Erythrocyte Disorders) Flashcards by michael perna

In a CBC:

RBC measures

Hemoglobin measures

Hematocrit measures

MCV measures

MCHC measures

RBC = # of red blood cells

Hemoglobin = Amount of hemoglobin in blood (Red color, oxygen carrying)

Hematocrit = Percent of volume that RBCs take up in blood

MCV = Average volume (size) of red blood cells
–>Microcytic, normocytic, Macrocytic

MCHC = Concentration of hemoglobin in an average red blood cell
–> Hypochromic, Normochromic

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Anemias are defined on the basis of….

Cell Size (MCV) and amount of Hgb (MCH)

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If MCV is less than the lower limit of normal it is what type of anemia?

Normal Range?

Greater than the upper limit?

Microcytic Anemia

Normocytic Anemia

Macrocytic Anemia

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If MCH is less than the lower limit of normal, it is what type of anemia?

If MCH is within the normal range?

Hypochromic Anemia

Normochromic Anemia

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An means

Emia means

An = Without
Emia = blood

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This is defined as a reduction in HEMOGLOBIN concentration in circulating blood, resulting in tissue hypoxia due to lack of oxygen-carrying capacity of blood

Anemia

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Symptoms of this illness include pale skin and mucous membranes, jaundice (if hemolytic), Tachycardia, Breathlessness, Dizziness, and Fatigue

Anemia

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There are three ways to become anemic…

Lose Blood

Destroy Too Much Blood (hemolysis)

Make Too Little Blood

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Hemolysis (anemia) can happen for 2 reasons

Extracorpuscular

Intracorpuscular

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When the cause of anemia is losing blood, it is what type of anemia?

When the cause is destruction of too much blood, it is what type of anemia?

When the cause is making too little blood, it is what type of anemia?

Blood loss (hemorrhage)

Hemolytic

Bone marrow suppression/nutritional deficiencies

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The etiology of blood loss anemia is traumatic, acute blood loss NOT > 20%

At first the hemoglobin level is normal. After 2-3 days, we see what (have ribosomal RNA, and no nucleus)?

Chronic Blood loss is different, and due to…

Reticulocytes

Iron Deficiency Anemia

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Hemolytic anemia is due to decreased red cell lifespan. It can be chronic or acute.

This type is inherited and due to red cell defects

This type is aquired

Intracorpuscular

Extracorpuscular

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In hemolytic anemia, increased bilirubin, increased LDH, and decreased haptoglobin are signs of….

Destruction

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In hemolytic anemia, increased reticulocytes and nucleated red cells in blood are signs of….

Production (of red cells?)

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This extracorpuscular cause of hemolytic anemia is due to physical trauma to red cells, schistocytes (fibrin strands that shred RBCs), and triangulocytes. It is important to find out why it is occurring

Microangiopathic Hemolytic Anemia

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The causes of this illness include:

Artificial Heart Valves
Malignancy
Obstetric Complications
Sepsis
Trauma

Microangiopathic Hemolytic Anemia

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This type of anemia is characterized by IgG, the spleen, and spherocytes

Warm Autoimmune Hemolytic Anemia (Warm AIHA)

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This type of anemia is characterized by IgM, complement, intravascular hemolysis, and agglutination

Cold Autoimmune Hemolytic Anemia (Cold AIHA)

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This is the most common form of AIHA

_____ antibodies bind to RBCs best at 37 degree body temperature (98.6F)

What occurs in the spleen?

Warm AIHA

IgG

Splenomegaly

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In Warm AIHA, these RBCs are formed from nibbling by macrophages (Kupfer cells) causing loss of membrane, leading to a spherical shape

Spherocytes

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This is the less common AIHA

_____ antibody binds to RBCs at 0-4 degrees celsius, in the ears, hands, and toes in cold weather

It causes extravascular hemolysis via membrane attack complexes in what organs? (2)

Cold AIHA

IgM

Spleen and Liver

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In cold AIHA, this is binding of IgM that causes cross linking of red cells, and sludging of blood

What can it ultimately lead to?

Agglutination

Raynaud’s Phenomenon

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This is a test for AIHAs, it can detect the antibodies on RBCs using blood samples

Direct Coombs Test/Direct Antiglobulin Test

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A determination of warm AIHA is positive for what?

A determination of cold AIHA is positive for what?

IgG

Compliment

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Warm AIHA can be secondary to 2 things It can also be induced by:

Lymphoproliferative Disorder and Autoimmune Disease Drugs

Cold AIHA can be secondary to 2 things

Infection and Lymphoproliferative Disorder

What is found in the blood film of warm AIHA Cold AIHA?

Warm: Spherocytes Cold: Agglutination

This is an intracorpuscular anemia characterized by QUALITATIVE defect in hemoglobin (aka hemoglobinopathy)

Sickle Cell Anemia

This intracorpuscular anemia is characterized by a single AA substitution in the beta chain of hemoglobin It can be heterozygous or homozygous, which has worse prognosis? It creates these cells which are fragile (burst easily), and they get stuck in vessels

Sickle Cell Anemia Homozygous Sickle Cells

In sickle cell anemia, what kind of mutation occurs in the Beta chain gene, leading to abnormal beta chains? The cell will aggregate and polymerize upon deoxygenation. They get stuck in vessels and are fragile.

Point Mutation (valine substituted for glutamate)

Sickle Cell Anemia is more prominent in what ethnic group? It's severity is variable

Blacks

What 3 things does sickle cell disease cause? Treatment includes preventing triggers, vaccinating, and blood transfusion

Chronic Hemolysis, vaso-occlusive disease, and increased infections

This intracorpuscular anemia is a hereditary QUANTITATIVE defect in Hb. Not enough alpha or beta chains can be made. It has variable disease severity

Thalassemia

What type of anemia is thalassemia?

Hypochromic, microcytic with increased RBC and target cells

This blood disease causes tons of spherocytes with a spectrin defect. What cures it?

Hereditary Spherocytosis Splenectomy

Hereditary Spherocytosis is autosomal ______ It causes a defect in the RBC membrane due to a mutation in ______, a membrane protein

Dominant Spectrin

Hereditary Spherocytosis causes spheroidal erythrocytes called ______ that are more vulnerable to sequestration/destruction by the spleen On smears, red cells lack normal central pallor Also, splenomegaly (enlargement of spleen)

Spherocytes

40-50% of patients with hereditary spherocytosis experience _____

Gall stones (cholelithiasis)

Glucose-6-Phosphate Dehydrogenase Deficiency causes increased ______ which cause cell lysis Related to exposure to what kind of drug? G6PD is self limiting

Peroxides Oxidative Drug

What cells develop in G6PD due to removal of Heinz bodies?

Bite Cells

In G6PD, some patients are asymptomatic Other have _____________ hemolysis triggered by broad beans (favism-Africa), drugs (antibiotics, aspirin, infections) It resolves spontaneously

Episodic hemolysis

Look at chart on the role of G6PD on slide 62 related to pentose phosphate pathway

G6PD deficient cells die in part due to reduced ______, oxidants attack hemoglobin bonds This causes heme to break away from globin, globin denatures and sticks to the RBC membrane, forming ______ This organ bites out Heinz bodies, leading to bite cells?

GSH Heinz Body Spleen

Anemia associated with making too little blood has too few building blocks, like this type Or it can be due to too few erythroblasts, like this type Or there could be not enough room, like this type

Iron Deficiency Anemia Aplastic Anemia Myelophthlistic Anemia

The most important cause of iron deficiency anemia is

GI Bleeding

What category of anemia is iron deficiency anemia? It's a symptom, not a disease! Must find cause.

Microcytic, hypochromic anemia

This is a symptom of iron deficiency anemia, tongue inflammation/loss of papilla

Atrophic Glossitis

There are 3 causes of iron deficiency

Decreased iron intake (bad diet, bad absorption) Increased iron loss (GI bleeding, menstrual cycle, hemorrhage) Increased iron requirement (pregnancy)

Anemia caused by infections (osteomyelitis, endocarditis), inflammation (immune disorders like RA), and malignancy are anemias of chronic disease. Iron metabolism may be disturbed. It is usually mild anemia. What type of anemia is it?

Normochromic, normocytic

These are anemias of defective DNA synthesis, and has nuclear/cytoplasmic asynchrony It is caused by a decrease in these molecules, associated with pernicious/folate anemia

Megaloblastic anemia B12/Folate

What type of anemia is a megaloblastic anemia?

Macrocytic, hyperchromic

Vitamin B12 is needed to make..

DNA! (Megaloblastic anemias)

Anemias of Chronic Diseases are caused by,.. Iron metabolism is disturbed What are the RBCs under the microscope? Is anemia mild or severe?

Infections Inflammation Malignancy Normochromic, normocytic Mild

Megaloblastic anemia is initially caused by retarded _____ synthesis Which leads to impaired ____ synthesis Which leads to cells that look like what?

DNA RNA Big cells, with immature nucleus and mature cytoplasm

This can occur with megaloblastic anemia, and includes tongue swelling and loss of papilla (discussed earlier as well)

Atrophic Glossitis

This type of anemia is characterized by pancytopenia (deficiency of all three cellular components of the blood), empty marrow, and idiopathic etiology

Aplastic Anemia

There are 5 potential causes of aplastic anemia

``` Idiopathic Drugs Viruses Pregnancy Fanconi Anemia ```

This red blood cell disorders causes RBCs to be released from marrow in excess quantities. It has an unknown cause.

Polycythemia

Polycythemia is caused by hyperplasia of what?

Erythroid Precursors

In polycythemia, increased MCV/ viscosity leads to.... What disease develops?

Organ Congestion Leukemia

This anemia is when there is not enough room

Myelophthisic Anemia

What is the cause of myelophtisic Anemia? Associated with anemia and thrombocytopenia

Extensive inflitration of marrow by a tumor/lesion | metastatic breast, lung, or prostate cancer, and advanced TB

Right heart failure leads to what?

Congestion of peripheral tissues which leads to liver and tract congestion, as well as edema and ascites

Left heart failure leads to what?

Decreased cardiac output (leads to activity intolerance and signs of decreased tissue perfusion) Pulmonary congestion

When left heart failure leads to pulmonary congestion, what occurs?

Impaired gas exchange and pulmonary edema (look at diagram with heart on it)

Cyanosis and pulmonary edema (dyspnea) are consequences of failure on which side of the heart?

Left

Hepatomegaly, jugular venous distention, splenomegaly, ascites, and peripheral edema are consequences of failure on which side of the heart?

Right

This is a major health problem affecting 25% of the population, it is asymptomatic until late. It contributes to coronary artery disease, stroke, cardiac hypertrophy, and heart failure

Hypertension

What is the cause/mechanism of hypertension?

Depends, but largely unknown (idiopathic)

What is the clinical measure of hypertension?

>140/90

This is idiopathic hypertension, and comprises 95% of hypertension cases

Essential Hypertension

This hypertension comprises 5% of hypertension cases

Secondary Hypertension

This is the classification for BP > 180/120

Malignant Hypertension

This is hypertension that is urgent (180/110) or is an emergency (with end organ damage)

Hypertensive Crisis

What are the boundaries for classification of hypertension?

Normal >120 and >80 Pre-hypertensive 120-139 or 80-89 Stage I 140-159 or 90-99 Stage II greater than or equal to 160, or greater than or equal to 100

White essential hypertension is idiopathic, it may be related to: Reduced ______ excretion ______ disease ______ factors _____ factors

Sodium Excretion Vascular Disease Genetic Factors Environmental Factors (Drug, EtOH)

Essential hypertension accelerates what condition? It potentiates what 2 conditions?

Atherogenesis Aortic Dissection and Stroke

Essential hypertension causes small blood vessels to narrow, especially in this organ It hardens the arteries, leading to either: Benign hypertension, called Severe Hypertension, called

Kidneys Hyaline Arteriosclerosis Hyperplastic arteriosclerosis

This is defined as normal, clot free blood flow that is able to form a hemostatic plug if there is vascular injury

Hemostasis

This is the pathological counterpart of hemostasis, the formation of a blood clot within a vessel

Thrombosis

This is a solid mass of coagulated blood inside an uninjured vessel in the cardiovascular system (or after a relatively minor injury It may be life saving or life threatening, clotting stops bleeding but can block a blood vesse

Thrombosis

This is an intravascular solid, liquid, or gaseous mass carried by the blood to a site distant from its point of origin What do they mostly derive from?

Embolism Thrombuses (this process is called thromboembolism)

Eventually, emboli lodge in vessels that are too small. This type of embolism causes necrosis of downstream tissue (infarction) This type of embolism causes hypoxia, hypotension, right sided heart failure

Systemic Embolization Pulmonary Embolization

This is the term for venous emboli that eventuall block pulmonary arteries, leading to death. Most are small and silent

Pulmonary Thromboembolism

Pulmonary thromboembolisms originate 95% of the time from?

Deep Vein Thrombosis (thrombi in deep leg)

This is an arterial emboli 80% arise from ____________ Thrombi Final resting place depends upon their point of origin 75% or the time it ends up here 10% of the time it ends up here

Systemic Thromboembolism Intracardiac Mural Thrombi Lower extremities (75%) CNS (10%)

When an infarct occurs in a myocardial location is called

Coagulative Necrosis

When an infarct occurs in a cerebral location it is called

Liquefactive Necrosis

When an infarct occurs in the pulmonary system, it is called this and associated with this disease

Caseous Necrosis TB

When an infarct occurs in the bowels it is called

Liquefactive Necrosis

When an infarct occurs in the distal extremities, it is called

Gangrenous Necrosis

This is a retroviral disease of the lentivirus family and is characterized by a depletion of CD4+ T-lymphocytes and profound immunosuppression

AIDS

How many people are infected with AIDS in the US? In Africa? When were the peak number of deaths in

1 million 23 million 1995 (it's been declining since)

Transmission of AIDS occurs via What percent of it occurs in male-male sex? Heterosexual contact? IV Drug Use? Blood Transfusion? Parental Transmission to fetus/newborn?

Exchange of blood or bodily fluids 48% 34% 16% 1% 1%

AIDS transmission is extremely low risk for dentists when protective barriers are worn Seroconversion from a needle stick/exposure of nonintact skin from an HIV patient occurs at what percent per exposure? For a patient with hepatitis it is...

0.3% 6-30%

What is the structure of HIV?

RNA retrovirus

How many strands of RNA are in the AIDS virus? What core does it have? What type of membrane does it have, where is it derived from, and what does it have in its surface

2 single strands Viral protein core (p24) Phospholipid bilayer derived from the host cell membrane and studded with gp120 and gp41

HIV entry into host cells requires what molecule? What envelope protein of the virus fuses with the CD4 cell membrane, binding the virion to the cell? Which envelope protein inserts into the target membrane, fusing the virus with the cell? The virus then enters what part of the host cell?

CD4 gp120 gp41 Cytoplasm

In the progression of HIV infection, during initial infection, the first cells infected are ____________ This results in major depletion of lymphocytes

Mucosal Lymphoid tissue CD4+ T cells

In the progression of HIV infections, during the acute phase (which is after initial infection), viral replication leads to _____ within days (about 3-6 weeks) The host immune response is termed

Viremia Acute HIV Syndrome

This occurs during the acute phase of HIV infection, and is nonspecific symptoms of any virus like fever, sore throat, occurring between weeks 3-17 So viral load is low at 12 weeks

Acute HIV Syndrome

During progression of HIV infection, during the chronic phase (after the acute phase), there is continued T cell depletion with new CD4 cells being made but unable to keep up with loss for several years What occurs?

Asymptomatic or persistent lymphadenopathy May have zoster or candida infection

During this phase of progression of an HIV infection, the CD4 count is less than 500 cells/ul, fever is present for one month, and there is weight loss/fatigue

Crisis Phase

During this phase of progression of HIV infection, the CD4 cell count is less than 200 cells/uL, leading to pneumonia/TB, opportunistic infections, malignancies, AIDS dementia It occurs by how many years if left untreated?

AIDS 10 years (antiretrovirals have changed the course of the disease)

Regarding cells affected by HIV, these are the first immune cells effected Its count eventually declines, and this ratio declines as well

CD4+ Inducer T cells Helper/suppressor ratio (CD4/CD8 ratio)

These cells are affected by HIV becasue there is reduced response to specific antigens due to loss of stimulation from T-helper cells

B cells

These cells affected by HIV are mucosal and capture the virus, transporting it to regional lymph nodes. Follicular ones in the germinal centers of lymph nodes are important ______ of HIV

Dendritic Cells Reservoirs

These cells are affected in tissues by HIV, like CD4 T cells Where specifically does this occur? Why are they a good reservoir for HIV?

Monocytes/Macrophages Brain and Lungs They are resistant to the cytotoxic effects of HIV

(AIDS malignancies) This used to be the most common AIDS tumor, but has decreased due to antiretroviral treatment What areas does it affect What virus causes it?

Kaposi Sarcoma Mouth, skin, lungs Herpes virus HHV-8

This is the 2nd most common AIDS malignancy in patients What virus plays a role? Lymphoma of the _____ is considered an AIDS defining condition

Non-Hodgkin Lymphoma EBV Brain

AIDS defining cancers (Kaposi, Lymphoma) are on the decrease due to antiretrovirals Non AIDS defining cancers are increasing

Liver Cancer, Hodgkins lymphoma, Anal Cancer

Erythrocyte Disorders) Flashcards

(111 cards)