Principles Of Medicine/pathology

Front 1

Population most likely to have Beta Thallassemia?

Back 1

Blacks, Greeks, Italians

Front 2

Population most likely to have alpha Thallassemia?

Back 2

Blacks, Asians

autosomal recessive

Front 3

What are the childbearing consequences of alpha thallassemia?

Back 3

↑ Spontaneous Abortions, so they have the Highest incidence of Choriocarcinoma

Front 4

What autosomal recessive disease leading to microcytic anemia will show normal appearing globin chains (Alpha, Beta, Delta, Gamma) on electrophoresis?

Back 4

alpha thallassemia seen most often in blacks and Asian's.

Every globin is reduced in quantity but normal appearing because there is a problem in making α Globin Chain, which is required for all hemoglobin production.

Front 5

In what diseases are Target cells seen?

Back 5

Thallassemia, Hemoglobinopathies, Liver disease, and Asplenia

Together these are "HALT", said the hunter to the target

Front 6

What is the consequence of 2 α Globin Chain deletions, vs 3, vs 4?

Back 6

2 mild anemia
3 = HbH resulting in Beta4
4 hydrops fetalis = Hb Barts

Remember α is required to make the other globins

Front 7

What is HbH and Hb Barts?

Back 7

HbH is the result of 3 α deletions seen in α Thallassemia (blacks and Asians) --> Beta 4

Hb Bart is the result of 4 α deletions, hydrops fetalis and gama 4

remember α is required to make Beta, if there is no α...no Beta

Front 8

β Thallassemia is found in what populations?

Back 8

Blacks, Italians, and Greeks

Front 9

What are the medical consequences of β Thallassemia?

Back 9

minor/heterozygous β production difficulties --> inadquately increased HbF, anemia

major β --> complete β deletion --> inadequate HbF increase, constant need for blood transfusions --> hemochromatosis/hemosiderosis (too much iron) --> cardiac failure, bone deformities

Front 10

What are the differential findings and other test findings in Thallassemia's

Back 10

CBC: normal/increased RBC, RDW (increases in RDW are microcytic)

Iron Studies: normal

differential - microcytic, basophilic stippling (persistence of ribosomes), target cells (because cells are small, the excess membrane bulges in the middle), tear drop (macrophages remove excess globin)

electrophoresis - normal in alpha deletion, increased HbA2 (2α, 2δ) and HbF (2α, 2γ) in Beta's

Front 11

When MCV is low (<80) think ____ or ____.

Back 11

blood loss or Fe deficiency

Front 12

When MCV is high (>100) think ___ or ____.

Back 12

B12 deficiency do to alcoholism with lots of nuclei lobes in the PMN's and mental status changes.

or folate deficiency with peripheral neuropathy

Front 13

What is the disease?

microcytic, low MCV, MCH, Hgb, Hct,

Back 13

Iron deficiency anemia

Front 14

What is the general disease?

low Hgb and Hct
high MCV and MCH

Back 14

macrocytic anemia

Front 15

What is the cause of this anemia?

Normal MCV, MCH, Hbg, Hct

Back 15

acute blood loss

Front 16

What is the disease?

low Hct, Hbg,
normal RDW and MCH

Back 16

Anemia of chronic disease/inflammation

Front 17

What is this:
WBC 7.5
RBC 3.0
HGB 9.0
HCT 28
MCV 75
MCH 25
MCHC 32
RDW 20

Back 17

chronic (increased RDW) iron def. microcytic anemia (low MCV, MCH, Hbg, Hct)

Front 18

What is this:
WBC 18
RBC 4
Hgb 8.5
Hct 25
MCV 100
MCH 33
MCHC 35
RDW 13
MPV 9
PMN with many lobes

Back 18

macrocytic anemia due to B12 def. anemia (give away, PMN with many lobes) acute (RDW is normal, Hbg was 12.2 recently)

Front 19

pancytopenia

in what diseases?

Back 19

very few cell seen, all RBC's present are the same size (low RDW) as is seen with

aplastc anemia
paroxysmal nocturnal hemoglobinuria

Front 20

What is Colley's anemia?

Back 20

Beta thallassemia major

(β0/β0): Severe β ↓
Long Term need of transfusions resulting in iron overload → Hemosiderosis → bone deformities, heart failure.

Front 21

When RDW is low think ___.

Back 21

macrocytic anemia

Front 22

When RDW is high think __.

Back 22

microcytic anemia

Front 23

crenation (wrinkled ruined-like cells) =

Back 23

renal dysfunction

Front 24

Name 3 causes of sideroblastic anemia (a microcytic anemia with sideroblastic/iron rings).

Back 24

Lead poisoning (busts fellochelatose)

B6 deficiency (no porphorin in mitochondria because of synthesis problem)

alcoholism (damaged mitochondria)


either way, iron can not get out of the cell

Front 25

Two causes of basophilic stippling

Back 25

microcytic anemia in thallassemia and lead poisoning

Front 26

What is the disease?
low Iron, % binding, ferritin, high TIBC

Back 26

Iron deficiency anemia

probably microcytic with high RDW

Front 27

What is the disease?
low iron, % binding, TIBC, high ferritin

Back 27

chronic disease

the body tries to hid/sequester Fe in macrophages in the bone marrow to keep it from pathogens

Front 28

what type of infections would a person without a spleen (due to removal, SS dz, etc)?

Back 28

Streptococcus, Haemophillus, and Neisseria

Front 29

If only RBC's are destroyed in normocytic pancytopenia consider what assaulting agent?

Back 29

Parvo virus (the little DNA naked one) which causes RBC aplastic anemia

Front 30

If RBC's, WBC's and platelets are destroyed in normocytic pancytopenia, consider what assaulting agent?

Back 30

Hep C aplastic anemia

Front 31

A patient presents with normocytic pancytopenia, petechia, pallor, malaise, and infection. On bone marrow biopsy you see fatty infiltrate in hypocellurlar findings. What do you suspect?

Back 31

applastic anemia of heriditary, parvo, Hep C, or other causes

Front 32

What are the differences between warm and cold autoimmune normocytic anemia?

Back 32

Warm is caused by extravascularly destroyed IgG mediated type II hypersensitivity to RBC's because of SLE or drugs.

Cold is type III hypersensitivity to IgM from mycobacteria in either intra or extravascular destruction.

Front 33

spherocytosis (lost central palor and biconcavity), increased reticulocyte, ↓ Haptoglobin, and Hemoglobinuria in normocytic anemia would indicate what disease?

Back 33

cold IgM mediated hype III hypersensitivity to mycobacteria, hemoglobinuria because the haptoglobin carries the hemoglobin in the blood (only with intravascular decay)

Front 34

+ Coumb's test indicates?

Back 34

autoimmunity such as warm and cold hemolytic normocytic anemia

Front 35

increased reticulocyte ct and schistocytes in normocytic anemia might indicate?

Back 35

DIC/HUS, HELLP

Front 36

what is a common cause of chronic disease causing normocytic anemia?

Back 36

kidney disease-chronic

Front 37

Phoslo 667

Back 37

for kidney problems

calcium acitate to drop phosporus levels in renal failure patients

Front 38

macrocytic anemias usually have __ platelet counts, normocytic usually have __ platelet counts. Why?

Back 38

macrocytic low (they are big/swollen and can't get out of marrow so they are eaten by macrophages)

normocytic are high due to inflammatory processes associated with chronic disease

Front 39

MOA of rituximab?

Back 39

anti CD20 antibody (from lymphoma therapy but used elsewhere now)

causes lysis of B lymphocyte

used for antibody mediated hemolytic anemia

Front 40

What are the treatment options for IgG (warm) auto antibody mediated normocytic hemolysis?

How is this different than IgM (cold) hemolysis?

Back 40

warm = splenectomy or rituximab

cold = no splenectomy

Front 41

What could this disease be:
spleenomegally
increased LDH and bilirubin
decreased haptoglobin
normocytic anemia
jaundice
increased reticulocyte
spherocytosis
patient has SLE

Back 41

IgG warm hemolytic anemia

Front 42

What disease would this person have according to the clinical findings?

Insidious course
Associated with aplastic anemia
increased LDH and bilirubin
decreased haptoglobin
Venous thrombi
Hemolytic findings
Pancytopenia

Back 42

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

from enhanced compliment to the RBC's

Front 43

What X linked disease is associated with normocytic anemia, increased reticulocyte, increased LDH and bilirubin, decreased haptoglobin, Heinz bodies, and bite cells?

Back 43

G-6-P dehydrogenase deficiency found in blacks, mediterraians, and oxidizing drugs (like antimalarials)

Front 44

What is the point mutation that causes sickle cell anemia?

Back 44

HbA --> HbS because of GAG(glutamine)-->GTG(valine)

Front 45

What is the pathogenesis of sickle cell anemia?

Back 45

loss of Hbg solubility --> lines and globs up --> distorts RBC

Front 46

What is the inheritance pattern of sickle cell disease different from trait?

Back 46

it is autosomal recessive so it must be from both parents.

trait is mild anemia due to inheritance from 1 parent (Hgb S and A)

Front 47

How do you diagnose SC anemia?

Back 47

peripheral smear
electrophoresis for HbS

Front 48

What are the long term treatments for Sickle cell anemia.

Back 48

hydroxyuria - oral antimetabolytes, chemotherapy used in myeloproliferative dz, causes increased production in Hbg F

Iron chelation

Narcotics

support

Front 49

What kind of anemia do you find in TTP?

Back 49

microangiopathic hemolytic anemia

beat up cells because they are trying to get past clot

Front 50

LDH goes down or up in hemolysis?

Back 50

up

Front 51

How long does a retiuclocyte look like a reticulocyte?

Back 51

24 hours

Front 52

What are the signs and symptoms of TTP?

Back 52

fever, kidney problems, purpura, microangiopathic hemolytic anemia, neurologic events

Front 53

G-6-P D inherited or acquired?

Back 53

inherited but doesn't show up until adulthood

Front 54

sickle cell anemia is worse than sickle cell trait because...?

Back 54

some Hgb A present

screwed up polymerizing agent

Front 55

Iron dose?

Back 55

300mg tid

any type...go for cheap

Front 56

Dietary suggestions for iron deficiency?

Back 56

eat green leafy, meat, beans, with vitamin C (not calcium or abx or bisphosphonate)

take iron rich food with iron poor foods

Front 57

What do you do for patients that can't absorb or tolerate iron supplementation?

Back 57

IV iron

watch for anaphylaxis (1/100) so give with benedryl and steroids...but watch

Front 58

Iron chelating drugs (2).

Back 58

Deferoxamine (Desferal)
Deferasirox (Exjade) oral versin of Deferoxamine

binds free iron

Front 59

What are the side effects of these drugs:
Deferoxamine (Desferal)
Deferasirox (Exjade)

Back 59

back/abd pain
nausea/vom
rash
conjunctivitis
tinitis


These are iron chelators

Front 60

what is the difference between megaloblastic and macrocytic anemia

Back 60

megaloblasts are in the marrow
macrocytes are in the circulating blood

Front 61

When is is homocysteine elevated compared to methionine?

Back 61

B12 deficiency

this is reduced in the change from Methyl-Tetrahydrofolate to THF on the way to make DNA

Front 62

When do you not give folate??

Back 62

when the patient is B12. You can give both together but not alone.

Front 63

What is the folate dose? and foods?

Back 63

1 mg tid veggies not meat

Front 64

What is the B12 dose? and food?

Back 64

IM most common, 1000 mcg/month, rest of life

fish, meat dairy

Front 65

What is the route of administration of EPO?

Back 65

IV, sub q, IM

Front 66

What is Hydroxyurea (Hydrea) used to treat?

Back 66

Chemo for head and neck Ca, leukemia, etc

sickle cell anemia, thrombocytosis, because it increases Hgb F. But it can lower WBC so it isn't used much.

Decreases painful vaso-occlusive crises and hospital admissions

Front 67

how much pRBC will raise a Hct 10%?

Back 67

10ml/kg

Front 68

How much platelet do you administere?

Back 68

1 unit of platelets/10 kg body weight raises platelet count 17-50,000

Front 69

Who is most susceptible to transfusion reactions?

Back 69

multiparous mothers and post of lots of blood (ie Ca)

Front 70

What is post transfusion purpura? Who usually gets it?

Back 70

occurs 5-15 days after a transfusion of RBCs induces an alloantibody against the P1 antigen

The recipient is usually P1 negative, female and multiparous

Front 71

What are the signs of post transfusion purpura?

Back 71

fever
low platelet level
bleeding

Front 72

What is the major toxicity for Exjade?

Back 72

chelating agent

GI toxicity
back/ab pain

Front 73

A women starts taking iron, can't tolerate it, you start to give parental iron. What is the potential problem/toxicity?

Back 73

anaphylaxis

Front 74

Patient with pernicious anemia. What is the schedule for B12 replacement?

Back 74

parentral daily for a week, weekly for a month, monthly forever

Front 75

What causes post transfusion purpura?

Back 75

5-7 days alloimmunization event

immunity to P1 antigen on platelet

Front 76

diseases with too much iron

Back 76

hemociderosis
hemochromotosis

Front 77

What are times when polycythemia are due to RBC problems (abnormal phys).

Back 77

Carbon monoxide exposure
Hemoglobinopathy with excessive binding
Polycythemia rubra vera

Front 78

What is spurious polycythemia?

Back 78

Reduced plasma volume (they are dehydrated, burned, or low on protein not RBC's)
Reduced whole blood volume (total is lower)
Same number of RBCs

Front 79

What is polycythemia?

Back 79

same plasma volume
more RBC volume
less plasma volume

Front 80

Gaisboch's syndrome

Back 80

Chronic plasma volume reduction seen in Type A middle aged executives with caffeine and tobacco excess. Diuretic use for hypertension may contribute.

Front 81

What is the testing scheme to look for polycythemia?

Back 81

H & P to see if there is another reason
EPO level (to separate secondary cause of PC from Polycythemia Rubra Vera)

Front 82

If there is evidence of polycythemia and the EPO is normal or low, what is the Dx?

Back 82

Polycythemia Rubra Vera

primary polycythemia due to genetic mutation of JAK-2, myeloproliferative dz so platelets and WBC are high too

Front 83

What is the target of EPO?

Back 83

the JAK-2 receptor in the blast forming units of bone marrow precursor cells

Front 84

Person has high WBC, RBC, H&H, platelet levels
spleenomegally
headache and sluggish tired thinking
HTN
low EPO

What might they have?

Back 84

Polycythemia Rubra Vera

Front 85

What is the treatment (now and future) for polycythemia Rubra vera?

Back 85

now blood donation

Jak-2 inhibitor

Front 86

When is it appropriate for erythropoietin to rise despite normal hematocrit values?

Back 86

When the kidney is low in oxygen such as cardiac, lung, and Hbg causes.

Front 87

What will be the presentation of someone with Essential Thrombocytosis?

What are their lab and chromosome results?

Back 87

Erythromelalgia - Warm, red, painful sensations in toes and feet (worse with showers)
Hemorrhage
Thrombosis

platelet ct >600K
RBC's, WBC's Hct/Hbg normal
Philadelphia chromosome neg (associated with chronic myelogenous leukemia (CML))
Jak-2 mutation

Front 88

What does this person have?

Erythromelalgia - Warm, red, painful sensations in toes and feet (worse with showers)
Hemorrhage
Thrombosis

platelet ct >600K
RBC's, WBC's Hct/Hbg normal
Philadelphia chromosome neg (associated with chronic myelogenous leukemia (CML))
Jak-2 mutation

Back 88

ET (esential thrombocytosis)

Front 89

What is the treatment for Essential Thromobocydosis?

Back 89

Hydroxyuria
Interfuron
ASA

Front 90

What is the cause of Myelofibrosis?

Back 90

myeloproliferative disorder manifested as excessive collagen deposition in the bone marrow. A rare disorder of unknown cause. Some people have damaged DNA.

Front 91

What does this patient have?

Splenomegaly
Hepatomegaly
Anemia with circulating nucleated red cells and teardrop proliferation
Thrombocytosis
Leukocytosis

Back 91

Myelofibrosis

Front 92

What is Eltrombopag used in?

Back 92

ITP (Idiopathic Thrombocytopenia?)

use it for life, quite expensive, works awesome. increases megaloblastic production in the marrow.

Front 93

What is Heparin Induced Thrombocytopenia (mild or major)?

Back 93

An acquired hypercoagulable state arising from induction of an antibody against the heparin-platelet factor IV site

Onset 4-10 days after heparin exposure. Mild cases seen in cardiac patients who go to cath lab q 3-6 months.

Front 94

What do these clinical symptoms describe?

Thrombocytopenia-moderate (can even be w/in normal range but a drop by 50%)
Arterial thrombosis concurrent with venous thrombosis

Back 94

Heparin induced thrombocytopenia (HIT)

look into their history
do test for Hep Ab (takes 4 days)

Front 95

What do you NOT give to heparin induced thrombocytopenia patients (2 things)?

Back 95

Heparin
coumadin (initially prothrombic)

Front 96

What diseases has systemic concurrent clotting and bleeding. They have purpura, are bleeding out of every orofic, hypotension, and every organ is failing. This can be chronic (Ca) in cause or sudden.

Back 96

DIC

Front 97

How do you treat DIC?

Back 97

treat underlying cause and support
do NOT give platelets, anticoagulant, activated protein C etc

Front 98

disease of decreased heme synthesis

Back 98

microcytic iron deficient anemia

chronic dz is decrease release of iron to transferrin
thalassemia is decreased globin synthesis
lead poisoning and other sideroblastic anemias have protein denaturation leading to heme/iron damage

Front 99

disease of protein denaturation leading to heme and iron sequestering

Back 99

sideroblastic anemias: lead poisoning, low B6, and alcohol poisoning.

Front 100

anemia of gene mutation leading to decreased globin synthesis

Back 100

thalassemias

Front 101

anemia of decreased release of iron to transferrin

Back 101

microcytic anemia of chronic disease