on top of the RBCs

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5
Q

Where do the RBCs settle in a centrifuge tube?

A

At the bottom, below the buffy coat layer and plasma

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6
Q

hematocrit

A

The proportion of blood by volume made up of red blood cells

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7
Q

formula for determining hematocrit

A

the length of the RBC layer and dividing it by the total length of the column of blood [RBCs/(RBCs + buffy coat + plasma)].

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8
Q

the “differential” of a CBC

A

percentages of the different types of white blood cells in the blood

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9
Q

peripheral smear

A

A drop of blood can also be smeared on a glass slide, stained, and examined under the microscope to look for any abnormally shaped cells or cellular inclusions

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10
Q

What makes up the bulk of the cellular components of blood?

A

Erythrocytes

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11
Q

Unique features of an RBC

A

1) lack a nucleus
2) lack mitochondria
3) contain lots of hemoglobin

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12
Q

Do RBCs have mitochondria or nuclei?

A

They have these organelles in the bone marrow but lose them prior to their being released into the periphery.

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13
Q

hemolysis

A

premature breakdown and RBC destruction

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14
Q

Mutation causing sickle cell anemia

A

a substitution of valine for glutamic acid at the 6th position of the beta-globin chain) makes hemoglobin S

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15
Q

Why do RBCs have limited ability to respond to changes in the environment

A

mature RBCs lack nuclei, they can’t make new RNA. Once they’re released in the periphery, they have limited ability to repair themselves. Also, since they lack mitochondria, they are dependent on anaerobic metabolism for generation of ATP to maintain cellular processes

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16
Q

the most common human enzyme defect

A

glucose-6-phosphate dehydrogenase (G6PD) deficiency, an X-linked disorder seen in ~15% of the African male population can also be a cause for hemolytic anemia.

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17
Q

How do RBC to undergo large reversible deformations while maintaining its structural integrity?

A

2D elastic network of cytoskeleton, tethered to sites on cytoplasmic domains of transmembrane proteins embedded in the plasma membrane

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18
Q

Anemia can result from deficiency in the following vitamins/minerals: ____, ____, ____

A

iron, B12, Folic acid

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19
Q

erythropoietin is made in the

A

kidney

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20
Q

5 different types of WBCs

A 
lymphocytes
neutrophils (also known as polymorphonuclear cells or PMNs)
monocytes
eosinophils 
basophils
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21
Q

____________ are the key players in the adaptive immune response, which involves the development of “memory” following exposure to an infectious agent, providing the ability to respond more vigorously to repeated exposure to the same agent

A

Lymphocytes

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22
Q

“myeloid” cell types include

A

neutrophils, monocytes, eosinophils, and basophils

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23
Q

Innate immunity

A

protection against infection that relies on mechanisms that exist before infection, are capable of a rapid response to microbes, and react in essentially the same way to repeat infections

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24
Q

hemostasis

A

the arrest of bleeding

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25
Q

megakaryocytes

A

large, polyploid cells in the bone marrow that fragment to form platelets. 1 megakaryocyte=5000 platelets

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26
Q

what does the fibrinolytic system do?

A

breaks down formed clots

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27
Q

“Job” of innate immunity

A

to detect intruders that have ventured too deep into the body’s structures, and then arrange for their inactivation, destruction, and removal.

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28
Q

Innate immunity recognizes three sorts of things:

A

 pathogen-associated molecular patterns (PAMP);
 damage- associated molecular patterns (DAMP);
 The absence of normal cell surface molecules

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29
Q

pattern-recognition receptors, PRR

A

Innate immune cells have PRR on their surface or on inner membranes to identify pathogen-associated molecular patterns (PAMPs).)

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30
Q

“Toll-like receptors,” TLRs

A

Each TLR can recognize a foreign molecular structure that we humans don’t have. Receptors on membrane for bacterial structures (cell wall structures, for example).

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31
Q

The factors released in an inflammatory response are

A

cytokines and chemokines

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32
Q

Inflammation

A

increased blood vessel diameter, stickiness, and leakiness, efflux of fluid and phagocytic white blood cells into the tissues. The intent is to quickly get defense and healing agents into the damaged or invaded area.

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33
Q

What part of the immune system is fastest? Innate or adaptive?

A

Innate is fastest, but it cannot adapt

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34
Q

Controlled substances are divided into 5 schedules according to potential for:

A
  • Medical usefulness
  • Abuse potential
  • Degree to which they may lead to physical / psychological dependence if they are abused.
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35
Q

Which schedule has the highest potential for abuse? Are they prescribed?

A

Schedule 1, not prescribed because no medical use

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36
Q

Which schedule of drugs have the highest potential for abuse among those that can be prescribed?

A

schedule 2

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37
Q

Current requirements for prescribing controlled drugs:

A
  • DEA number
  • Schedule I drugs may not be prescribed.
  • All schedule II - IV drugs require a prescription (in Colorado II - V).
  • Schedule II prescriptions must be in ink in prescriber’s handwriting, and cannot be telephoned to pharmacist or refilled .
  • Schedule III and IV (plus V in Colorado) may be telephoned to pharmacist and may be refilled no more than 5 times in 6 months (if so noted on prescription).
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38
Q

Components of a written prescription

A 
drug name
date
ID of prescriber
patient info
Drug strength, quantity, dosage
Directions to patient (regimen)
refill info
signature
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39
Q

Federal regulation of drugs

A

Controls drugs through the FDA
FDA regulates:
• Evaluation of safety and efficacy of new drugs prior to availability, removal of dietary supplements deemed unsafe
• Equivalency of brand name versus generic drugs
• Placement of drugs into prescription vs non-prescription categories

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40
Q

State regulation of drugs

A

Controls who may prescribe drugs through the licensing process of medical or dental boards. Exception is the prescribing of Controlled Substances which requires registration with the Drug Enforcement Administration (DEA)

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41
Q

Four Basic Drug Categories

A

Prescription – Controlled Substances – OTC - Dietary Supplements

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42
Q

The Food, Drug, and Cosmetic of 1938 (and Kefauver Amendments of 1962) requires that for new drugs:

A

manufacturers must demonstrate proof of efficacy (do they work?) as well as safety before they can be marketed to the public.

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43
Q

Pre-Clinical Testing of a new drug

A

5-8 years, animals,
• rodent and non-rodent species; pharmacology, drug metabolism, and toxicity .
• determine safe dosage range for humans.
• Successful review of data leads to Investigational New Drug (IND) application to the FDA. An NDA is filed for a specific indication (use).

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44
Q

Clinical trials, phase 1

A 
Clinical pharmacology (1 yr)  [Is it safe, pharmacokinetics?]
•	Very select normal volunteers (usually < 100, healthy males, 18-45 y/o)
•	Toxicity (dose level of first appearance) and metabolism studies
•	Determine if animal / human response differ significantly
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45
Q

Clinical trials, phase 2

A

Clinical investigation (2 yrs) [Does it work in patients?]
• Select patient pool (200-300), no other medical problems.
• Comparison to placebo or existing treatment.
• Safety and efficacy, final dosing and regimen adjustments
• Usually at university or government medical center under supervision of IRB with patient consent.

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46
Q

Clinical Trials, phase 3

A

Full scale clinical trial (3 yrs) [Does it work, double blind?]
• 1000-6000 patients, settings similar to ultimate use of drugs.
• Efficacy measured against established therapy. Monitor adverse reactions from chronic use.
• Positive results after unmasking of code may result in approval of New Drug Application (NDA)

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47
Q

Post-marketing Surveillance, phase 4

A
  • required to submit reports to the FDA of adverse effects of drugs on market
  • Studies often continue after approval. Collect data on mortality / morbidity - monitor safety under actual conditions of use
  • Study groups omitted during phases I and II (high risk-pregnancy, elderly, children); also patients with multiple disease
  • FDA can revoke approval/restrict drug use (e.g., require certain lab tests to be performed) if unpredictable adverse effects become apparent when drug is made available to large, uncontrolled populations
  • Low incidence drug effects will be missed in phases I-III
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48
Q

Pharmaceutical Equivalents

A

Drugs containing same:

  • active ingredient (s) in the same dosage formulation (capsule, tablet, solution, etc.) that have the
  • route of administration
  • strength or concentration
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49
Q

Pharmaceutical Alternatives

A

Drug products containing the same therapeutic moiety, but are different:
- salts, esters, or complexes of that moiety,
or
-different dosages forms (e.g., capsules vs tablets) or
-strengths (e.g., 200 mg vs 250 mg)

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50
Q

Bioequivalent Drug Products

A

• pharmaceutical equivalent formulations that display comparable bioavailability

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51
Q

Bioavailability

A

the rate and extent to which the active ingredient is absorbed from a drug formulation and becomes available at the site of action (i.e., drug molecule entering bloodstream).

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52
Q

How is bioavailability measured?

A

The extent of absorption (bioavailability) is measured by the area under the plasma concentration-time curve (AUC)

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53
Q

How is the rate of absorption measured?

A

the rate of absorption is estimated by the maximum of peak drug concentration (Cmax)

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54
Q

Therapeutic Equivalents

A

Pharmaceutical equivalents that, when administered to the same individual in the same dosage regimen, provide the same efficacy and safety.

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55
Q

Is proof of efficacy required for drugs and dietary supplements?

A

evidence via clinical trial is required for drugs, no proof of efficacy is required for dietary supplements

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56
Q

Is proof of purity required for drugs and dietary supplements?

A

for drugs yes, and for dietary supplements-yes since 2011

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57
Q

Is quality control required for drugs and dietary supplements?

A

yes, for both

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58
Q

1 grain=____ mg

A

64.8mg

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59
Q

1gram= _____ grains

A

15.43

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60
Q

1 drop= ____ mL

A

0.05

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61
Q

1t= ___mL

1T=___mL

A

5mL

15mL

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62
Q

1fluid oz= ___mL
1 quart= ___ mL
1 pint= ___mL
1 gallon= ___mL

A

30
946
473
3785

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63
Q

anemia

A

insufficient red cell mass to deliver oxygen to peripheral tissues

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64
Q

thrombosis

A

formation of a clot within a blood vessel, occludes flow

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65
Q

Five ties of WBCs in the blood

A

lymphocytes, neutrophils, monocytes, eosinophils, basophils

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66
Q

How many platelets come from a single polypliod megakaryocytic?

A

5000

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67
Q

lymphoma

A

“extramedullary”=outside of bone marrow. Collection of malignant lymphoid cells in the lymph nodes and lymph organs

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68
Q

Leukemia

A

malignant cells arise from bone marrow and are usually in the bloodstream. Can be acute or chronic, and lymphoid or myeloid

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69
Q

Acute Leukemia

A

cells are immature in their degree of differentiation and clinical course is usually rapidly progressive without intervention

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70
Q

Chronic leukemia

A

cells are more mature in their differentiation and disease follows a more indolent clinical course

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71
Q

lymphoid leukemia

A

arises from a lymphocytic origin

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72
Q

Myeloid leukemia

A

arising from one of the myeloid cell types in the marrow (neutrophils, monocytes, eosinophils, or basophils)

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73
Q

Flow cytometry uses a ____ to measure the scattering of light

A

laser

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74
Q

Coulter principle

A

counts cells and sizes as the cell passes through a hole, the voltage drops and is measured (proportional to cell size)

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75
Q

RBC count units

A

millions/uL or godzillion (10^12)/L

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76
Q

Hemoglobin abbreviation and units

A

HGB, g/dL or g/L. In vitro measurement of [Hb] released by lysed cells in whole blood

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77
Q

Hematocrit abbreviation and units

A

HCT, ratio of total RBC volume/whole blood, how much of a given volume of blood is occupied by RBCs, expressed as % or L/L

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78
Q

MCH

A

Mean cell hemoglobin: the mean quantity of Hb in a single red cell.
Units: picograms
MCH=(HGB/RBC) X 10

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79
Q

MCHC

A

mean cell hemoglobin concentration: average [Hb] in red cells
Units: g/dL or g/L
MCHC=(HGB/HCT) X 100

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80
Q

MCV

A

mean cell volume: the mean size of the red cells counted
Units: femtoliters fL (10^-15)
MCV=(HCT/RBC) X10

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81
Q

Patter recognition receptor

A

PRR, primitive protein expressed by the innate immune system that identifies PAMPs on intruders

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82
Q

Pathogen-assocaited molecular pattern

A

PAMP, molecules associated with a group of pathogens that are recognized by the innate immune system. Recognized by PRRs and TLRs

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83
Q

Toll-like receptor

A

TLR, a type of PRR that recognizes foreign molecular structure that humans don’t have. TLR binding triggers a cascade that leads to inflammation and release of cytokines/chemokines

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84
Q

Damage-associated molecular pattern

A

DAMPs, molecules that initiate an immune response (noninfectious, whereas PAMPs initiate/perpetuate an infectious response), expressed by cells in trouble, cells that have been invaded, etc

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85
Q

Common patterns recognized by TLRs

A

lipoproteins, zymosan, glycolipids, dsRNA, ssRNA, lipopolysaccharides, flagellin, unmethylated CpG in DNA

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86
Q

What is the final transcription factor that is most commonly activated in inflammation?

A

NF-kB

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87
Q

cytokine

A

short range mediator made by any cell that affects the behavior of the same or another cell

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88
Q

chemokine

A

small cytokines that are short range mediator made by any cell, primarily cause inflammation. The are CHEMOtactic cytoKINES, can recruit phagocytic WBCs (like around a splinter)

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89
Q

______ are the cells that bridge innate and adaptive immunity

A

dendritic cells (DC)

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90
Q

Dendritic cells

A

phagocytic, antigen-presenting cells. At a would site, DC activated by cyto/chemokines, takes up material from invaders. Activated DC leaves, travels to nearest draining lymph node. Shows material to lymphocytes to get adaptive immune response

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91
Q

T cells

A

survey the surfaces of the cells by recognizing antigens presented by DCs with their surface receptors. this activates the T cell, it proliferates, daughters travel to antigen innovation site, release lymphokines to augment inflammation, attracts macrophages and monocytes

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92
Q

B cells

A

Recognize antiges via cell surface receptors, become activated, proliferate. They secrete Abs (soluble versions of the receptors)

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93
Q

IgG

A

most abundant, 2 adjacent IgG molecules bind an antigen and cooperate to activate complement, a system of proteins that enhance inflammation and pathogen destruction. Pass from mom–> baby

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94
Q

IgM

A

large polymetric immunoglobulin. 1st Ab to appead in blood after exposure to antigen (better at activating complement than IgG), gets replaced by IgG

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95
Q

IgD

A

form of Ab inserted into B cell membranes as their antigen receptor

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96
Q

IgA

A

Most important class of Ab in secretions (saliva, tears), resistant to digestive enzymes

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97
Q

IgE

A

attach to mast cells. when they encounter an antigen it will cause the cast cell to make prostaglandins, leukotrines, and cytokines and release its granules with powerful inflammatory mediators like histamine. Allergy symptoms! Role in parasite resistance

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98
Q

Type 1 hypersensitivity

A

too much IgE, seen in asthma and anaphylactic shock

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99
Q

anaphylactic shock

A

mast cells suddenly degranulates, release histamine

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100
Q

Type 2 immunopathology

A

autoimmunity due to Abs reacting to self

treated with immunosuppresant and anti-inflammatory drugs

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101
Q

Type 3 immunopathology

A

Abs made to soluble antigens, activate inflammation and damage tissue
symptoms: arthritis, glomerulonephritis, pleurisy, rash, systemic lupus erythematosus (SLE)-Abs attach DNA, RA=Abs attack abs

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102
Q

Chronic frustrated immune response

A

antigen is not “self” but something that cannot get rid of: IBC, celiac

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103
Q

type 4 immunopathology

A

T cell mediated, can be autoimmune.

TB, hepatitis

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104
Q

Reticulocyte count

A

measures how fast RBCs are made and released into blood. Measured as a % of 1000 red cells counted
Normal=0.4-1.7% of red cells counted

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105
Q

Reticulocyte index

A

RI: corrects reticulocyte conunt for [red cells] and stress reticulocytosis
RI=reticulocyte count X (patient HGB/Normal HGB) X (1/stress factor)
Normal RI between 1.0 and 2.0

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106
Q

Symptoms of anemia

A

shortness of breath, fatigue, rapid HR, dizziness, claudication or pain with exercise, pallor

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107
Q

Physical signs indicating anemia

A

tachycardia, tachypnea, dyspnea, conjunctiva, lymph nodes and size of liver/spleen

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108
Q

Family history indications for anemia

A

gallstones, jaundice, splenomagaly, splenectomy, cholysystectomy

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109
Q

Distribution of iron in the body

A 
65% Hb
6% Mb
25% Ferritin
small amt transferrin
<1% in enzymes
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110
Q

Major causes of iron deficiency

A

decrease in iron uptake, increased iron loss, increase in iron requirements (infancy, pregnancy, lactation, adolescence)

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111
Q

Labs for iron deficient anemia

A

decrease in O2 carrying capacity (HGB, HCT, decrease in production (low reticulocyte count, RI)
later…
CBC will show microcytosis, low MCV, hypochromia

addtl tests may show decreased serum iron, increased total iron binding capacity, low serum ferritin, increased erythrocyte protoporphyria

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112
Q

Effects of over accumulation of iron

A

damage to liver, heart, and endocrine glands

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113
Q

Treatments for over accumulation of iron (hemochromatosis)

A

(increased absorption)-therapeutic phlebotomy

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114
Q

Treatment for hemosiderosis (often from transfusions)

A

IF or sc/sq chelators (desferal). Some are now oral

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115
Q

lifespan of a platelet, number produced per day

A

7-10 days, 200 billion produced/day

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116
Q

lifespan of an erythrocyte, number produced per day

A

120 days, 175 billion produced/day

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117
Q

lifespan of neutrophil, number produced per day

A

7 hour half life, 70 billion produced per day

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118
Q

Embryonic hemaptopoiesis occurs in the:_____. This ceases after ____months

A

yolk sac, 3

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119
Q

Fetal hematopoiesis takes place in the_____ between months __ and __

A

liver and spleen, 2, 7

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120
Q

By the time of birth, hematopoesis occurs in the:

A

bone marrow (entire skeleton active bone marrow)

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121
Q

hematopoiesis outside of the bone marrow after birth is abnormal and called

A

extramedullary hematopoiesis

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122
Q

Marrow space is encased by _____ bone, and interspersed by _____ bone lined by osteoblasts and osteoclasts

A

cortical, trabecular

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123
Q

Between trabecula is a network of vascular ______ with walls of _____ endothelial cells

A

sinusoids, leaky

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124
Q

ASYMMERTRIC CELL DIVISION

A

1 HSC daughter and 1 multipotent progenitor cell made after a HSC divides

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125
Q

Progenitor cells can be:

A

Multipotent, oligopotent, or lineage restricted

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126
Q

Multipotent progenitor cells

A

capable of differentiating to all lymphoid and myeloid lineages

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127
Q

Oligopotent progenitor cells

A

common myeloid progenitor cells and common lymphoid progenitor cells

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128
Q

What type of progenitor cell comes after Oligopotent progenitor cells?

A

Lineage-restricted progenitor cells

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129
Q

One lineage-restricted progenitor cell, in this case a blast forming unit-erythroid (BFU-E), gives rise to around _____ mature red blood cells

A

2000

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130
Q

________ – BLUE on Wright stain

_______ – PINK on Wright stain

A

ribosomes

hemoglobin

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131
Q

Erythropoesis species order

A

Pronormoblast, Basophilic Normoblast, Polychromatophilic Normoblast, Orthochromic Normoblast, Reticulocyte, Mature RBC

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132
Q

Timespan of erythropoesis

A

2 – 7 days for pronormoblast-orthochromic normoblast
1 day to extrude the nucleus from orthochromic normoblast
Reticulocyte matures 2 – 3 days in bone marrow before it is released into the peripheral blood

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133
Q

Rate of _________ determines the hemoglobin level of normal individuals
Initiated by ________, a hormone produced by the kidneys

A

erythropoiesis, erythropoietin

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134
Q

Functions of erythropoetin

A
  • Activate stem cells of bone marrow to differentiate into pronormoblasts
  • Increases rate of mitosis and maturation process
  • Increases rate of hemoglobin production
  • Causes increased rate of reticulocyte release into peripheral
    blood
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135
Q

Granulocyte types are distinguished from each other by the appearance of their:

A

secondary (specific) cytoplasmic granules

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136
Q

staining of secondary granules in granulocytes

A

Neutrophils: pink to rose-violet granules
Eosinophils: reddish-orange granules
Basophils: dark purple granules

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137
Q

Auer rods are only seen in _______ under abnormal conditions

A

myeloblasts

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138
Q

Granulopoiesis order

A

Myeloblast>Promyelocyte> Myelocyte > Metamyelocyte > Band > Segmented Granulocyte

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139
Q

Main cytokine initiating neutrophil production:

A

Granulocyte-colony stimulating factor (G-CSF)

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140
Q

Myeloblasts, promyelocytes, and myelocytes undergo cell division (mitotic pool) time frame

A

(4 – 5 cell divisions, 3-6 days spent in this pool)

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141
Q

Do Metamyelocytes, bands, and segs divide? How long do they spend in the maturation and storage pools?

A

5-7 days in maturation and storage pools; 3 times as many cells as in the mitotic pool

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142
Q

Where are neutrophils located once they enter the bloodstream?

A

50% circulate freely (circulating pool);
50% adhere to walls of blood vessels
(marginal pool)
Continually move between pools

143
Q

Average time a neutrophil spends in peripheral blood is:

A

10 hours

144
Q

Neutrophil Granulocytes contain____ and have prominent _____ activity

A

destructive enzymes, most famously myeloperoxidase, used to destroy infectious organisms, most commonly bacteria

phagocytic

145
Q

Mature segmented neutrophil granulocytes are also known as _____, for ‘polymorphonuclear leukocytes’ (due to their highly and variably segmented nuclei), and also by the abbreviated terms ‘segs’ and ‘polys.’

A

PMNs

146
Q

Mature eosinophils usually have _ nuclear lobes

Lifespan of around ____ days

A

2, 8-12 days

147
Q

Eosinophil Granulocytes contain

A

destructive enzymes, which are used to fight organisms too big to phagocytose (fungi, protozoans, parasites)
minimal phagocytosis

148
Q

In addition to parasite/fungus/protozoa, Eosinophil Granulocytes modulate

A

mast cell activity in hypersensitivity response/allergic disease

149
Q

Main cytokine initiating eosinophil production:

A

Interleukin-5 (IL-5)

150
Q

Both ______ and ______ are involved in hypersensitivity/allergic processes, and in innate defenses against microbes

A

Basophil Granulocytes and Mast Cells

151
Q

Main cytokine initiating basophil production is:

A

IL3

152
Q

Main cytokine initiating mast cell production is

A

Stem Cell Factor (SCF) for mast cells

153
Q

MATURE BASOPHIL appearance

A
  • Prominent large dark blue (basophilic) cytoplasmic granules, which obscure the nucleus
  • Multilobular but non-segmented nucleus
  • Found in blood and marrow at low levels
154
Q

Early T-lymphoid progenitor cells migrate to the ____, the site of:

A

thymus

T cell maturation

155
Q

Where does B cell maturation occur

A

in the marrow

156
Q

Monopoiesis is initiated by

A

M-CSF (monocyte-colony stimulating factor)

157
Q

Mature monocytes circulate in peripheral blood an average of __ days, before entering tissue to become _______
Some mature monocytes and macrophages reside in the ______

A

20
macrophages
marrow

158
Q

Monopoiesis order

A

monoblast > Promonocyte > Monocyte

159
Q

A million platelets are produced every ____ (around _______ a day), and can be increased by up to ___ fold

A

second, 100 trillion, 20 fold

160
Q

Megakaryopoiesis is Initiated by action of the cytokine ________ on an megakaryocyte/erythroid progenitor cell

A

thrombopoietin (TPO)

161
Q

Are megakaryoblasts abundant?

A

No, rare! though they account for only 0.05% of nucleated marrow cells

162
Q

Description of megakaryocytes

A

Very large cells with highly folded, multilobular nuclei and abundant finely granular cytoplasm. They possess pseudopods, which they insert in bone marrow sinuses to allow direct shedding of platelets into the circulation

163
Q

ITEMS COMMONLY ASSESSED ON BM EVALUATION:

ON THE ASPIRATE SMEARS

A
  • Marrow Differential
  • Cell Morphology
  • Iron Content
164
Q

ITEMS COMMONLY ASSESSED ON BM EVALUATION:

ON THE CORE BIOPSY

A
  • Marrow Cellularity - Myeloid:Erythroid Ratio – normally 2:1 to 4:1. - Megakaryocyte Frequency
  • Focal Findings
165
Q

MARROW CELLULARITY rough estimation

A

ROUGH RULE IS THAT MARROW CELLULARITY (AS A PERCENTAGE) SHOULD APPROXIMATELY BE 100 – AGE

166
Q

Why could marrow be HYPERCELLULAR?

A
  • increased proliferation of one more lineages; usually due to increased signalling by HGFs (e.g. secondary erythroid hyperplasia in smokers)
  • Could be neoplastic due to a neoplasm of hematopoietic cells
167
Q

Hypocellular marrow is categorized as either:

A

hypocellular (cellularity decreased but some marrow cells present) or aplastic (marrow cells essentially absent)

168
Q

Possible causes or hypocellular marrow

A
  • Possible causes include: - autoimmune attack on marrow cells – -viral attack on marrow cells - -hematopoietic neoplasms - –malnourished state (rare)
169
Q

Fetal hemoglobin is comprised of

A

a2y2

170
Q

hemoglobin A2 is made of

A

a2d2

171
Q

When substrate binding alters the binding affinity for additional substrate

A

allosteric binding

172
Q

When oxygen is low and does not occupy any of the 4 sites, Hb is in the __ conformation

A

T (taut)

173
Q

The phenomenon of binding to substrate leading to increased affinity for additional substrate

A

positive cooperativity

174
Q

Because of ______, the oxygen dissociation curve for Hb is sigmoidal

A

cooperativity

175
Q

P50

values for Hb and Mb

A

the partial pressure of oxygen at which the oxygen carrying protein is 50% saturated
Hb: 27mmHg
Mb: 2.75mmHg

176
Q

What is the PPO2 in the tissues?

A

40mmHg

177
Q

numbers defining shape of Hb O2 dissociation curve

A 
10-10
30-60
60-90
40-75
(PP-%saturation)
178
Q

Factors causing a RIGHT shift of Hb curve

A

decreased pH, increased temp, increased DPG

179
Q

Factors causing a LEFT shift of the Hb curve

A

increased pH, decreased temp, decreased DPG

180
Q

How does 2,3-BPG (DPG) alter Hb’s O2 affinity?

A

it is a byproduct of anaerobic glycolysis pathway that binds to deO2 Hb and stabilizes it in the T configuration leading to a decreased affinity of the Hb for O2

181
Q

Embryos have 3 hemoglobins present between 4 and 14 weeks:

Describe O2 affinity

A 
Gower 1 (Z2E2), Gower 2 (a2E2), and Portland (Z2y2). 
higher O2 affinity than HbA
182
Q

Why do fetal cells have a higher affinity for O2?

A

because HbF binds 2,3-BPG poorly, stabilizing Hb in the R state, shifting the curve to the left

183
Q

At birth, there is _____% HbF and ___% HbA

A

65-95, 20

184
Q

Who might have elevated fetal hemoglobin levels?

A

renature babies, infants of mothers with diabetes, hemolytic anemias, myelodysplasia, leukemia

185
Q

HbA2 comprises __% of adult Hb

A

2%

186
Q

______ is generally found in people with high-affinity hemoglobin variants because of

A

erythrocytosis, increased EPO release

187
Q

____ is generally found in people with low-affinity hemoglobin

A

cyanosis

188
Q

To bind oxygen, hemoglobin needs to be in the __ form. If iron is in the ____ form, _______ results

A

reduced, ferrous +2 form

ferric +3, methemoglobin

189
Q

Methemoglobinemia can occur because of too much ______ production or because of decreased methemoglobin reduction.

A

methemoglobin

** may be acquired or genetic

190
Q

How can methemoglobinemia be acquired?

A

oxidation of heme by free radicals, exposure to chemicals (benzocaine), nitrate contaminated water

191
Q

Genetically cause methemoglobinemia is most often a _______ deficiency

A

cytochrome b5 reductase

192
Q

Methemoglobinemeia causes a ___ shift of the Hb curve

A

left

193
Q

When might you suspect methemoglobinemia?

A

Cyanotic but normal arterial blood gas. Blood doesn’t turn red when exposed to O2

194
Q

_____ is given intravenously to patients with methemoglobinemia and acts as an electron acceptor

A

methylene blue

195
Q

CO ____ Hb’s affinity for O2

A

increases

196
Q

Symptoms of CO poisoning

A

headache, malaise, nausea, dizziness,

High levels: seizures, coma, MI

197
Q

Treatment for CO poisoning

A

100% O2 and hyperbaric O2

198
Q

pulse oximetry maybe incorrect because of

A

incorrectly placed probes, nail polish, dark skin, shivering, anemia, shock, abnormal hemoglobins

199
Q

What can be used to detect carboxyhemoglobin and methemoglobin?

A

co-oximetry

200
Q

Cond’ts associated with anemia of chronic disease

A

chronic infections, chronic non-infectious inflammatory diseases, malignant diseases, Pb poisoning, renal insufficiency, endocrine disorders

201
Q

Lead inhibits:

A

attachment of the iron to the porphyrin ring so hemoglobin cannot be synthesized

202
Q

Signs and symptoms of renal insufficiency-caused anemia

A

fatigue, pallor, decresed exercise tolerance, dyspnea, tachypnea

203
Q

What type of anemia is generally seen with renal insufficiency?

A

normochromatic, normocytic with EPO deficiency

204
Q

Symptoms of adrenal insufficiency

A

nausea, vomiting, dehydration, weakness and circulatory collapse

205
Q

anemia with hypothyroidism

A

mostly normochromatic and normocytic. Decreased retics and RI
(may be microcytic or macrocytic)

206
Q

anemia with hyperthyroidism

A

usually normocytic, Decreased retics and RI

may be microcytic

207
Q

anemia with adrenal insufficiency

A

mild normocytic anemia, Decreased retics and RI

208
Q

Treatment of anemia from chronic infection/inflammation/malignancy

A

treatment of underlying disease to decrease cytokines and interleukins. For some diseases treat with EPO

209
Q

Treatment for anemia caused by lead intoxication

A

chelation of Pb with administration of specific agents to relieve intoxication

210
Q

Treatment for renal insufficiency anemia

A

EPO

211
Q

Treatment for anemia caused by endocrine disorder

A

hormone replacement

212
Q

EPO should only be used when:

A

1) Absolute deficiency

2) decrease out of proportion to the hematocrit level and where a response has been documented

213
Q

RBC transfusions should be used when

A

the severity of anemia has potential for cardiovascular decompensation

214
Q

sideroblastic anemia

A

Impaired production of protoporphyrin or incorporation of iron. Iron in mitochondria surround nucleus

215
Q

Both B12 and folate deficiency causes ______anemia because they arrest in S phase, undergo destruction

A

megloblastic

216
Q

Although anemia is most common, B12 and folate deficiency causes ____ and _____ in some patients

A

neutropenia, thrombocytopenia

217
Q

Folate is absorbed in the _____ and B12 is absorbed in the ____

A

jejumum

terminal ilium

218
Q

Folate is absorbed in the ____, then it is ____ and stored in the ____

A

jejunum, methylated, liver

219
Q

Main causes of B12 deficiency

A

autoimmune disease (anti-IF), intrinsic factor deficiency, malabsorption, defective transport/storage, metabolic defect

220
Q

The main cause of folate deficiency is:

But other causes include:

A

dietary insufficiency

malabsorption, drugs and toxins, folate metabolism errors, increased demands, increased loss or metabolism

221
Q

Increased demands for folate include:

A

hemolysis, pregnancy, psoriasis, myeloproliferative disorders

222
Q

Is folate or B12 deficiency more likely associated with alcohol abuse and poor nutrition?

A

folate

223
Q

Which deficiency develops more quickly-folate or B12?

A

Folate deficiency is rapid (weeks-months)

B12 is slow (years-usually prob. with malabsorption)

224
Q

Hematologic changes of B12 and Folate deficiency

A
  • megaloblastic precursors (red and white)
  • erythroid hyperplasia
  • peripheral bloos macrocytosis, ovalocytes, hypersegmented neutrophils
  • increased bill
  • RI<1
225
Q

severe hematologic changes seen in B12 and folate anemia

A

poikilocytes, fragmentation, neutropenia, thrombocytopenia

226
Q

Are neurological symptoms classic in folate or B12 deficiency?

A

B12, infrequent in folate

227
Q

Neuro features of B12 deficiency

A

sensory loss first
proprioception
ataxia, spasticity, gait disturbances, + babinski
cognitive and emotional changes

228
Q

If a person has neurological symptoms from a B12 deficiency, do they always have anemia? Are neuro symptoms reversible

A

Nope, 20% don’t.

Neuro symptoms may be irreversible

229
Q

Lab tests for both B12 and folate deficiency

A

increased MCV, low RI and retic count, increased unconjugated bill, increased LDH

230
Q

Tests that would show B12 change but normal folate

A

serum methylmalonic acid, serum 2-methyl citric acid would be increased in B12 deficiency but normal for folate

231
Q

95% of B12 (cobalamin) deficiency arise from ____ causes

A

GI (particularly absorption of B12)

232
Q

The _____ test can be used to test the absorption of B12

A

schilling

233
Q

If it is unclear whether a patient has B12 or folate deficiency, give ____ first. Why?

A

B12. Giving folate to a B12 deficient patient induced neurological symptoms

234
Q

Management for cobalamin deficiency

A

1mg injections weekly for a few weeks, then monthly. If malabsorption not an issue, 2ug orally 2x a day

235
Q

Management for folate deficiency

A

1mg/day orally or parenterally

236
Q

Does anemia reverse quickly for B12 or folate deficiency?

A 
both! Retics after 2-3 days, peak 7-10 days
HGB increases in 1-2 weeks
WBC increase in 1 week
MCV lower in weeks-months
blood count normal in 8 weeks
237
Q

When B12 is ingested, it binds to a protein carrier _____. It is absorbed in the ____. It binds ___ and it stored in the ____

A

intrinsic factor (IF)
terminal ilium
TcII
liver

238
Q

The most common cause of B12 deficiency is

A

pernicious anemia due to autoimmune destruction of IF-producing parietal cells

239
Q

Cytopenias are dues to either

A

increased destruction or decreased production (or in rare case, both)

240
Q

In cytopenias due to increased destruction, what would you expect to see? Why?

A

compensatory hyperplasia of one or more lineage

241
Q

In cytopenias due to decreased production, what will you see?

A

marrow will not show a compensatory hyperplasia

242
Q

PETECHIAE

A

microhemorrhages within skin

indicative of thrombocytopenia

243
Q

What might a giant platelet indicate?

A

usually indicates increased thrombopoeisis

244
Q

‘leukemoid reaction’

A

usually secondary to severe infectious or inflammatory disease

245
Q

Findings characteristic of leukemia reaction over CML

A
  • Signs of infectious or inflammatory disease
  • Toxic-appearing neutrophils on blood smear (toxic granules, Dohle bodies)
  • Normal basophils levels in blood
  • Normal appearing megakaryocytes in marrow
246
Q

Findings characteristic of CML over a leukemia reaction

A
  • No symptoms
  • Enlarged spleen and/or liver
  • Neutrophils do not have toxic features on blood smear
  • Absolute basophilia
  • Abnormal-appearing megakaryocytes in marrow
247
Q

Myelophthisis

A

replacement of bone marrow by fibrosis, tumors or granulomas.

248
Q

pancytopenia

A

reduction in the number of RBCs, WBCs, and platelets.

249
Q

CBC findings of iron deficient anemia (IDA)

A

Decreased: RBC, HGB, HCT, MCV, MCH, MCHC
Increased: RDW

250
Q

Morphological features seen on the peripheral blood smear in cases of Iron Deficient Anemia

A

decreased absolute RBC number, RBCs are mycrocytic and hypochromatic, large central pallor

251
Q

Typical CBC findings of megaloblastic anemia

A

Decreased: RBC, HGB, and HCT
Increased: MCV, MCH, RDW

252
Q

Morphological features seen on peripheral blood smear for megaloblastic anemia

A

Macrocytic RBCs (but decreased in number), large central pallor, hyper segmented neutrophils

253
Q

Most common causes of megaloblastic anemia

A

B12 and folate deficiency

254
Q

Etiology and pathogenesis of megaloblastic anemia

A

Results from inhibition of DNA synthesis, so cells arrest in S phase and continue to grow without division–> macrocytosis

255
Q

Hemolysis

A

decrease in red cell survival or an increase in turnover beyond the normal range

256
Q

Generally, marrow reticulocytes develop in the marrow for __-___ days, but during stress or hemolysis, maturation may decrease to __-__ days

A

10-14 days

5-7 days

257
Q

Most RBC death occurs in the ____, while 10% occurs _____

A

spleen (extravascular-macrophages of reticuloendothelieal system)
intravascual

258
Q

Cellular processes associated with normal turnover of RBCs

A
  • decrease in red cell enzyme with age
  • oxidative injury over time
  • change in calcium balance
  • changes in membrane carbohydrate and surface constituents
  • antibodies to membrane constituents
259
Q

Intravascular hemolysis

A

Hb released into circulation, tetramer dissociates into dimers which bind haptoglobin. This complex is removed from circulation by liver. Iron can be oxidized to form methemaglobin. Dissociation of glob in releases me theme, which binds to albumin. These are taken up by hepatic parenchymal cells and converted into bilirubin

260
Q

Extravascular hemolysis

A

RBC ingested by macrophages of reticuloendothelieal system. Heme separated from glob in, iron removed, stured in ferritin. Porphyrin ring –> bilirubin (lipid soluble), conjugated with glucuronic acid by P450 (H2O soluble), expelled as fecal urobilogen

261
Q

Morphology of hemolytic anemia

A

traditionally spherocytes

262
Q

Bilirubin levels in hemolytic anemia

A

total bili increased (most is unconjugated)

263
Q

Housekeeping enzymes LDH and SGOT in hemolytic anemia

A

increased because there is RBC destruction

264
Q

Hemoglobin lab results in hemolytic anemia

A

increased with intravascular hemolysis

265
Q

Haptoglobin lab results in hemolytic anemia

A

very low levels in intravascular hemolysis

266
Q

Methemalbumin, metheme in hemolytic anemia

A

increased with intravascular hemolysis

267
Q

Hereditary spherocytosis

A

familial hereditary disorder characterized by anemia, intermittent jaundice, splenomegaly, and responsiveness to removal of spleen.
Multiple molecular bnormalities, spectrin is most common. Hallmark = loss of membrane=spherocyte, osmotic fragility

268
Q

clinical features of Hereditary spherocytosis

A

1:5,000, anemia, jaundice, variable onset, 75% AD, 25% AR.

Presenting complications: hyperhemolysis, aplastic crisis

269
Q

laboratory findings of Hereditary spherocytosis

A

variation in HCT, HGB (mild to severe anemia possible)
Increased RI, MCHC
Decreased MCV
spherocytes
unconjugated hyperbilirubinemia, osmotic fragility

270
Q

What problem with Hereditary spherocytosis might be observable on an Xray?

A

bilirubin stones (cholelithiasis, cholecystitis)

271
Q

G6PD deficiency

A

X linked recessive, wide variety of AA substitutions =variety of enzyme activities
Worldwide, highest distribution in S. Europe, Africa, S. Chins, India, SEA—>relationship to malaria

272
Q

Pathophysiology of G6PD deficiency

A

Loss of G6PD activity=inability to make G-SH (oxidant stress), oxidation of hemoglobin, denatured glob in attaches to membrane (heinz bodies) + oxidation spectrin–>decreased deformability of RBC=trapped in spleen–>extravascular hemolysis

273
Q

Clinical features of G6PD deficiency

A
  • intermittent episodes of acute anemia, hyperbiliruinemia, hemolysis, reticulocytosis,
  • associated with oxidant stress: infection, drug, ingestion of certain foods.
  • Chronic hemolytic anemia
  • neonatal hyperbili
  • many drugs contraindicated
274
Q

Morphological features of G6PD deficiency

A

no specific morphological features, may see microspherocytes, “blister” cells or “bite” cells

275
Q

Autoimmune hemolytic anemia

A

Abs to universal RBC antigens, causes either intravascular or extravascular hemolysis. Two types: warm (IgG) and cold (IgM)

276
Q

cold autoimmune hemolytic anemia

A

Mostly IgM, one specific type of IgG, aggressively activate complement through C5-9 complex—> intravascular hemolysis

277
Q

Warm autoimmune hemolytic anemia

A

Usually IgG with a high affinity to the RBC membrane with very little and incomplete complement activation—> Extravascular hemolysis

278
Q

Direct Coombs test

A

blood sample from a pt. with autoimmune mediated hemolytic anemia has Abs attached to the surface antigens when extracted from the pt. Incubated with Coomb’s reagent which forms links between the human Abs stuck to the surface.–> perceptible agglutination

279
Q

Indirect Coomb’s test

A

Pt’s serum (containting Abs) is added to donor blood. Abs stick to Donor’s blood antigens and form complexes, anti-human Ig’s (coomb’s Abs) are added. Agglutination of RBCs occurs when coomb’s Abs bridge the Abs stuck to RBCs

280
Q

Clinical findings of autoimmune hemolytic anemia

A
  • acute or chronic onset of anemia with pallor, jaundice, and dark urine
  • Decrease in HGB, increased retics, increased bili
  • Presence of DAT (direct Ab test-coomb’s test)
  • may have spherocytes (also can have teardrop or bite cells
281
Q

What does the spleen do?

A

Clearance of intravascular particles

adaptive immune response: origin of IgM agglutinins, esp. for encapsulated organisms

282
Q

Main risk of splenectomy

A 
overwhelming sepsis (particular S. pneumonia)
Risks highest in kids <5 but also adults (220X risk of sepsis of normal pop)
283
Q

Management of splenectomy

A

1) vaccinate against H. influenza b, S pneumonia and meningiococcal before splenectomy
2) Prophylactic antibiotics
3) See Dr immediately for fever >38.5 (lifelong)

284
Q

What globin genes are clustered on chromosome 11?

A

epsilon, gamma, delta, beta

285
Q

What globin genes are clustered on chromosome 16?

A

Zeta2, Zeta1, Alpha2, Alpha1

286
Q

Structural variant seen in Hb C

A

B6 witch of Glu–> Lys, crystallization

287
Q

Sickle cell disease

A

Occurs with 2 abnormal B genes. Many variations but common ones are HbS, Hb C, Hb E, B+ thalassemia, B0 thalassemia

288
Q

Sickle cell anemia is really a problem with the vasculature. Explain

A

vasculature gets damaged as a response to “sticking” of RBCs (WBCs). The sicking of a RBC is reversible, but the membrane damage (especially to post-capillary venues) is irreversible. Hypoxia, oxidant injury, apoptosis, abnormal vasoregulation, Inflammation of endothelial tissue causes remodeling and narrowing

289
Q

Sickle cell trait

A

One normal B chain, One Bsickle chain. HbA> HbS , normal CSC, rare splenic infarct in white males at high altitude

290
Q

Reticulocyte count is strongly elevated in :

and slightly elevated in:

A

HbSS, Hb SB0 thalassemia

Hb SC disease, Hb SB+ thalassemia

291
Q

Direct damage to endothelium in sickle cell anemia

A

up regulation of adhesion molecules, exuberant repair mechanisms, apoptosis

292
Q

The severity of sickle cell anemia is strongly correlated with

A

RBC adhesion

293
Q

What was correlated with diminished survival in sickle cell patients in the Cooperative Natl. history study?

A

Constitutive elevation of WBCs

294
Q

Sickle cell solubility testing

A

detects the sickle hemoglobin in concentrations as low as 8-20% so it cannot distinguish sickle cell trait from the disease or the type of disease

295
Q

What is the most accurate way to diagnose hemoglominophaties?

A

hemoglobin separation on a gel, can also use isoelectric focusing, HPLC

296
Q

Characteristics of a Sickle cell

A

sickle shaped, rigid, sticky (even when not sickled), lived for increased Retics

297
Q

Which hemoglobinopathies might microcytosis and hypochromia suggest?

A

thalassemsia

298
Q

Which hemoglobinopathies might target cells suggest?

A

thalassemsi, Hemoglobin C (if crystals are present)

299
Q

What is a preventable cause of death in infants with Sickle cell disease

A

splenic damage, trapping of RBCs

overwhelming bacterial infections, preventable with prophylactic penicillin

300
Q

Sickle cell and stroke

A

large vessel ischemic stroke:intimal hyperplasia, proliferation of fibroblasts and smooth muscle, slitting of internal elastic lamina
Adults at risk for CNS hemorrhage (same as above-weakened vessels)

301
Q

Sickle lung disease

A

progressive obliteration of pulmonary vasculature: Begins with restrictive changes, frequently leads to pulmonary hypertension, leading cause of death in adults with sickle cell disease/ Loss of vasoregulation, decreased exhaled NO, administration of arginine may improve plum. hypertension

302
Q

Sickle nephropathy in HbSS

A

Initial hyperfiltration and enlarged glomeruli

10-15% HbSS patients, sidled cells adhere in afferent/efferent arterioles, RBC fragments deposited in basement membrane

303
Q

Sickle Retinopathy in HbSS

A

11-45% pts. causes retinal detachment, hemorrhage, blindness

304
Q

What bone/muscular problems can be associated with HbSS?

A

Avascular necrosis of femoral/humeral heads, leg ulcers, chronic pain syndrome

305
Q

Acute sickle cell event

A

endothelial injury (hypoxia, cytokines)-vascular leak, endothelial retraction from increased RBC adhesion in post-capillary venues, vascular disruption and inflammation, vasoconstriction (decreased NO)

306
Q

Treatment of sickle cell pain

A

Goal: get endothelium less sticky. Antibiotics for fever, pain control, O2 and hydration

307
Q

Acute chest syndrome/multiorgan failure in HbSS

A

rapid onset of low O2, chest pain, fever, worsened anemia.

  • kidney, liver failure possible
  • rapid transfusion therapy is critical
308
Q

Treatment of hemoglobinopathies

A

transfusion, induction of HbF (hydroxyurea, butyrate), bone marrow transplant

309
Q

Why aren’t bone marrow transplants done on all sickle cell patients?

A

Only 17% of eligible recipients have suitable HLA-matched sibling donor

310
Q

How does transfusion therapy help HbSS? What are the risks?

A

dilutes sickled RBCs, decreases stroke risk and abnormal flow to cerebral vessels. Risks: infection, alloimmunization, iron overload

311
Q

hydroxyurea characteristics

A

induces fetal hemoglobin, reduces sickle hemoglobin polymerization, increases MCV, less hemolysis with improved anemia, less RBC adhesion, increased NO generation, decreases vascular injury. Also decreases WBC

312
Q

Clinical effects of hydroxyurea

A
  • reduction in acute pain events and acute chest syndrome
  • Improvement in mortality
  • no evidence of reduction in chronic organ injury
313
Q

Pathophysiology of Thalassemias

A

Decreased synthesis of α- or B-globin chains
leads to imbalance in chains
- free excess chains bind to RBC membrane
- membrane oxidative injury
- increased membrane rigidity
- decreased membrane stability(B-thalassemia)

314
Q

Manifestations of Thalassemias

A 
Underproduction of normal Hb
Small RBC: (↓MCV) 
 ↓MCH, MCHC, normal RDW
Increased RBC fragility → ↓ RBC survival
Increased Retics
Increased release of RBC contents:
 (unconjugated) bilirubin, LDH, AST, 
splenomegaly
Bilirubin gallstones
Anemia MAY be present
315
Q

Consequence of iron overload:

A

Growth delay

Endocrinopathies

316
Q

Severities of a-thalassemia gene deletions

A

1-gene deletion = Clinically silent (MCV normal)

2-gene deletion= Microcytosis ± anemia (nl/low)

3-gene deletion= Microcytic anemia (Hb H disease) (low)

4-gene deletion= Fetal hydrops (intrauterine death)

317
Q

Which a-thalassemsis are transfusion dependent?

A

sometimes HbH

318
Q

HbH disease symptoms

A

hypochromia, microcytic anemia, increased retics, splenomegaly, cholelithiasis, iron overload, infection, anemia exacerbated by vitamin deficiency

319
Q

Can electrophoresis be used to diagnoses a-thalssemia?

A

A-globin is part of every hemoglobin, so there is no difference in the relative quantities. Must have microcytosis w/o iron deficiency and normal electrophoresis

320
Q

Hb E disease

A

(GlyLys at 26) – unstable mRNA so less production

321
Q

β-thalassemia major (Cooley’s anemia)vs

β-thalassemia trait:

A

Both genes for beta chains are missing vs

one gene missing

322
Q

β Thalassemia minor (trait): Clinical Syndromes

A

Minor or no anemia (↓ MCV ↓MCH ↑RBC)

323
Q

β Thalassemia intermedia

A

Moderately severe anemia
May have splenomegaly
Growth delay, bone deformities
Increased iron absorption with hemosiderosis

324
Q

Thalassemia major (Cooley’s anemia or HbEβo)

A

severe transfusion-dependent anemia
splenomegaly
Growth delay, endocrine failure
dense skull/marrow expansion with osteopenia
Increased iron absorption with hemosiderosis

325
Q

Treatment of βoThalassemia (Cooley’s Anemia)

A

Transfusion Therapy

Induction of Fetal Hemoglobin
	~ hydroxyurea therapy
	~ butyrate therapy
Bone Marrow Transplant
	~ standard of care in βothalassemia (Cooley’s anemia
326
Q

Survival of βoThalassemia (Cooley’s Anemia)

  • W/o transfusions
  • Trasfusions with RBCs
  • Transfusions + chelation
A

-W/o transfusions: >20 years

327
Q

Should iron supplements be given to those with thalassemias?

A

Iron supplementation should NOT be given unless clearly iron deficient, microcytosis is not due to low iron stores

328
Q

Possible causes of microcytic anemia

A
  1. Iron deficiency
  2. Thalassemia syndromes
  3. Severe lead poisoning (children)
  4. Chronic disease/inflammation
329
Q

What is involved in initial screening for safety of blood transfusions?

A

donor interview, review of high risk behavior, abbreviated physical appearance, HCT, skin prep to reduce infection

330
Q

What infectious diseases are screened in transfusion blood samples?

A

Syphilis, Hep A,B, C, HIV 1, 2, HTLV I, II, WNV, CMV

331
Q

The fact that there are alterations in antigens on blood cells that do not result in functional differences is an example of

A

alloantigens

332
Q

When do people develop Abs to blood types other than their own?

A

By 1 year

333
Q

Pre transfusion testing of donor’s blood

A

ABO, Rh(D), antibody screen

334
Q

Crossmatch

A

add recipients’s serum to donor cells and look for agglutination at RT. Add coomb’s to look for IgG complement, takes about 45 min

335
Q

In an urgent situation, what kind of blood is used?

A

O, Rh(D) negative

336
Q

Acute hemolytic transfusion reaction (pathophys, symptoms, mgmt)

A

Hemolysis of transfused cells iwht activation of clotting, inflammatory mediators, and renal failure.

fever, chills, nausea, chest pain, back pain, pain at transfusion site, hypotension, dyspnea, dark urine

risk is low, mortality hush. Stop transfusion, IV fluids and diuretics, heparin

337
Q

Delayed hemolytic transfusion reaction (pathophys, symptoms, mgmt)

A

formation of alloantibodies post-transfusion, destruction of red cells by extravascular hemolysis

fever, jaundice, anemia

Supportive care and detection, definition, and documentation for future care

338
Q

(Transfusion reaction) Febrile reactions (pathophys, symptoms, mgmt)

A

leukoagglutins in recipient cytokines or other biologically active compounds

Fever, maybe chills

Supportive, consider leukocyte poor products for future

339
Q

(Transfusion) allergic reactions (pathophys, symptoms, mgmt)

A

Most causes not identified

itching, hives, chills/fever, in severe cases anaphylaxis, dyspnea, pulmonary edema

340
Q

Transfusion related acute lung injury (pathophys, symptoms, mgmt)

A

w/i 4 hrs after transfusion. Pt: infection surgery, cytokine therapy
Blood factors:lipids, Abs, cytokines
Two factors interact and result in lung injury

Tachypnea, dyspnea, hypoxia, diffuse interstitial marlins with normal cardio exam

Supportive, consider yonder products

341
Q

Transfusion related: Dilutional coagulopathy (pathophys, symptoms, mgmt)

A

massive blood loss

Bleeding

replacement of clotting factors or platelets

342
Q

Bacterial contamination of blood transfusion (pathophys, symptoms, mgmt)

A

bacteria/endotoxin

chills, high fever, hypotension, symptoms of sepsis or endotoxemia

Stop transfusion , ID organism, antibiotics and supportive care

343
Q

Graft vs. host disease (pathophys, symptoms, mgmt)

A

lymphocytes from donor transfused into immunoincompetent host

Can involve skin, liver, GI tract, bone marrow

Preventative management

344
Q

Iron overload in transfusion patients (pathophys, symptoms, mgmt)

A

increased iron, no way to excrete

dysfunctional organ symptoms

iron chelators: deferoxamine, Exjade

345
Q

What is Toll? Why is it important?

A

single gene that controls innate immunity in a fly. Toll knock outs die from invaders. The flies only have innate immunity, no adaptive immunity

346
Q

How many toll like receptors do humans have?

A

10

347
Q

Are toll-like receptors (TLRs) on the outside or inside of a cell?

A

TLRs can be on the inside or outside to recognize foreign threats (dsRNA or cell wall, respectively)

348
Q

Toleragen

A

antigen that does not create an immune response

349
Q

immunogen vs antigen

A

antigen: anything that reacts with the immune system
immunogen: known to cause immune response.
EX: flu vaccine makers want the best immunogen of all of their possible antigens.

350
Q

“Crossing over” of lymph and blood occurs in the:

A

post capillary venules

351
Q

allotype

A

genetically determined difference in molecular structure of an antibody between members of the same species (generally a 1-2 amino acid substitution of the constant heavy or light chain). No biological significance

352
Q

Isotype

A

present in all members of a species, the class of an antibody heavy or light chain: IgM, IgG, IgA are all isotopes

353
Q

Idiotype

A

variation on an individual, unique differences in the antibody binding region (hyper variable region)

Blood and Lymph (3 decks)

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