Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

M.T.B > Internal Med- Hematology > Flashcards

Internal Med- Hematology Flashcards

1
Q

What is the best initial test for evaluation of

anemia?

A

Complete blood count (CBC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

If symptoms of anemia are based on the severity of disease and not the etiology, what Sx would you expect with the following Hematocrit levels;

1) 30-35%
2) 25-30%
3) 20-25%
4) <20%

A

1) No symptoms
2) Dyspnea (worse on exertion), fatigue
3) Lightheadedness, angina
4) Syncope, chest pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Whats the most common cause of death in anemia?

A 
MI due to cardiac ischemia. Because myocytes cannot distinguish between:
Anemia
Hypoxia
Coronary artery disease
Carbon monoxide poisoning
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the causes of Microcytosis or Low MCV?

A
  • Iron deficiency
  • Thalassemia
  • Sideroblastic anemia
  • Anemia of chronic disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Is the reticulocyte count in Microcytic anemias low or high?
Why?

A

Microcytic anemias generally have a low reticulocyte count. Because most causes of microcytosis are production problems.
*Only alpha thalassemia with 3 genes deleted has an elevated reticulocyte count.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is a unique feature of Sideroblastic anemia based on the MCV

A

It can present as either microcytic or macrocytic anemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some of the causes of Macrocytic anemia or High MCV

A
  • B12 and folate deficiency
  • Sideroblastic anemia
  • Alcoholism
  • Medications such as zidovudine or phenytoin
  • Myelodysplastic syndrome (MDS)
  • Liver disease or hypothyroidism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Is the reticulocyte count in Macrocytic anemias low or high?

A

Macrocytic anemias all give a low reticulocyte count.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Explain why Normocytic Anemia has a normal MCV

A

Acute blood loss or hemolysis is slow to make the MCV change.
Blood loss ultimately leads to iron deficiency and microcytosis. Eventually, hemolysis will increase the reticulocyte count, and this will raise the MCV since reticulocytes are slightly larger than normal cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the indications to transfuse a Pt with Anemia?

A
  1. symptomatic patient
  2. very low hematocrit
  3. elderly Pt or Pt with heart disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How much does a unit of Racked Red Blood cells (PRBCs) raise the hematocrit by?

A

3 points per unit, or 1 g/dL of Hg.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When is Fresh frozen plasma (FFP) indicated?

When is it contraindicated?

A

those with an elevated
prothrombin time, activated partial thromboplastin time (aPTT), or INR and
bleeding.

Not the choice in Hemophilia A or B or von Willebrand
disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When is Cryoprecipitate used?

A

To replace fibrinogen and has some utility in

disseminated intravascular coagulation. High levels of Factor VIII and VWF are found in it.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

When is giving Platelets indicated?

When is it contraindicated?

A

if platelet count is <50,000

Contraindicated in TTP.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the pathophysiology behind Microcytic Anemia of Chronic disease?

A

Anemia of chronic disease is of unclear etiology. Iron is locked in storage or trapped in macrophages or in ferritin. Hemoglobin synthesis will not occur because the iron just does not move forward. The precise mechanism is clear only in renal failure in which there is a deficiency of erythropoietin. Initially the MCV is normal, and then decreases. Hepcidin regulates iron absorption. Hepcidin levels are low in anemia of chronic disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the most common cause of Sideroblastic anemia?

A

In general, the most common cause is alcohol’s suppressive effect on the bone marrow. Less common causes are lead poisoning, isoniazid, and vitamin B6 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What feature(s) in the Pt’s history would make Iron deficiency anemia the most likely diagnosis?

A

Blood loss (GI bleeding), Menstruation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What feature(s) in the Pt’s history would make Anemia of Chronic disease the most likely diagnosis?

A

Cancer or chronic infection, Rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What feature(s) in the Pt’s history would make Sideroblastic anemia the most likely diagnosis?

A

Alcoholic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the most common cause of microcytic anemia? What are the Sx?

A

Thalassemia. Most patients with thalassemia trait alone are asymptomatic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the best initial test when suspecting microcytic anemia?

A

Iron studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is the unique feature for Iron deficiency anemia on Iron studies?

A

Low ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the unique feature for Sideroblastic anemia on Iron studies?

A

High iron. It is the only form of microcytic anemia in which the circulating iron level is elevated.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the unique feature for Thalassemia Iron studies?

A

This is a genetic disease with normal iron studies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Why is it that a third of patients with Iron deficiency anemia have a normal or increased ferritin level?

A

Because ferritin is an acute phase reactant. Any counter current infection or inflammation can raise the ferritin level.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Both iron deficiency and the anemia of chronic disease are associated with a low serum iron level, how do you tell them apart?

A

Iron deficiency is associated with an increase in the total iron binding capacity (TIBC). In anemia of chronic disease, the body holds on to iron so circulating iron is decreased and TIBC is low.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Besides a low MCV and low ferritin, what other lab finding would point to Iron deficiency anemia?

A

Increased red cell distribution of width (RDW). This is because the newer cells are more iron deficient and smaller. As the body runs out of iron, the newer cells have less hemoglobin and get progressively smaller.
There is also an elevated platelet count (body thinks its bleeding)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the most accurate test for Iron deficiency anemia?

A

A bone marrow biopsy for stainable iron which is decreased. Rarely done.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the most accurate test for Sideroblastic anemia?

A

Prussian blue staining for ringed sideroblasts is the most accurate test. Basophilic stippling can occur in any cause of sideroblastic anemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the most accurate test for Thalassemia?

A

Hemoglobin electrophoresis is the most accurate test. But for alpha thalassemia, genetic studies are the most accurate test. Alpha thalassemia is diagnosed by DNA analysis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q 
What would be the clinical findings for Alpha thalassemia if the following findings were noted on Electrophoresis;
1 gene deletion
2 gene deletion
3 gene deletion
4 gene deletion
A

1 gene deletion- normal
2 gene deletion- normal electrophoresis, mild anemia
3 gene deletion- moderate anemia with hemoglobin H, which are beta-4 tetrads; **increased reticulocytes
4 gene deletion- gamma-4 tetrads or Bart’s hemoglobin;
CHF causes death in utero

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

How would you Tx Iron deficiency anemia?

A

Replace iron with oral ferrous sulfate. If this is insufficient, Tx with intramuscular iron.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

How would you Tx anemia of Chronic disease?

A

Correct the underlying disease. Only the anemia associated with end-stage renal failure routinely responds to erythropoietin replacement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

How would you Tx Sideroblastic anemia?

A

Correct the cause. Some patients respond to vitamin B6 or pyridoxine replacement. This is why isoniazid can lead to sideroblastic anemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

How would you Tx Thalassemia?

A

Trait is not treated. Beta thalassemia major (Cooley anemia) is managed with chronic transfusion lifelong. Iron overload is managed with deferasirox or deferiprone, oral iron chelators. Deferoxamine is a parenteral version of an iron chelator.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Which iron chelators are suitable for hemochromatosis resulting from transfusion?

A

Oral iron chelators- deferiprone and deferasirox

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

A 73-year-old man comes to the office with fatigue that has become progressively worse over the last several months. He is also short of breath when he walks up one flight of stairs. He drinks 4 vodka martinis a day. He has numbness and tingling in his feet. On physical examination he has decreased sensation of his feet. His hematocrit is 28% and his MCV is 114 fL (elevated).

What is the most appropriate next step in management?

a. Vitamin B12 level.
b. Folate level.
c. Peripheral blood smear.
d. Schilling test.
e. Methylmalonic acid level.

A

*C.
Although a macrocytic anemia could be from B12 or folate deficiency, direct alcohol effect on the bone marrow, or liver disease, the first step is a peripheral smear. This is to detect hypersegmented neutrophils. Once hypersegmented neutrophils are seen, then you would get B12 and folate levels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What is the relationship between high MCV Megaloblastic anemia and high MCV non-megaloblastic anemia?

A

Megaloblastic anemia is the presence of hypersegmented neutrophils. Many factors raise the MCV, but only B12 and folate deficiency and antimetabolite medications cause hypersegmentation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What are some of the causes of Vitamin B12 deficiency?

A
  • Pernicious anemia
  • Pancreatic insufficiency
  • Dietary deficiency (vegan/strict vegetarian)
  • Crohn disease, celiac, tropical sprue, radiation, or any disease damaging the terminal ileum
  • Blind loop syndrome (gastrectomy or gastric bypass for weight loss)
  • Diphyllobothrium latum, HIV, metformin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What are some of the causes of Folate deficiency?

A
  • Dietary deficiency (goat’s milk has no folate and provides only limited iron and B12)
  • Psoriasis and skin loss or turnover
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What would in the Pt’s history would suggest folate deficiency if the Pt has rheumatoid arthritis?

A

Methotrexate use

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What will make alcohol to be the most likely cause of macrocytic anemia and neurological problems?

A

Absence of hypersegmented neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is the most common neurological abnormality in a Pt with B12 deficiency?
What is the least common?

A

Peripheral neuropathy is the most common. Dementia is the least common.

Posterior column damage to position and vibratory sensation or “subacute combined degeneration” of the cord is classic. Look for ataxia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is the relationship between B12 deficiency, homocysteine levels, and methylmalonic acid
levels?

A

While both B12 and folate deficiency increase homocysteine levels, B12 deficiency is the only one associated with an increased methylmalonic acid levels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Can B12 and folate deficiency be Ddx hematologically or on blood smear?

A

No. B12 and folate deficiency are identical hematologically and on blood smear.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Why do B12 and folate deficiency have Low reticulocytes?

A

B12 and folate are essential for cell proliferation. Deficiencies may affect all cell lines (pancytopenia). Also Red cells are destroyed as they leave the marrow, so the reticulocyte count is low.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

A 73-year-old woman comes with decreased position and vibratory sensation of the lower extremities, a hematocrit of 28%, MCV of 114 fL, and hypersegmented neutrophils. Her B12 level is decreased, but near the borderline of normal.

What is the most appropriate next step in the management of this patient?

a. Methylmalonic acid level.
b. Anti-intrinsic factor antibodies.
c. Anti-parietal cell antibodies.
d. Schillings test.
e. Folate level.
f. Homocysteine level.

A

*A.
USMLE Step 2 CK frequently tests the fact that while both B12 and folate deficiency increase homocysteine levels, only B12 is associated with an increased MMA. The B12 level can be normal in as many as a third of patients with B12 deficiency because the carrier protein, transcobalamin, is an acute phase reactant and can be elevated from many forms of stress such as infection, cancer, or trauma. When the story suggests B12 deficiency and the B12 level is equivocal, use an increased MMA level to confirm the diagnosis of vitamin B12 deficiency.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What would in the Pt’s history would suggest B12 deficiency if the Pt has diabetes?

A

Use of Metformin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is Pernicious anemia?

How is the Dx confirmed?

A

a B12 anemia caused by lack of intrinsic factor in the stomach, resulting in decreased absorption.

It is confirmed with anti-intrinsic factor and anti-parietal cell antibodies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Why do B12 and folate deficiency present with High LDH and High bilirubin?

A

Because red cells are destroyed as they leave the marrow.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What is the relationship between pancreatic function and B12 deficiency?

A

Pancreatic enzymes are needed to absorb B12. They free it from carrier proteins (Pancreatic enzymes are needed to remove B12 from the R-protein so it can
bind with intrinsic factor).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

How would you Tx B12 and folate deficiency?

A

Replace what is deficient. Folate replacement corrects the hematologic problems of B12 deficiency, but not the neurological problems. so for folate deficiency replace both B12 and folate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Which of the following is a complication of B12 or folate replacement?

a. Seizures.
b. Hemolysis.
c. Hypokalemia.
d. Hyperkalemia.
e. Diarrhea.

A

*C
Extremely rapid cell production in the bone marrow causes hypokalemia. There is no other condition in which cells are generated so rapidly that they use up all the potassium. Hyperkalemia from massive tissue or cellular breakdown has many causes. Hypokalemia from cell production is rare. When replacing B12 and folate, particularly if there is pancytopenia, cells in the marrow are produced so rapidly that the marrow packages up all the potassium, lowering the serum level. Observe and replace.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

What are some of the clinical features seen in all forms of hemolysis (Hemolytic Anemia)?

A
  • Sudden decrease in hematocrit
  • Increased levels of LDH, indirect bilirubin, and reticulocytes
  • Decreased haptoglobin level
  • Slight increase in MCV because reticulocytes are larger than normal cells
  • Hyperkalemia from cell breakdown
  • Folate deficiency from increased cell production using it up; folate stores are limited
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

What causes Sickle Cell Disease?

A

A point mutation at position 6 of the beta globin chain: Valine replaces Glutamic acid.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

What causes acute painful vasoocclusive crisis in Sickle Cell Disease?

A
  • Hypoxia
  • Dehydration
  • Infection/fever
  • Cold temperatures
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

What set of clinical features in the presentation would make Sickle Cell Disease the most likely Dx?

A

An African American patient with sudden, severe pain in the chest, back, and thighs that may be accompanied by fever.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

What are some of the pathologies associated with Sickle Cell Disease?

A
  • Bilirubin gallstones from chronically elevated bilirubin levels
  • Increased infection from autosplenectomy, particularly encapsulated organisms
  • Osteomyelitis, most commonly from Salmonella
  • Retinopathy
  • Stroke
  • Lower extremity skin ulcers
  • Avascular necrosis of the femoral head (x-ray is the first test; MRI is most accurate)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

How do children with Sickle Cell Disease commonly present?

A

Dactylitis (inflammation of fingers)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is the best initial test when suspecting Sickle Cell Disease? How does it help Ddx with Sickle cell trait (AS disease)?

What is the most accurate test?

A

The best initial test is a peripheral smear. Sickle cell trait (AS disease) does not give sickled cells.

The most accurate test is the hemoglobin electrophoresis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

What in Mgx can help lower mortality in sickle cell disease?

A
  • Hydroxyurea in prevention

- Antibiotics with fever

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Which of the following can be found on smear in sickle cell disease?

a. Basophilic stippling.
b. Howell-Jolly bodies.
c. Bite cells.
d. Schistocytes.
e. Morulae.

A

*B.
These are precipitated remnants of nuclear material seen inside the red cells of a patient who does not have a spleen. There is no change in therapy or management based on the presence of Howell-Jolly bodies. Basophilic stippling is associated with a number of causes of sideroblastic anemia, especially lead poisoning. Bite cells are seen in glucose 6 phosphate dehydrogenase deficiency. Schistocytes are fragmented red cells seen with intravascular hemolysis. Morulae are seen inside neutrophils in Ehrlichia infections.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

How would you manage a bout of sickle cell disease?

A
  1. Begin with oxygen, hydration, analgesia.
  2. If fever or a white cell count higher than usual is present, then antibiotics are given. Use ceftriaxone, levofloxacin, or moxifloxacin.
  3. Folic acid replacement is necessary on a chronic basis.
  4. Give pneumococcal vaccination because of autosplenectomy.
  5. Hydroxyurea prevents recurrences of sickle cell crises by increasing
    hemoglobin F. Increase the dose of hydroxyurea until the hepatocyte growth
    factor (HGF) level rises above 10–15%. If WBC is low, do not increase
    the dose.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

If a Pt has sickle cell disease and a fever, should you wait for test results to confirm infection? Why?

A

No, don’t wait for results of testing to start antibiotics if there is a fever. The absence of a functional spleen leads to overwhelming infection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

In sickle cell disease, when is exchange transfusion indicated?

A

In severe vasoocclusive crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

A 43-year-old man with sickle cell disease is admitted with an acute pain crisis. His only routine medication is folic acid. His hematocrit on admission is 34%. On the third hospital day, the hematocrit drops to 22%.

What is the best initial test?

a. Reticulocyte count.
b. Peripheral smear.
c. Folate level.
d. Parvovirus B-19 IgM level.
e. Bone marrow.

A

A. Patients with sickle cell disease usually have very high reticulocyte counts because of the chronic compensated hemolysis. Parvovirus B-19 causes an aplastic crisis which freezes the growth of the marrow. Nothing will be visible on blood smear. Although the bone marrow will show giant pronormoblasts, this would not be done routinely, and certainly never as the initial test. The first clue to parvovirus is a sudden drop in reticulocyte level.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

What is the most accurate test for parvovirus B-19

What is the best
initial therapy?

A

PCR for DNA. This is
more accurate than the IgM level.

Intravenous immunoglobulins.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

What is Sickle Cell Trait and how does it present?

A

Sickle cell trait means the patient is heterozygous for the sickle gene (AS). They are clinically asymptomatic and have both a normal CBC level and a normal smear result.
The only manifestation of sickle cell trait is a defect in the ability to concentrate the urine or “isosthenuria.”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

What is Hereditary Spherocytosis?

A

It is a defect in the cytoskeleton of the red cell leading to an abnormal round shape and loss of the normal flexibility characteristic of the biconcave disc that allows red cells to bend in the spleen.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

What is the most accurate test to Dx Hereditary Spherocytosis?

A

Eosin-5-maleimide flow cytometry. It is more accurate than osmotic fragility testing (in which cells are placed in a slightly hypotonic solution, and the increased swelling of the cells leads to hemolysis).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

How would you Mgx Hereditary Spherocytosis?

A
  1. Chronic folic acid replacement supports red cell production.
  2. Splenectomy stops the hemolysis but does not eliminate the spherocytes.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

What are some of the causes of Autoimmune (Warm or IgG) Hemolysis?

A
  • Chronic lymphocytic leukemia (CLL)
  • Lymphoma
  • Systemic lupus erythematosus (SLE)
  • Drugs: penicillin, alpha-methyldopa, rifampin, phenytoin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

What is the most accurate diagnostic test for Autoimmune (Warm or IgG) Hemolysis?

A

Coombs test, which detects IgG antibody on the surface of the red cells. The direct and indirect Coombs tests tell basically the same thing, but the indirect test is associated with a greater amount of antibody.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

What is the relationship between autoimmune hemolysis and spherocytes?

A

Autoantibodies remove small amounts of red cell membrane and lead to a smaller membrane, forcing the cell to become round. Biconcave discs need a greater surface area than a sphere. Autoimmune hemolysis is associated with microspherocytes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Why doesn’t the smear show fragmented cells in autoimmune hemolysis?

A

Because the red cell destruction occurs inside the spleen or liver, not in the blood vessel.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

How would you Mgx autoimmune hemolysis?

A
  1. Glucocorticoids such as prednisone are the “best initial therapy.”
  2. Recurrent episodes respond to splenectomy.
  3. Severe, acute hemolysis not responding to prednisone is controlled with intravenous immunoglobulin (IVIg). [IVIg will bind on to all the receptors on the macrophages that are supposed to destroy the RBC’s with autoimmune antibodies]
  4. Rituximab, azathioprine, cyclophosphamide, or cyclosporine is used when splenectomy does not control the hemolysis. ADAMTS 13 level is low in
    TTP.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

What is Cold Agglutinin Disease?

A

It is a rare type of autoimmune hemolytic anemia characterized by the presence of high concentrations of circulating antibodies, usually IgM, directed against red blood cells, causing them to agglutinate and undergo lysis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

Which conditions/ diseases are associated with Cold Agglutinin Disease?

A

Epstein-Barr virus, Waldenström macroglobulinemia, or Mycoplasma pneumoniae.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

What is the clinical presentation of Cold Agglutinin Disease?

A

Symptoms occur in colder parts of the body such as numbness or mottling of the nose, ears, fingers, and toes. Symptoms resolve on warming up the body part.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

How would you Dx Cold Agglutinin Disease?

A

The direct Coombs test is positive only for complement. The smear is normal, or may show only spherocytes. Cold agglutinin titer is the most accurate test.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

How would you Mgx Cold Agglutinin Disease?

A
  1. Keep the patient warm.
  2. Administer rituximab and sometimes plasmapheresis.
  3. Cyclophosphamide, cyclosporine, or other immunosuppressive agents stop the production of the antibody.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

What is your opinion on the use of steroids and or splenectomy in Cold Agglutinin Disease?

A

Steroids and splenectomy do not work in cold agglutinin disease.

83
Q

What are some of the similarities and differences between Cryoglobulins and Cold agglutinins?

A

Both are IgM and do not respond to steroids, cryoglobulins are associated with:

  • Hepatitis C
  • Joint pain
  • Glomerulonephritis
84
Q

What is Glucose 6 phosphate dehydrogenase (G6PD) deficiency?

A

It is an X-linked recessive disorder leading to an inability to generate glutathione reductase and protect the red cells from oxidant stress. The most common oxidant stress is infection.

85
Q

What features would make Glucose 6 phosphate dehydrogenase (G6PD) deficiency the most likely Dx?

A

An African American or Mediterranean men with sudden anemia and jaundice who have a normal-sized spleen with an infection or taking dapsone, quinidine, sulfa drugs, primaquine, nitrofurantoin or fava beans.

86
Q

What is the best initial test for Glucose 6 phosphate dehydrogenase (G6PD) deficiency?

What is the most accurate test?

A

The best initial test is for Heinz bodies and bite cells. The G6PD level will be normal after a hemolytic event.

The most accurate test is the G6PD level after waiting 1 to 2 months after an acute episode of hemolysis.

87
Q

How would you Tx Glucose 6 phosphate dehydrogenase (G6PD) deficiency?

A

Nothing reverses the hemolysis. Avoid oxidant stress.

88
Q

What are some of the commonalities between Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)?

A

-Both are caused by deficiency of metalloproteinase ADAMTS 13.
-HUS and TTP both
have normal PT/aPTT

Both disorders are 
characterized by:
-Intravascular hemolysis with fragmented red cells (schistocytes)
-Thrombocytopenia
-Renal insufficiency
89
Q

What are some of the differences between Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)?

A

HUS is associated with E. coli 0157:H7 and is more frequent in children. TTP is associated with ticlopidine, clopidogrel, cyclosporine, AIDS, and SLE.

90
Q

Which of the following can be used in Tx of Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP):

  • plasmapheresis
  • platelets
  • FFP
A

Plasmapheresis or FFP.

Do not transfuse platelets into patients with HUS or TTP. Platelet transfusion worsens the disease.

91
Q

Explain the pathophysiology in Paroxysmal Nocturnal Hemoglobinuria

A

CD55/CD59 proteins found on the surface of RBCs protect them from complement-mediated hemolysis. PNH is a deficiency in GPI-anchor molecules that keep CD55/CD59 attached to cells, resulting in hemolysis by complement. PNH it leads to hemolysis and thrombosis.

92
Q

What clinical picture would make Paroxysmal nocturnal hemoglobinuria (PNH) the most likely diagnosis?

A
  • Episodic dark urine
  • Clots in unusual places (not just DVT or pulmonary embolism)
  • Pancytopenia
93
Q

What is the relationship between Paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia, myelodysplasia
or acute leukemia?

A

PNH is a stem cell defect that may cause aplastic anemia, myelodysplasia, or acute leukemia.

94
Q

What is the most accurate way to Dx Paroxysmal nocturnal hemoglobinuria (PNH)?

A

Level of CD55 and CD59 (Decreased).
Flow cytometry is another way of saying CD55/CD59 testing.
The Ham test and the sucrose hemolysis test are obsolete.

95
Q

How would you Mgx Paroxysmal nocturnal hemoglobinuria (PNH)?

A
  1. Prednisone is the best initial therapy for hemolysis. The mechanism is not clear.
  2. Allogeneic bone marrow transplant is the only method of cure.
  3. Eculizumab inactivates C5 in the complement pathway and decreases red cell destruction. Eculizumab is, essentially, a complement inhibitor. Eculizumab is for hemolysis and thrombosis.
  4. Give folic acid and replacement with transfusions as needed.
96
Q

What is the most

common site of thrombosis in Paroxysmal nocturnal hemoglobinuria (PNH)?

A

Large vessel thrombosis of the mesenteric and hepatic veins.

97
Q

What is Aplastic Anemia and what is it’s etiology?

A

Aplastic anemia is pancytopenia of unclear etiology. Any infection or cancer can invade the bone marrow, causing decreased production or hypoplasia.

98
Q

What are some of the causes of pancytopenia?

A
  • Radiation and toxins such as toluene, insecticides (DDT)
  • Drug effect: sulfa, phenytoin, carbamazepine, chloramphenicol, alcohol, chemotherapy, Thyroid-inhibiting medications such as propylthiouracil (PTU) and methimazole
  • SLE
  • PNH
  • Infection: HIV, hepatitis, CMV, EBV
  • B12 and folate deficiency
99
Q

How would you Dx Aplastic anemia?

A

Aplastic anemia is confirmed by excluding all the causes of pancytopenia. The most accurate test is a bone marrow biopsy.

100
Q

How would you Mgx Aplastic anemia?

A

Supportive therapy such as blood transfusion for anemia, antibiotics for infection, and platelets for bleeding.
Treat any underlying cause that is identified.
If Pt is <50 and there is a matched donor=> allogeneic bone marrow transplantation (BMT)
If Pt is >50 and there is no matched donor=> Antithymocyte globulin (ATG) and cyclosporine.

101
Q

What is Polycythemia Vera?

A

Polycythemia vera (p. vera) is the unregulated overproduction of all 3 cell lines, but red cell overproduction is the most prominent. There is a mutation in the JAK2 protein which regulates marrow production.

102
Q

What clinical features would make Polycythemia Vera the most likely Dx?

A

Symptoms of hyperviscosity from the increased red blood cell mass such as:

  • Headache, blurred vision, and tinnitus
  • Hypertension
  • Fatigue
  • Splenomegaly
  • Bleeding from engorged blood vessels
  • Thrombosis from hyperviscosity
103
Q

In Polycythemia Vera, why does a warm shower result in pruritus?

A

Because of histamine release from increased numbers of basophils.

104
Q

What is the accurate Dx test for Polycythemia Vera?

A

the JAK2 mutation, found in 95% of patients.

105
Q

How would you Mgx Polycythemia Vera?

A
  1. Phlebotomy and aspirin prevent thrombosis
  2. Hydroxyurea helps lower the cell count
  3. Allopurinol or rasburicase protects against uric acid rise
  4. Antihistamines
106
Q

When Mgx Polycythemia Vera, if hydroxyurea fails, what is the next best step?

A

Use Ruxolitinib. It’s an inhibitor of JAK 2.

107
Q

What is Essential thrombocytosis (ET)?

A

It is a markedly elevated platelet count above one million leading to both thrombosis and bleeding. Essential thrombocythemia belongs to a group of conditions called myeloproliferative disorders.

108
Q

How would you Mgx Essential thrombocytosis (ET)?

A

If Pt <60 + asymptomatic with a platelet count under 1.5 million=> no treatment

If Pt>60 + symptomatic or platelet count above 1.5 million=> hydroxyurea

*Anagrelide is used when there is red cell
suppression from hydroxyurea

109
Q

What is Myelofibrosis?

A

Is a myeloproliferative neoplasm leading to bone marrow fibrosis and extramedullary hematopoiesis from the spleen and liver, which become markedly enlarged.

110
Q

How would you Mgx Myelofibrosis?

A

Curative:
Allogeneic stem cell transplantation(option for younger patients)

Supportive:
Hyperproliferative phase
-Prevent thromboembolisms: antiplatelet drug (aspirin 100 mg)
-Control cell count: hydroxyurea, interferon alpha, cladribine

Pancytopenic phase
-JAK2 inhibitor: ruxolitinib
Periodic transfusions
-Low-dose thalidomide plus glucocorticoids

111
Q

What do you expect to see after doing a Peripheral blood smear and Bone Marrow aspiration

A

Peripheral blood smear=> Dacrocytes (teardrop-shaped cells)

Bone Marrow aspiration=> Punctio Sicca (Dry tap- also seen in hairy cell leukaemia and aplastic anemia)

112
Q

What are the general clinical and lab features of Acute Leukemias?

A

Patients present with signs of pancytopenia (fatigue, infection, bleeding) even though the white blood cell count is normal or increased in many patients. Despite an increase in white cell count, infection is a common presentation because leukemic cells (blasts) do not function normally in controlling infection.

113
Q

What is the best initial test for Acute Leukemias? What does it show?

A

Blood smear showing blasts. M3 or acute promyelocytic leukemia is most commonly associated with Auer rods (eosinophilic inclusions).

114
Q

What is the most accurate test for Acute Leukemias?

A

The most accurate test is flow cytometry, which will distinguish the different subtypes of acute leukemia. Flow cytometry is the method of detecting the specific CD subtypes associated with each type of leukemia.

115
Q

How would you Mgx Acute Leukemias?

A

Both AML and ALL are treated initially with chemotherapy to remove blasts from the peripheral blood smear. This is known as inducing remission.

  • If prognosis is poor, then go straight to BMT
  • if prognosis is good, give more chemotherapy.
  • Add all-trans-retinoic acid (ATRA) to those with M3 (promyelocytic leukemia).
  • Add intrathecal chemotherapy such as methotrexate to ALL treatment. This prevents relapse of ALL in the CNS.
116
Q

What is the best indicator of prognosis in acute leukemia?

A

Cytogenetics.
Good Cytogenetics= less chance of relapse= more chemotherapy

Bad Cytogenetics= more chance of relapse= immediate BMT

117
Q

What clinical features would make Chronic Myelogenous Leukemia the most likely Dx?

A
  • A patient with a persistently high WBC count that is all neutrophils.
  • *generally there is proliferation of all granulocytes
  • Pruritus is common after hot baths/showers from histamine release from basophils.
  • Splenomegaly presents with early satiety, abdominal fullness, and left upper quadrant pain.
118
Q

What is the most accurate test to Dx Chronic Myelogenous Leukemia?

A

BCR-ABL, which can be done by PCR or FISH (fluorescent in-situ hybridization) on peripheral blood.

119
Q

What is the Philadelphia chromosome and what is it’s relevance?

A

It is a chromosomal translocation of chromosomes 9 and 22 [T(9:22)], it then produces the BCR-ABL protein which is implicated in the pathogenesis of several leukaemias, most notably CML

120
Q

What is the best initial therapy in Chronic Myelogenous Leukemia?

A

A tyrosine kinase inhibitors=> imatinib

121
Q

What is the most effective cure for Chronic Myelogenous Leukemia?

A

A BMT can cure CML, but this should never be the first therapy.

122
Q

Which myeloproliferative disorder is the most likely to transform into acute leukemia?

A

CML

123
Q

What are the clinical phases of Chronic Myelogenous Leukemia?

A

3 Phase:
1. The chronic phase is characterized by nonspecific symptoms (fever, weight loss, night sweats) and splenomegaly and can persist for up to 10 years.
=> Blast count in Peripheral blood and bone marrow- <10%

  1. The accelerated phase is characterized by complications secondary to the suppression of the other cell lines (thrombocytopenia, anemia, recurrent infections).
    => Blast count in Peripheral blood and bone marrow- 10-20%
  2. The blast crisis, resembles that of acute leukemia.
    => Blast count in Peripheral blood and bone marrow- >20%
124
Q

What is a Leukostasis Reaction?

A

Leukostasis (also called symptomatic hyperleukocytosis) is a medical emergency most commonly seen in patients with acute myeloid leukemia. It is characterized by an extremely elevated blast cell count and symptoms of decreased tissue perfusion.

125
Q

A 54-year-old man comes to the emergency department for shortness of breath, blurry vision, confusion, and priapism. His WBC count is found to be 225,000/μL. The cells are predominantly neutrophils with about 4% blasts.

What is the most appropriate next step in the management of this case?

a. Leukapheresis.
b. BCR-ABL testing.
c. Bone marrow biopsy.
d. Bone marrow transplant.
e. Consult hematology/ oncology.
f. Flow cytometry.
g. Hydroxyurea.

A

*A
In acute leukostasis reaction, it is more important to remove the excessive white cells from the blood than to establish a specific diagnosis. Specific testing is not as important as treatment. No matter what the etiology, you still have to take the cells off. The symptoms are caused by blocking the delivery of oxygen to tissues because the red cells simply cannot get to the tissues. Afterward, you can establish a specific diagnosis. Hydroxyurea will lower the cell count, but not as rapidly as leukapheresis.

126
Q

What is Myelodysplastic Syndrome (MDS)?

A

Myelodysplastic syndrome (MDS) are a group of cancers in which immature blood cells in the bone marrow do not mature, leading to pancytopenia despite a hypercellular bone marrow.

127
Q

What value does a 5q deletion have with Myelodysplastic Syndrome (MDS)?

A

A 5q deletion is the characteristic abnormality of MDS. Patients with 5q have a better prognosis than do those without it.

128
Q

What are some of the Sx that occur with Myelodysplastic Syndrome (MDS)?

A
  • Fatigue and weight loss
  • Infection
  • Bleeding
  • Sometimes splenomegaly
  • Severity is based on the percentage of blasts
129
Q

In Myelodysplastic Syndrome (MDS), what do you expect to see on a CBC, smear and Bone marrow smear?

A

Anemia with an increased MCV, nucleated red cells, and a small number of blasts.

  • Marrow: hypercellular
  • Prussian blue stain shows ringed sideroblasts
  • Pelger-Huet cells- cells with bilobed nucleus (*the most distinct lab abnormality in MDS)
130
Q

What factors will influence the therapeutic approach to a Pt with Myelodysplastic Syndrome (MDS)?

A

The therapeutic approach depends on a patient’s presentation, age, and comorbidities. More aggressive therapy (e.g. chemotherapy, stem cell transplantation) is generally reserved for younger (<50), healthier patients.

131
Q

How would you Mgx a Pt with Myelodysplastic Syndrome (MDS)?

A
  1. Mild cytopenia: “watch and wait”
  2. Severe cytopenia:
    Supportive treatment
    Mainstay of treatment- RBC and platelet transfusions depending on cell counts.
    To compensate for the high cell turnover- vitamin supplementation (Vitamin B6, B12, folate).
    In cases of symptomatic anemia and low erythropoietin (EPO) levels- synthetic EPO
    In cases of neutropenia- granulocyte colony stimulating factor
    If infection occurs- antibiotics

Medical therapy:
Chemotherapy- If myeloblasts are elevated
Immunosuppressive agents
Lenalidomide (for those with the 5q deletion can decrease transfusion
dependence.)
Azacitidine (decreases transfusion dependence and increases survival in
MDS.)

Allogenous stem cell transplant is the only curative option.

132
Q

What is Chronic Lymphocytic Leukemia?

A

Chronic lymphocytic leukemia (CLL) belongs to the group of low-grade non-Hodgkin lymphomas (NHL) and is a B-cell lymphoma that presents with lymphocytic leukocytosis.

133
Q

What is Richter’s phenomenon?

A

It is the conversion of CLL into high-grade lymphoma, happens in 5% of patients.

134
Q

What is the mechanism by which infections, anemia and thrombocytopenia occur in Chronic Lymphocytic Leukemia?

A

They occur from marrow infiltration or autoimmune warm IgG antibodies. CLL is paradoxical. When the body needs a useful antibody for an infection, it is often not made; on the other hand, the CLL cells attack normal red cells and platelets.

135
Q

What is a smudge cell?

A

It is a lab artifact in which the fragile nucleus is crushed by the cover slip of a blood smear. It is not exclusive to CLL but a % > 10% is generally seen as pathognomonic for CLL

136
Q

What is the Rai Staging?

A 
A classification used to assess the prognosis in Pt with CLL based on clinical features.
0- Isolated lymphocytosis
I- Lymphadenopathy
II- Hepatomegaly and or splenomegaly 
III- Anemia
IV- Thrombocytopenia
137
Q

How would you Mgx Chronic Lymphocytic Leukemia (CLL)?

A

For stage 0 (elevated WBC), stage I (lymphadenopathy), and stage II (hepatosplenomegaly), there is no treatment.
Stage III (anemia) and stage IV (thrombocytopenia) are treated with fludarabine, cyclophosphamide, and rituximab.
*ibrutinib is a new drug approved by the FDA

  • Severe infection: intravenous immunoglobulins
  • Autoimmune thrombocytopenia or hemolysis: prednisone
138
Q

Which is less dangerous? Thrombocytopenia and anemia from autoimmune effect, or from marrow infiltration with CLL cells?

A

Autoimmune effect is less dangerous. This is treated with prednisone, and is not the same as stage III and IV CLL.

139
Q

What is Hairy Cell Leukemia?

A

It is a chronic lymphoid leukemia named after the hairlike cytoplasmic projections seen on the surface of the abnormal B-cells

140
Q

What is the best initial test to Dx Hairy cell leukemia (HCL)?

What is the most accurate test?

A

A smear showing hairy cells.

Immunotyping by flow cytometry (e.g., CD11c).

141
Q

How would you Tx Hairy cell leukemia (HCL)?

A

cladribine or pentostatin

142
Q

What is Non-Hodgkin Lymphoma (NHL)?

A

It is a proliferation of lymphocytes in the lymph nodes and spleen. NHL is most often widespread at presentation and can affect any lymph node or organ that has lymphoid tissue.

143
Q

What is the relationship bwtween Non-Hodgkin Lymphoma (NHL) and Chronic Lymphocytic Leukemia (CLL)?

A

NHL and CLL are extremely similar, but NHL is a solid mass and CLL is “liquid” or circulating.

144
Q

What clinical features would make Non-Hodgkin Lymphoma (NHL) the most likely Dx?

A
  • Painless lymphadenopathy
  • Nodes not warm, red, or tender
  • “B” symptoms: fever, weight loss, drenching night sweats
145
Q

What is the difference between the lymphadenopathy in Non-Hodgkin Lymphoma (NHL) and that in an Infection?

A

Infections, not NHL, would give nodes that are warm, red, and tender.

146
Q

What is the best initial test when suspecting Non-Hodgkin Lymphoma (NHL)?

A

Excisional biopsy not a needle aspiration of the lymph node because individual lymphocytes appear normal.

147
Q

What changes would you expect to see on a CBC in a Pt with Non-Hodgkin Lymphoma (NHL)?

A

The CBC is normal in most cases.

148
Q

How is Non-Hodgkin Lymphoma (NHL) staged?

A

Stage I: 1 lymph node group
Stage II: 2 or more lymph node groups on the same side of the diaphragm
Stage III: both sides of the diaphragm
Stage IV: widespread disease

149
Q

How is Non-Hodgkin Lymphoma (NHL) Mgx according to the stages?

A

Local disease (stage Ia and IIa): local radiation and small dose/course of chemotherapy.

Advanced disease (stage III and IV, any “B” symptoms): combination
chemotherapy with CHOP and rituximab, an antibody against CD20
NHL.
C = cyclophosphamide
H = adriamycin (doxorubicin)
O = vincristine (oncovin)
P = prednisone

150
Q

What is Mucosal Associated Lymphoid Tissue (MALT)?

A

It is also called MALToma or extranodal marginal zone lymphoma. It is a B-cell non-Hodgkin lymphoma (NHL) that typically affects elderly patients in the 7th and 8thdecades. MALTomas can be categorized according to their location as either gastric or nongastric. Gastric MALTomas are frequently associated with Helicobacter pylori (H. pylori) infection, whereas nongastric MALTomas are rather associated with autoimmune conditions (e.g., Sjögren syndrome, Hashimoto’s thyroiditis).

151
Q

How would you Tx Mucosal Associated Lymphoid Tissue (MALT)?

A

if Gastric MALToma=> Treat the Helicobacter with clarithromycin and amoxicillin.

if Nongastric MALToma=> depending on exact type and stage- chemo, radiation and surgery

152
Q

What are some of the differences between Hodgkin disease (HD) and Non-Hodgkin lymphoma (NHL)

A

HD- Mostly discovered in stage I, and stage II in 80%–90%
NHL- Mostly discovered in Stage III and stage IV in 80%–90%

HD- Centers around cervical area
NHL-Disseminated

HD- Reed-Sternberg cells on pathology
NHL- No Reed-Sternberg cells

153
Q

How would you Tx Hodgkin disease (HD)?

A

Stage Ia and IIa: local radiation with a small course of chemotherapy
Stage III and IV or anyone with “B” symptoms: ABVD
A= adriamycin (doxorubicin)
B= bleomycin
V= vinblastine
D= decarbazine

154
Q

What the 2 main complications of Radiation?

A

It increases the risk of solid tumors such as breast, thyroid, or lung cancer. Screening for breast cancer is recommended 8 years or more after treatment. Radiation also increases the chance of premature coronary artery disease.

155
Q

Which of the following is the most useful to determine dosing of chemotherapy in Hodgkin disease (HD)?

a. Echocardiogram.
b. Bone marrow biopsy.
c. Gender.
d. MUGA or nuclear ventriculogram.
e. Hematocrit.
f. Symptoms.

A

*D
Adriamycin (or doxorubicin) is cardiotoxic. The nuclear ventriculogram is the most accurate method of assessing left ventricular ejection fraction. Use the MUGA scan to determine whether cardiac toxicity has occurred prior to the development of symptoms. You can’t use adriamycin if the ejection fraction is less than 50%.

156
Q

What are the adverse effects of the following Chemotherapeutic agents;

  1. Adriamycin (or doxorubicin)
  2. Vincristine
  3. Bleomycin
  4. Cyclophosphamide
  5. Cisplatin
A
  1. Adriamycin (or doxorubicin)- Cardiomyopathy
  2. Vincristine- Neuropathy
  3. Bleomycin- Lung fibrosis
  4. Cyclophosphamide- Hemorrhagic cystitis
  5. Cisplatin- Renal and ototoxicity
157
Q

What is Multiple Myeloma?

A

It is a malignant plasma cell dyscrasia characterized by uncontrolled proliferation and the diffuse infiltration of monoclonal (=same type) plasma cells in the bone marrow. Malignant plasma cells generally produce monoclonal proteins(also known as M proteins or paraproteins), such as abnormal antibodies (e.g., IgG or IgA) or immunoglobulin light chains (e.g., Bence Jones protein). **But not IgM. A separate disease called Waldenström macroglobulinemia produces IgM.
**These immunoglobulins do not fight infection but clog up the kidney.

158
Q

What clinical features would make Multiple Myeloma the most likely Dx?

A

Anemia, bleeding and Infections: Proliferating plasma cells suppress normal bone marrow function, which leads to clinical findings of anemia, bleeding and/or infection.

Hypercalcemia and bone pain: plasma cell proliferation may result in extensive skeletal destruction (pathologic
fractures) with hypercalcemia.
A pathologic fracture means that the bone breaks under what would be considered normal use.

Renal failure: from accumulation of immunoglobulins and Bence-Jones protein in the kidney; hypercalcemia and hyperuricemia also damage the kidney

159
Q

1) What are the 2 best initial test when suspecting Multiple Myeloma?
2) What is the confirmatory test?

A

1) The first test done is usually an x-ray of the affected bone that will show lytic (“punched out”) lesions.

Serum protein electrophoresis (SPEP) shows an IgG (60%) or IgA (25%) spike of a single type or “clone.” This one clone is called a Monoclonal or “M” spike.

2) Bone marrow biopsy: greater than 10% plasma cells defines myeloma

160
Q

What common finding would you expect to see on a peripheral blood smear of a Pt with Multiple Myeloma?

A

Rouleaux formation- RBC’s stacked like coins due to Ig’s triggering RBC aggregation.

161
Q

What happens to the anion gap in Multiple Myeloma?

A

Myeloma has a decreased anion gap. IgG is cationic. Increased cationic substances will increase chloride and bicarbonate levels. This decreases the anion gap.

162
Q

What is the explanation for the difference between the urinary level of protein on urinalysis and the 24-hour urine?

a. False positive 24-hour urine is common in myeloma.
b. Calcium in urine creates a false negative urinalysis.
c. Uric acid creates a false positive 24-hour urine.
d. Bence-Jones protein is not detected by dipstick.
e. IgG in urine inactivates the urine dipstick.

A

*D

Bence-Jones protein is detected by urine immunoelectrophoresis. The urine dipstick will detect only albumin.

163
Q

What is the single most accurate test for myeloma?

a. Skull x-rays.
b. Bone marrow biopsy.
c. 24-hour urine.
d. SPEP.
e. Urine immunoelectrophoresis (Bence-Jones protein).

A

*B
Nothing besides myeloma is associated with greater than 10% plasma cells on bone marrow biopsy. The most common wrong answer is SPEP. Of those with an “M-spike” of immunoglobulins, 99% do not have myeloma. Most IgG spikes are from monoclonal gammopathy of unknown significance that does not progress or need treatment. Skull x-rays show lytic lesions, but this is not as specific as massive plasma cell levels in the marrow.

164
Q

What is the best initial therapy for Multiple Myeloma?

A

A combination of dexamethasone with lenalidomide or bortezomib or both.

The most effective therapy in those under age 70 is an autologous bone marrow transplant with stem cell support. This is used after induction chemotherapy with lenalidomide and steroids.

165
Q

What is Monoclonal gammopathy of unknown significance (MGUS)?

A

It is a condition in which an abnormal immunoglobin protein (known as a paraprotein) is found in the blood during standard laboratory blood tests.
(MGUS) has small numbers of plasma cells.
No Tx required- watch and wait

166
Q

What is Waldenström Macroglobulinemia?

A

It is a type of Non-Hodgkin Lymphoma associated with the overproduction of IgM from malignant B cells leading to hyperviscosity.

167
Q

What are some of the clinical features of Waldenström Macroglobulinemia?

A 
Due to hyperviscosity;
Blurry vision and vertigo
Engorged blood vessels in the eye
Mucosal bleeding
Raynaud phenomenon
168
Q

What is the best initial therapy for Waldenström Macroglobulinemia?

What is the long-term Tx?

A

Plasmapheresis to remove the IgM and decrease viscosity.

Long-term treatment is with rituximab or prednisone cyclophosphamide.

169
Q

What is the first step when evaluating Bleeding Disorder?

A

Determining if the bleeding seems

related to platelets or clotting factors.

170
Q

What are some of the similarities and differences between Platelet bleeding and Factor bleeding?

A

Platelet bleeding- Superficial (Epistaxis, gingival, petechiae, purpura, mucosal surfaces such as the gums, vaginal bleeding)

Factor bleeding- Deep (Joints and muscles)

Bleeding in the brain or the gastrointestinal system can be from either platelet or clotting factor deficiency.

171
Q

What clinical features would make Immune Thrombocytopenic Purpura (ITP) the most likely Dx?

A
  • Isolated thrombocytopenia (normal hematocrit, normal WBC count)
  • Normal-sized spleen
172
Q

A 23-year-old woman comes to the emergency department with markedly increased menstrual bleeding, gum bleeding when she brushes her teeth, and petechiae on physical examination. Physical examination is otherwise normal. The platelet count is 17,000/μL.

What is the most appropriate next step in therapy?

a. Bone marrow biopsy.
b. Intravenous immunoglobulins.
c. Prednisone.
d. Antiplatelet antibodies.
e. Platelet transfusion.

A

*C
The bleeding in this case is mild, meaning there is no intracranial bleeding or major GI bleeding, and the platelet is not profoundly low. Prednisone is the best initial therapy. Initiating prednisone is more important than checking for increased megakaryocytes or the presence of antiplatelet antibodies, which is characteristic of ITP. Bone marrow is rarely needed.

173
Q

Besides Isolated thrombocytopenia what else do we see on the CBC in Immune Thrombocytopenic Purpura (ITP)?

A
  • Megakaryocytes are elevated in number.

- Increase in mean platelet volume

174
Q 
What would be the best initial therapy in each of the following situations and platelet counts; 
1. No bleeding, count >30,000
2. Mild bleeding, count <30,000
3. Severe bleeding
(GI/CNS), count <10,000
4. Recurrent episodes, steroid dependent
5. Splenectomy or steroids not effective
A
  1. No treatment
  2. Glucocorticoids
  3. IVIG, Anti-Rho (anti-D)
  4. Splenectomy
  5. Romiplostim or eltrombopag, rituximab, azathioprine, cyclosporine, mycophenolate
175
Q

What should be done before a splenectomy is done?

A

Give vaccination to:

  • Neisseria meningitidis
  • Haemophilus influenzae
  • Pneumococcus
176
Q

What is Von Willebrand Disease (VWD)?

A

It is a disorder of hemostasis characterised by the level or functioning of von Willebrand factor (VWF).

177
Q

What clinical features would make Von Willebrand Disease (VWD) the most likely Dx?

A

bleeding related to platelets (epistaxis, gingival, gums) with a normal platelet count. VWD is markedly worsened after the use of aspirin.

  • Prolonged Bleeding time and sometimes aPTT
  • Low Factor VIII
  • Low VWF (antigen) level
178
Q

What is the best initial Tx for Von Willebrand Disease (VWD)?

A

DDAVP (desmopressin), which releases subendothelial stores of VWF.

179
Q

When Tx Von Willebrand Disease (VWD), if the Pt is not responding to desmopressin, what is the next best step?

A

Use factor VIII

replacement or VWF concentrate.

180
Q

What clinical features would make Hemophilia the most likely Dx?

A

Joint or muscle bleeding in a male child, since the condition is X-linked recessive.
A normal PT with
a prolonged aPTT.

181
Q

What is the relationship between Factor XI Deficiency and Hemophilia?

A

Factor XI deficiency is also called hemophilia C. It differs from hemophilia A or B in that there is no bleeding into joints and muscles.

182
Q

If a mixing study is done for a Pt who is bleeding, what do we expect if the bleeding is related to deficiency of clotting factors?

A

A correction of the aPTT to normal.

183
Q

What are the features of Factor XII Deficiency in terms of aPTT and bleeding time?

A

Elevated aPTT but paradoxically there is no bleeding. No therapy is
needed.

184
Q

What is the Tx for Factor XII Deficiency and why is it different from that of factors VII, VIII, IX, and X?

A

Fresh frozen plasma is the Tx. Because recombinant versions of factors VII, VIII, IX, and X are available.

185
Q

What recombinant therapy can be used for both for factor VIII deficiency and Von Willebrand’s disease (VWD)?

A

DDAVP (Desmopressin)

186
Q

What recombinant therapy is used for Von Willebrand’s disease (VWD)?

A

Recombinant VWF

187
Q

How would you Mgx warfarin toxicity?

A

Prothrombin complex concentrate (PCC). PCC has all the vitamin K dependent factors and works faster than giving either vitamin K or fresh frozen plasma (FFP). PCC has factors II, VII, IX, and X and proteins C and S.

188
Q

What are some of the risk factors to look for when considering Disseminated intravascular coagulation (DIC)?

A

Sepsis
Burns
Abruptio placentae or amniotic fluid embolus
Snake bites
Trauma resulting in tissue factor release
Cancer

189
Q

In Disseminated intravascular coagulation (DIC) what is the bleeding related to clotting factor deficiency or thrombocytopenia?

A

The bleeding is related to both clotting factor deficiency as well as
thrombocytopenia.

190
Q

What clinical features (lab) do you expect to see in a Pt with Disseminated intravascular coagulation (DIC)?

A

Elevation in both the PT and aPTT
Low platelet count
Elevated d-dimer and fibrin split products
Decreased fibrinogen level (it has been consumed)

191
Q

How would you Mgx Disseminated intravascular coagulation (DIC)?

A

If platelets are under 50,000/μL and the patient has serious bleeding, replace platelets as well as clotting factors by using FFP.
Heparin has no definite benefit.
Cryoprecipitate may be effective to replace fibrinogen levels if FFP does not control bleeding.

192
Q

What is the most common cause of Hypercoagulable States/Thrombophilia?

A

Factor V Leiden mutation.

193
Q

What is the best initial therapy for Hypercoagulable States/Thrombophilia?

A

Use warfarin to an INR of 2 to 3 for 6 months.

194
Q

Give a brief account of Heparin-induced thrombocytopenia (HIT), it’s causes and onset?

Which circulatory system does it affect, arterial or venous?

A

is more common with the use of unfractionated heparin, but can still occur with low molecular weight heparin. HIT presents 5 to 10 days after the start of heparin with a marked drop in platelet count (more than 30%). Both venous and arterial thromboses can occur, although venous clots are more common.

195
Q

Which test(s) would confirm Heparin-induced thrombocytopenia (HIT)?

A

An ELISA for platelet factor 4 (PF4) antibodies or the serotonin release assay.

196
Q

How would you Mgx Heparin-induced thrombocytopenia (HIT)?

A
  1. Immediately stop all heparin-containing products. You cannot just switch unfractionated heparin to low molecular weight heparin.
  2. Administer direct thrombin inhibitors: argatroban, bivalirudin, and fondaparinux. Fondaparinux is easier to use.
  3. Warfarin should not be used first, but after a direct thrombin inhibitor is started, use warfarin. Fondaparinux is safe.
197
Q

What is your opinion on transfusing platelets into those with Heparin-induced thrombocytopenia (HIT)?

A

Don’t, it may worsen the thrombosis.

198
Q

What is Antiphospholipid Syndrome?

A

It is an autoimmune disease that increases the risk of risk of thrombosis as a result of procoagulatory antibodies. It maybe primary or secondary.

199
Q

What are some the causes of Antiphospholipid Syndrome?

A 
Primary:
-associated with genetic marker HLA-DR7
Secondary:
-SLE
-Neoplasms
-Infections- HIV, Hepatitis, Syphilis &amp; TB
-Rheumatoid arthritis
200
Q

When should you suspect Antiphospholipid Syndrome?

A

History of thrombosis and or multiple spontaneous abortions

201
Q

What is the best initial test for Antiphospholipid Syndrome?

What is the most accurate test?

A

Mixing study- the aPTT will remain elevated even after the mix, because it is a circulating inhibitor.

Russell viper venom test is the most accurate test.

202
Q

What are the antiphospholipid antibodies and why are they not the best initial step or the most accurate tests to Dx Antiphospholipid Syndrome (APS)?

A
  1. Lupus anticoagulant
  2. Anticardiolipin antibodies (IgG and IgM)
    They are not specific.

Anticardiolipin antibodies- Pts with APS often test falsely positive for syphilis (positive VDRL or RPR) because the antigen used in syphilis tests is cardiolipin.

203
Q

How would you Mgx Antiphospholipid Syndrome (APS)?

A

Treat with heparin and warfarin as you would for any cause of DVT or
pulmonary embolus. APL syndrome may require lifelong anticoagulation.

M.T.B (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions