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Week 1 Other Terms
Anemia and Hemolysis
97
Medical
Professional
08/18/2012

Additional Medical Flashcards

 


 

Cards

Term
what blood cell is:

anuclear
agranular
biconcave
7-8 microns diameter
area of central pallor should be about 1/3 of diameter
Definition
erythrocytes
Term
What blood cell is:

anuclear
granular
2-4 micron diamter
seals openings in vascular tree
Definition
thrombocyte
Term
What blood cell is:

round-irregular nucleus
6-15 micrometers
adaptive immunity
Definition
lymphocyte
Term
what blood cell is:

bead shaped nucleus
"cerebroform nucleus"
12-20 micrometers

3 functions:
phagocytes, antigen presenting cells, immune system regulator to produce cytokines
Definition
monocytes
Term
what blood cell is:

lobes nucleus (2-3)
cytoplasm full of large eosinophilic granules
12-15 microns

function:
IgE mediated reactions
anti-parasitic
full of histamine granules
Definition
eosinophil
Term
cytoplasm full of grandules
dark purple/blue

functions:
allergic reactions, anaphylaxis
Definition
basophils
Term
What blood cell is:

3-5 lobed nucleus
granular cytoplasm (small granules)
12-15 micron diameter

function:
innate immunity
eat and kill bacteria and fungi
Definition
Neutrophils
(PMN, bands, segs)
Term
Order the following developmental stages from myeloblasts to neutrophis/eosinophils/basophils.


metamyelocyte
promyeloctye
myelocyte
myeloblast
neutrophil/eosinophil/basophil
band
Definition
myeloblast
promyeloctye
myelocyte
metamyelocyte
band
neutrophil/eosinophil/basophil
Term
red marrow vs. yellow marrow
Definition
red marrow = actively producing blood cells. Transitions to yellow marrow over time, starting with extremities.

yellow marrow = inactive fat filled
Term
What type of cell is identified by the cell surface markers:

CD34
kit
Thy
no CD38
no Lin
Definition
stem cells
Term
Match which type of differentiation is driven by which factor.

CFU-Meg
G-CSF
GM-CSF
EPO

Erythrocyte, Monocyte, Granulocyte, and Thrombocyte differentiation
Definition
Erythrocyte differentiation -- EPO
Granulocyte differentiation -- G-CSF
Monocyte differentiation -- GM-CSF
Thrombocyte differentiation -- CFU-Meg
Term
What differentiated blood cells are still proliferative?
Definition
lymphocytes
Term
Which blood cells are granular and which are agranular?
Definition
Granular:
basophil, eosinophil, neutrophil (PMN, band, seg)

Agranular:
monocyte
lymphocyte
erythrocyte
Term
Which blood cells are anuclear?
segmented nucleus?
Definition
anuclear?
thrombocyte
erythrocyte

segmented?
neutrophil (PMN)

bean shaped?
monocyte
Term
List platelet differentiation precursors.
Definition
Megakaryoblast
Promegakaryocyte
Mature Megakaryocyte
Breaks off peices of cytoplasm --> platelet
Term
What are the 4 broad categories of pathology that effect marrow function?
Definition
iatrogenic : radiation, chemotheraputic agents

clonal hemopathies : AML, MDS

malignancies : lymphoma, myelomas, metastatic lesion

aplastic anemia
Term
What are the 4 causes of increased neutrophil counts?
Definition
1. G-CSF - increased differentiation
2. Neoplasms
3. Splenectomy - no destruction
4. Inflammatory states - increased margination causes increased production?
Term
Describe Heme synthesis
RDS and regulation!
Definition
Steps 1, 6-8 in mitochondria
Steps 2-5 in cytosol

RDS is step 1, ALA synthase.
In bone marrow: increased by Iron
In liver: increased by Barbituates and drugs, decreased by glucose and heme.
Vit B6 is a cofactor, and is most commonly depleted by isoniazid therapy.

key regulation and ALA synthase (1), ALA dehydratase (2), and Ferrochelatase (8)

enzymes:
1. ALA synthetase
2. ALA dehydratase
3. PB deaminase
4. Uroporphyrinogen III synthase
5. UP III decarboxylase
6. CP III oxidase
7. Protoporphyrin III oxidase
8. Ferrochelatase
Term
Hypoproliferative Anemia
Definition
corrected RBC count < 2%
Term
Microcytic anemia
Definition
MCV < 80
Term
Macrocytic anemia
Definition
MCV > 94
Term
Normocytic anemia
Definition
MCV 80-94
Term
3 pieces of evidence that bone marrow is in distress (and therefore causing anemia)
Definition
1. Decreased platelets / WBC

2. immature cells on peripheral smear, like nucleated RBCs

3. Tear drop RBCs on peripheral smear
Term
What stimulates EPO (erythropoietin) production?
Definition
hypoxemia
severe anemia
left-shifted oxygen binding curvbe
high altitude
Term
What is the formula for the corrected Retic count?
Why?

What happens in Polychromasia?
Definition
Corrected retic = Hct/45 * retic%

45 represents the normal hematocrit
The percentage is falsely elevated in anemia

** in Polychromasia, polychromatic RBCs (even less mature than retics) appear due to very brisk hemolytic anemia. Need to half the "corrected retic" to correct the retic
Term
What should the corrected retic be in response to anemia?
Definition
corrected retic should be over 3%, demonstrating effective erythropoesis.
Term
What are the defining levels of Anemia in Children, Men, Women and Pregnant Women?

vs. Fetal Hemoglobin?
Definition
Pregnant Women < 11 g/dL

Women < 12.5 g/dL

Men < 13.5 g/dL
They have testosterone to increase erythropoiesis and don't have cyclical bleeding.

Children < 11.5 g/dL
Their oxygen binding curves are more right-shifted, meaning they give oxygen up to tissues more easily (bc they have higher BPG from higher phosophorus). So oxygen delivery is sufficient at lower Hct.

Newborns have Hct around 18.5 g/dL.
Fetal Hemoglobin (2alpha, 2 gamma) has a left shifted oxygen binding curve, which stimulates erythropoeitin synthesis, which increases Hb, Hct, and RBC.
Term
MCHC in microcytic anemias vs in spherocytosis
Definition
MCHC is low in microcytic anemias
high in spherocytosis
Term
What is the Cori cycle?
Definition
RBCs have no mitochondria, so they get their ATP from anaerobic glycolysis, producing lactic acid. Lactic acid is converted into glucose in the liver by gluconeogenesis, sending glucose back to the RBCs.
Term
Cords of Billroth
Definition
In spleen where splenic macrophages phagocytose senescent RBCs. They degreade heme to unconjugated bilirubin.
Term
Glutathione
Definition
Anti-oxidant that neutralized hydrogen peroxide.
Term
Methemoglobin reductase pathway
Definition
in RBCs, this reductase pathway reduces iron (Fe3+, methemoglobin) to ferrous (Fe2+)so that heme can bind O2.
Term
Ferritin.

Where is it synthesized.
How is it regulated.
Definition
Synthesized in bone marrow macrophages. Stores iron in the bone marrow macrophages.

Directly correlates with serum iron levels.

Synthesis is increased in response to inflammation (due to interleukin 1 and TNF alpha). Inflammation makes your body want to store iron in macrophages to keep it away from the potential bacterial infection, since bacteria thrive off iron.

Levels decrease in iron deficiency, increase in anemia of chronic disease and iron overload disease.

Hemosiderin is the insoluble degradation product of ferritin.
Term
Serum iron.
What does it respresent?
What is the normal level?
Definition
Represents iron bound to transferrin.

Normal level is 100 microg/dL

Levels decrease in iron deficiency and anemia of chronic disease, increase in iron overload disease
Term
Serum TIBC (total Iron binding capacity)

What does it represent.
What is its normal level.
How does it related to ferritin.
Definition
essentially the same as the concentration of transferrin.

Normal level is 300 microg/dL.

Made in the Liver. Increased in response to low ferritin stores.
Term
What is iron saturation =
Definition
= (serum iron/ TIBC) * 100%

normally 33%
(100/300)
Term
What types of globin chains make up each of the following.

HbA
HbA2
HbF
Definition
HbA -- 2 alpha, 2 beta

HbA2 -- 2alpha, 2delta

HbF -- 2alpha, 2gamma
Term
How does iron get absorbed?
Definition
Duodenum, absorb 10% of iron from diet. Only absorb reduced form (ferrous Fe2+) as from red meat, not ferric Fe3+.

Gastric acid frees iron from food.
Ascorbic acid reduces Fe3+ to the absorbable Fe2+.

HFE (hemochromatosis) gene regulates (increases) transferrin endocytosis to absorb iron in intestinal cells.
Term
What antibodies can pass through the placenta?
Definition
Only IgG
Term
Typing vs Screening blood
Definition
Typing is direct and Screening is indirect. Both find the patients antibody status (blood type).

Typing uses whole blood from the patient and anti-AB antibodys on a slide, look for agglutination.

Screening uses only patients serum (containing antibodies) and a sample of donor RBC with a known antigen, then look for agglutination.
Term
What is FFP?
(a type of blood transfusion)

When do you use it?
Definition
Acellular. Coagulation factors plus fibrinogen. No RBC antigens, but still lots of antibody in the plasma that can react with a host.
This is not virally inactivated.

Use:
DIC
liver disease
dilutional coagulopathy
urgent warfarin reversal
Term
What is Cryoprecipitate?
(a type of blood transfusion)

When do you use it?
Definition
The cold insoluble remnant of thawed FFP.
Acellular. Full of FVIII, FXIII, fibrinogen, fibronectin, vWF.

Use:
hypofibrinogenemeia
FXIII deficiency
Term
Leukoreduction.

What is it?
Uses?
Definition
Removing WBCs from blood.

uses:

Reduces febrile transfusion reactions because its the WBCs that produce cytokines. Give WBCs is like giving a cytokine bolus.

Reduces viral transmission, like CMV.

Prevents HLA immunization of recipient (ie. will have reaction to this HLA type in future).


problems:

cost
Term
Irradiation (for blood transfusion).

What is it?
Uses?
Definition
Gamma irradiation. Prevent CD8 T cells in the blood from being able to proliferate. This reduces the shelf life of the blood and so is only done for people at risk of GVHD.

Uses:

Prevents graft versus host disease (GVHD), which is fatal in about 80% of cases. At risk patients:
- immunodeficiencies
- newborns (esp with erythrobastosis fetalis)
- recipients of intrauterine transfusions, hematopoietic stem cells, blood products from relatives, HLA-selected platelets (homogenous)

Problems:

Reduces shelf life of blood products because causes potassium to leak from the cells.
Term
Washing (for blood transfusion).

What is it?
Uses?
Definition
Removes plasma proteins and antibodies from blood products. Removes potassium that has leaked from platelet units.

Uses:

Neonatal alloimmune thrombocytopenia - Removes PLA-1 antibodies from maternal platelet donations.

Patients with IgA deficiency

Patients with history of anaphylactic reactions to blood products.

Problems:

Once washed, must be used within 24 hours or sterility will be lost.
Term
Volume reduction (for blood transfusion).

What is it?
Uses?
Definition
To transfuse a lot of platelets with very little plasma volume.

Use:
apheresis units with volume overloaded patients

problem:
up to half of the product is lost because this is done by centrifuging
neonatal and pediatric populations
Term
Phenotypic Matching (for blood transfusion).

What is it?
Uses?
Definition
Make sure donor RBCs are lacking the same minor antigens that the recipient is lacking: C, E, K

uses:
Sickle cell patients to prevent alloimmunization to minor antigens.
Term
Risks of Blood transfusions:

List Infectious diseases that can be transfused with blood transfusions and how we screen for them.
Definition
HBV - surface antigen test only bc uncommon in US, window of 40-60 days

HCV - Nucleic acid test bc common (1%), window of 10-30 days

HIV-1 - Nucleic acid test, window 9-10 days

HTLV1/11 (causes adult T-cell leukemia/lymphoma)

Syphilis

CMV (lives in lymphocytes, most common infection from transfusion) - we only screen some units and save them for immunosupressed patients


also consider:

West Nile Virus - minipool Nucleic acid test bc rare (test 100 at once)

Malaria - no effective screen, just history

Babesiosis - no effective screen, just history (tickborne protozoa replicates in RBCs)

vCJD - only history if have been to UK/Europe (same prion as mad cow, death within 7-38 months)
Term
Describe Folate absorption.
Definition
In jejunum. Comes in Fresh fruits/veges. Cooking denatures it. Yay vegans!

Intestinal conjugase converts digested polyglutamates to monoglutamates, which can then be absorbed in the jejunum.

Need 50 microg/day, diet usually 700 but only absorb 10%. Liver stores a 3-4 month supply.

Absorption inhbited by:
- alcohol (and release from liver)
- OCPs
- phenytoin (inhibits intestinal conjugase)
- Celiac disease
- Bacterial overgrowth

Due to post-absorption processing, Folate levels reduced by:
- 5-FU
- Methotrexate, trimethoprim, sulfamethoxazole
Term
Serum homocysteine
Definition
Damages endothelial cells leading to thrombosis. Leads to strokes, etc.

Most commonly elevated in US due to folate deficiency. Normally Vit B12 transfers a methyl group to homocysteine to produce methionine.

Elevated by both B12 and folate deficiency.
Term
What does folate deficiency in a mother due to an unborn fetus?
Definition
Increases risk of open neural tube defects. This is from deficient folate PRIOR TO CONCEPTION and in first month of gestation.
Term
Reduced Neutrophil count.
How does it impair the immune system?
Why infection are common?
What will decrease neutrophil count?
Definition
Impairs:
Can't fight extracellular pathogens as well.

Infections:
Bacterial pathogens, even those less virulent
Fungi : Candida
Opportunistic Molds : Aspergillus

Etiology:
Cytotoxic drugs
Acute leukemia
Chronic granulomatous dz
Chediak-Higashi syndrome
Term
Reduced Cell-Mediated immunity (T-cells).
How does it impair the immune system?
Why infection are common?
What will decrease T cells?
Definition
Impairs:
Can't fight intracellular pathogens as well.

Infections:
Any organism with an intracellular phase.
Mycobacteria
Listeria
Cryptococcus
Toxoplasmosis
Pneumocystis

Etiology:
HIV
Glucocorticoids (steroids)
immunosuppression for organ transplant
lymphomas (esp. Hodgkin's disease)
PCP
Mycobacteria
Toxoplasmosis
Strongyloides
Term
Reduced Humoral Immunity (B cells / antibodies).
How does it impair the immune system?
Why infection are common?
What will decrease B cell count?
Definition
Impairs:
Can't fight extracellular encapsulated pathogens.
(Less complement and macrophage activation)

Infections:
encapsulated bacteria like S. pneumoniae, H. influenzae, N. meningitidis

Etiology:
Splenectomy
Multiple myeloma
Immunodeficiency
X-linked agammaglobinemia
Term
What pathogen do you need to consider if patient:

Has been exposed to grasses / hay
Definition
Molds
Term
What pathogen do you need to consider if patient:

Has been exposed to animals
Definition
salmonella

toxopasmosis

bartonella
Term
What pathogen do you need to consider if patient:

Has been exposed to children (eeekk!)
Definition
Viruses like
CMV
adenovirus
parvovirus
Term
What pathogen do you need to consider if patient:

Has been exposed to sex.
Definition
syphilis
HIV
HBV
HCV
Term
Hemolysis.
Lab findings.
Definition
High retic count > 75000
Corrected retic > 3%

High non-conjugated (indirect) bilirubin

High LDH (lactic dehydrogenase, in RBCs)

Low haptoglobins (binds free hemoglobin, now all occupied) - esp in intravascular

Usually normocytic MCV = 80-94
*though intravascular may lead to microcytic

Hemosiderosis - systemic iron deposition

Increase urine urobilinogen
Term
Haptoglobin
Definition
decreases when free serum Hb increases during intravascular hemolysis. It attaches to free Hb.
Term
Hemosiderinuria
and
Hemosiderosis
Definition
Hemosiderin is the insoluble degradation product of ferritin / Iron.

Hemosiderinuria - brown urine
Renal tubules convert iron from serum Hb in hemolysis into hemosiderin, which is peed out. A sign of intravascular hemolysis.

Hemosiderosis
Deposition of iron around the body. Seen in severe hemolysis.
Term
What differentiates intravascular from extravascular hemolytic anemia?
Definition
both
- high LDH


Intravascular
- Hemoglobinemia/Hemoglobinuria
- Hemosiderinuria
- low haptoglobin

eg.
paroxysmal nocturnal hemoglobinuria
mechanical destruction
microangiopathic anemia (DIC, TTP, HUS, HTN, HELLP)


Extravascular (phagocytosed by macrophages in spleen and liver)
-high unconjugated bilirubin --> jaundice and black gallstones.

eg.
hereditary spherocytosis
G6PD deficiency
sickle cell
pyruvate kinase deficiency
Term
Signs of an inherited hemolytic anemia
Definition
neonatal jaundice
recurrent hepatitis
hyperbilirubinemia
"premature" gallstones (bilirubinate)
"premature" anemia, splenectomy
Family history of a hereditary hemolytic anemia
chronic or recurrent symptoms / pains
Term
Howell-Jolly bodies
Definition
nuclear remnants in circulating RBCs

seen in peripheral smear in Sickle cell anemia and after splenectomy in Hereditary spherocytosis.
Term
Schistocytes
Definition
RBCs on peripheral smear that look like "helmet cells" - they have been cleaved by mechanical destruction intravascularly, as in microangiopathic anemia.
(DIC, TTP, HUS, SLE, malignant HTN, HELLP)
Term
Burton's lines
Definition
Lead lines on gingivae as seen in lead poisoning. May be an indication of lead poisoning as the cause of a microcytic hypochromic anemia.
Term
elevated methylmalonic acid in a macrocytic anemia tells you what?
Definition
That the megaloblastic macrocytic anemia is caused by vit B12 deficiency, not folate deficiency.
Term
Yeasts
vs.
Molds
vs.
Dimorphic Fungi
?
Definition
Yeasts
eg. Candida, Cryptococcus
pseudohyphae (with narrowings)


Molds
eg. Aspergillus (common infection post stem cell transplant)
True hyphae - no narrowings


Dimorphic Fungi
eg. Histoplasma (Ohio/Mississippi river valley), Coccidiodomyces (West US)
Yeast in the heat (in body), mold in the cold
Term
What cell is this?

Primary hemostasis, makes hemostatic plug
Dense granules of ADP and Ca
Alpha granules of vWF and fibrinogen
Definition
Platelets
Term
what do these terms mean:

anisocytosis
poikilocytosis
Definition
size variation

shape variation
Term
what is Gp1b
Definition
vWF receptor on platelets
Term
what is Gp2b/3a
Definition
Fibrinogen receptor on platelets
increased by ADP
Term
Causes of eosinophilia
Definition
NAACP

neoplastic
asthma
allergy
collagen vascular disorder
parasites
Term
What normal cell has CD28, CD3, and CD4
Definition
T helper cell
Term
What normal cell has CD28, CD3, and CD8
Definition
cytotoxic T cell
Term
What normal cell has CD19 and CD20
Definition
B cell
Term
What normal cell has CD14 and MHCII
Definition
Macrophages
develop from monocytes in tissues from gamma-interferon exposure
Term
Where do you see hypersegmented neutrophils?
Definition
Vit B12 deficiency
Folate deficiency
Term
Where do you see "crew cut skull"?
Definition
Sickle Cell anemia
Major Beta Thalassemia

from marrow expansion
Term
WBC differential: List the WBCs in order of largest proportion to least.
Definition
"Neutrophils Like Making Everything Better"

Neutrophils
Lymphocytes
Monocytes
Eosinophils
Basophils
Term
What cells is this?

IgE triggers degranulation
Degranulation is prevented by cromolyn sodium
Type 1 hypersensitivity
Granules contain histamine (vasodilate), heparin (anticoagulate by propogating antithrombin), and eosinophil chemotactic factors
Definition
Mast cells
Term
Acanthocyte
Definition
spiny/spur RBC

liver dz
abetalipoproteinemia
Term
Basophilic stippling
Definition
"BASte the ox TAIL"

Thalassemias
Anemia of Chronic dz
Iron deficiency
Lead poisoning
Term
Bite cell
Definition
G6PD d
Term
Macro-ovalocyte
Definition
Megaloblastic anemia
Marrow failure
Term
Schistocyte
Definition
Helmet cell RBC

DIC
TTP/HUS
traumatic hemolysis
Term
Spherocyte
Definition
hereditary spherocytosis
Autoimmune hemolysis
Term
Teardrop RBC
Definition
Bone marrow infiltration
eg. myelofibrosis
Term
Target cell
Definition
"HALT said the hunter to his targer"

HbC dz
Asplenia
Liver dz
Thalassemias
Term
Heinz body
Definition
denature Hb from oxidized iron

alpha thalassemia
G6PD d

--> bite cells
Term
Howell Jolly body
Definition
nucelar remnant in RBC, should be taken out by spleen

asplenia or functional hyposplenia

eg. sickle cell
Term
List causes of Microcytic anemia
<80
Definition
problem with Hb:
"CLITS"

chronic dz
lead poisoning
iron deficiency
thalassemias
sideroblastic anemia
Term
List Normocyteic anemias
low retic vs. high retic
Definition
low retic "ALARM"

Aplastic
Leukemia
Anemia of CD
Renal failure
Myelofibrosis or Marrow failure

high retic "GASP"

G6PDd
Autoimmune
Sickle and Spherocyteosis
PNH
Term
List normocytic anemias.

Nonhemolytic
vs.
Hemolytic intrinsic
vs.
Hemolytic extrinsic
Definition
Nonhemolytic: (synth problem)

Anemia of CD (new onset)
Aplastic anemia
Kidney dz

Hemolytic - Intrinsic:

Hereditary spherocytosis
G6PD d
PK d
HbC
Sickle Cell
PNH
Elliptocytosis and Pyropoikilocytosis

Hemolytic - Extrinsic:

Autoimmune
Microangiopathic
Macroangiopathic
Infection
Hypersplenism
Hypophosphatemia
Term
Macrocytic Anemias. list.
>100
Definition
Megaloblastic: (problem with DNA synth)
(Macro-ovalocytes!)

Vit B12 deficiency
Folate deficiency

NonMegaloblastic (target cells!)

Liver dz
Alcoholism
Hypothyroid
Reticulocytosis
Drgus
Term
Intravascular Hemolysis vs. Extravascular Hemolysis

(signs)
Definition
Intravascular
Low haptoglobin bc binds free Hb
Hb in urine

Extravascular
High UCB (indirect) --> jaundice
Term
Steps of Heme degradation to Bilirubin
Definition
Heme ---(heme oxygenase)--> Bilverdin, Iron, CO, NADP

Bilverdin---(bilverdin reductase)-->Bilirubin, NADP

Bilirubin--> to liver in blood bound to albumin
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