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Hematology Flashcards

1
Q

What symptom would you expect if the CBC shows a hematocrit of >30-35%?

A

None

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2
Q

What symptom would you expect if the CBC shows a hematocrit of 25-30%?

A

Dyspnea (worse on exertion), fatigue

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3
Q

What symptom would you expect if the CBC shows a hematocrit of 20-25%?

A

Lightheadedness, angina

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4
Q

What symptom would you expect if the CBC shows a hematocrit of <20-25%?

A

Syncope, chest pain

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5
Q

This is a component of CBC that determines the etiology of anemia.

A

Mean Corpuscular Volume

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6
Q

Give 4 causes of low MCV?

A

Iron deficiency
Thalassemia
Sideroblastic anemia
Anemia of chronic disease

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7
Q

At what level of hematocrit do you transfuse blood to a patient?

A

It depends!

  1. Is the patient SYMPTOMATIC (SOB, lightheaded, confused, syncope, hypotension, tachycardia, chest pain)? Transfuse
  2. IS the hematocrit VERY LOW (25-30) in an ELDERLY or one with HEART DISEASE? Transfuse
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8
Q

A 71-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 of the bone marrow, or liver disease, the first step is a peripheral smear

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9
Q

This type of anemia can be either microcytic or macrocytic

A

Sideroblastic anemia

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10
Q

Blood loss and hemolysis will raise/lower the reticulocyte count

A

raise

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11
Q

Methotrexate causes:

a. macrocytic anemia
b. microcytic anemia
c. normocytic anemia
d. monocytic anemia

A

A

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12
Q

Rheumatoid Arthritis causes:

a. macrocytic anemia
b. microcytic anemia
c. normocytic anemia
d. monocytic anemia

A

D

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13
Q

This blood product is a unit of whole blood with about 150 mL of plasma removed. Each unit raise the hematocrit by about 3 points per unit or 1 g/dL of Hg.

A

Packed red blood cells

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14
Q

This blood product replaces clotting factors in those with an elevated prothrombin time, activated partial thromboplastin time (aPTT), or INR and bleeding. It is used as replacement with plasmapheresis. This is not a choice for hemophilia A or B or vWF disease.

A

Fresh Frozen Plasma

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15
Q

This blood product is used to replace fibrinogen and has some utility in DIC. It provides high amounts of clotting factors in a smaller plasma volume. High levels of Factor VIII and VWF are found in it.

A

Cryoprecipitate

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16
Q

This blood product as all Vitamin K factors used to reverse warfarin toxicity

A

Prothrombin Complex Concentrate (PCC)

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17
Q

This is a type of anemia that can be caused by alcohol, lead poisoning, isoniazin, and vit B6 deficiency. It results from the inability of iron to be incorporated with heme.

A

Sideroblastic anemia

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18
Q

This type of anemia is an extremely common cause of microcytosis. Most patient with this trait alone are asymptomatic.

A

Thalassemia

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19
Q

This type of anemia is commonly found in those with cancer or chronic infection as well as rheumatoid arthritis.

A

Anemia of Chronic Disease

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20
Q

This type of anemia is associated with blood loss and menstruation

A

Iron Deficiency Anemia

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21
Q

Target cells are most common in?

A

Thalassemia

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22
Q

What is the most likely diagnosis if Iron studies reveals low ferritin?

A

Iron Deficiency Anemia

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23
Q

What is the most likely diagnosis if Iron studies reveals high iron?

A

Sideroblastic anemia

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24
Q

What is the most likely diagnosis if Iron studies are normal?

A

Thalassemia

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25
Q

What is the most accurate test for IDA?

A

Bone marrow biopsy

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26
Q

What is the most accurate test for Sideroblastic anemia?

A

Prussian blue staining for ringed sideroblasts

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27
Q

What is the most accurate test for Thalassemia?

A

Hemoglobin electrophoresis

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28
Q

What is the most accurate test for Alpha Thalassemia?

A

Genetic studies/DNA analysis

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29
Q

How would you treat IDA?

A

Replace iron with oral ferrous sulfate. If this is insufficienct, give intramuscular iron

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30
Q

How would you treat anemia of chronic disease?

A

Correct underlying disease

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31
Q

How would you treat sideroblastic anemia?

A

Correct the cause. Some patients respond to vitamin B6 or Pyridoxine replacement.

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32
Q

How would you treat Thalassemia?

A

Trait is not treated.

Beta Thalassemia (Cooley anemia) is managed with chronic transfusion lifelong.

Iron overload is managed with deferasirox or deferiprone (oral iron chelators), Deferoxamine (Parenteral iron chelator)

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33
Q

This type of anemia has the presence of hypersegmented neutrophils.

A

Megaloblastic anemia

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34
Q

These 3 factors are the only one that causes hypersegmentation of neutrophils.

A

B12 deficiency
Folate deficiency
Antimetabolite medications

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35
Q

What nutrient deficiencies will you expect from Celiac disease?

A

B12 deficiency
Folate deficiency
Iron deficiency

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36
Q

What is the most common symptom of B12 deficiency?

A

Peripheral neuropathy

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37
Q

B12 deficiency is associated with an increased/decreased methylmalonic acid level

A

increased

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38
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
d. Homocysteine level

A

A. While both B12 and folate deficiency increase homocysteine levels, only B12 is associated with increased MMA.

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39
Q

How would you confirm Pernicious Anemia?

A

Anti-intrinsic factor and anti-parietal cell antibodies

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40
Q

B12 and Folate deficiency can cause?

A

Pancytopenia and Macrocytic anemia

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41
Q

Metformin is associated with what deficiency?

A

B12 deficiency

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42
Q

How would you treat Macrocytic Anemia?

A

Replace what is deficient. Folate replacement corrects the hematologic problems of B12 deficiency, but not the neurological problems.

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43
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.

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44
Q

This enzymes are needed to remove B12 from R-protein so it can bind with intrinsic factor.

A

Pancreatic enzymes

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45
Q

This is caused by a point mutation at position 6 of the beta globin chain: valine replaces glutamic acid

A

Sickle Cell Disease

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46
Q

What will you expect in the reticulocyte count of a patient with Sickle Cell Disease?

A

Always High

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47
Q

Acute vasoocclusive crisis of Sickle Cell Disease is caused by:

A

Hypoxia
Dehydration
Infection/fever
Cold temperatures

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48
Q

Patient is African American with sudden, severe pain in the chest, back, and thighs that may be accompanied by fever. It is rare for an adult to present with an acute crisis without a clear history of the disease. What is the most likely diagnosis?

A

Sickle Cell Disease

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49
Q

What is the best initial test for Sickle Cell Disease?

A

Peripheral Smear

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50
Q

What is the most accurate test for Sickle Cell Disease?

A

Hemoglobin Electrophoresis

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51
Q

These managements lower mortality in sickle cell disease

A

Hydroxyurea in prevention

Antiobiotics with fever

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52
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.

basophilic stippling - sideroblastic anemia, lead poisoning
bite cells - G6PD
schistocytes - fragmented red cells seen in intravascular hemolysis
morulae - seen inside neutrophils in Ehrlichia infections

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53
Q

What antibiotics can you give if patient with sickle cell disease has fever or a white cell count higher than usual?

A

Ceftriaxone
Levofloxacin
Moxifloxacin

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54
Q

When do you consider exchange transfusion in a patient with sickle cell disease?

A

If there is severe vasoocclusive crisis presenting with:
Acute chest syndrome
Priaprism
Stroke
Visual disturbance from retinal infarction

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55
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. The first clue to parvovirus is a sudden drop in reticulocyte level.

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56
Q

This is the only manifestation of sickle cell trait.

A

Isosthenuria - defect in the ability to concentrate the urine

No treatment

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57
Q

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

A

Hereditary Spherocytosis

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58
Q

Patient presents with recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, family history of anemia or hemolysis, bilirubin gallstones. What is the most likely diagnosis?

A

Hereditary Spherocytosis

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59
Q

What is the most accurate test for Hereditary Spherocytosis?

A

eosin-5-maleimide flow cytometry

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60
Q

What will you expect in the diagnostic tests of Hereditary Spherocytosis?

A
  • Low MCV
  • Increased mean corpuscular hemoglobin concentration (MCHC)
  • Negative Coombs test
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61
Q

What are your treatment options for Hereditary Spherocytosis?

A
  1. Chronic folic acid replacement supports red cell production
  2. Splenectomy stops the hemolysis but does not eliminate the spherocytes
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62
Q

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

A

Coombs test

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63
Q

Clear cause/s of Autoimmune (Warm or IgG) Hemolysis are:

a. Chronic lymphocytic leukemia (CLL)
b. Lymphoma
d. SLE
d. Drugs: penicillin, alpha-methyldopa, rifampicin, phenytoin
e. All of the above

A

E

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64
Q

What is the best initial therapy for Autoimmune Hemolysis?

A

Glucorticoids (Prednisone)

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65
Q

How would you treat sever acute autoimmune hemolysis not responding to Prednisone?

A

IVIg

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66
Q

How would you treat recurrent episodes of Autoimmune Hemolysis?

A

Splenectomy

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67
Q

If splenectomy was not able to control the hemolysis in autoimmune hemolysis, what are your treatment options?

A

Rituximab
Azathioprine
Cyclophosphamide, or
Cyclosporine

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68
Q

These are antibodies against the red cell developing in association with Epstein-Barr virus, Waldenstrom macroglobulinemia, or Mycoplasma pneumoniae.

A

Cold aggltinins/ IgM antibodies

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69
Q

What is the most accurate test for Cold Agglutinin Disease?

A

Cold Agglutinin Titer

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70
Q

How would you treat a patient with 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
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71
Q

This is an X-linked recessive disorder leading to an inability to generate glutathione reductase and protect the red cells from oxidant stress.

A

Glucose 6-phosphate dehydrogenase deficiency

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72
Q

This is the most common oxidant stress in G6PD.

A

Infection

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73
Q

What are other causes of oxidant stress in G6Pd aside from infection?

A 
Dapsone
Quinidine
Sulfa drugs
Primaquine
Nitrofurantoin
Fava beans
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74
Q

Patient is usually an African American or Mediterranean men with sudden anemia and jaundice who have a normal-sized spleen with an infection. What is the most likely diagnosis?

A

G6PD deficiency

75
Q

What will you see in the Methylene Blue staining for G6PD?

A

Heinz bodies

76
Q

These 2 diseases are caused by deficiency of metalloproteinase ADAMTS 13.

A

Hemolytic Uremic Syndrome (HUS)

Thrombotic Thrombocytopenic Purpura (TTP)

77
Q

HUS is associated with

a. E. Coli
b. 0157:H7
c. Ticlopidine
d. Clopidogrel
e. Cyclosporine
f. AIDS
g. SLE
h. a & b
i. c-g

A

H

78
Q

TTP is associated with

a. E. Coli
b. 0157:H7
c. Ticlopidine
d. Clopidogrel
e. Cyclosporine
f. AIDS
g. SLE
h. a & b
i. c-g

A

I

79
Q

What treatment is contraindicated to HUS or TTP and would only worsen it?

A

Platelet Transfusion

80
Q

If TTP or HUS is not related to drugs or diarrhea, how would you treat?

A

Steroids

81
Q

How would you treat severe cases of HUS or TTP?

A

Plasmapheresis or plasma exchange

82
Q

This is a clonal stem cell defect with increased sensitivity of red cells to complement in acidosis. This is from deficiency of the complement regulatory proteins CD 55 and 59 also known as decay accelerating factor.

A

Paroxysmal Nocturnal Hemoglobinuria

83
Q

Patient presents with episodic dark urine, pancytopenia and iron deficiency anemia, and clots in unusual places (not just DVT or pulmonary embolism). What is the most likely diagnosis?

A

Paroxysmal Nocturnal Hemoglobinuria

84
Q

What gene is defective in Paroxysmal Nocturnal Hemoglobinuria?

A

phospatidylinositol class A (PIG-A)

85
Q

If patient has defective phospatidylinositol class A (PIG-A), what will it lead to?

A

overactivation of the complement system (leads to hemolysis and thrombosis in a patient with PNH)

86
Q

What is the most accurate test for Paroxysmal Nocturnal Hemoglobinuria?

A

decreased level of CD55 and CD59 through Flow Cytometry

87
Q

This is the best initial test for hemolysis in Paroxysmal Nocturnal Hemoglobinuria?

A

Prednisone

88
Q

This is the only method of cure for Paroxysmal Nocturnal Hemoglobinuria.

A

Bone Marrow Transplant

89
Q

This is a complement inhibitor that can be used as part of treatment of Paroxysmal Nocturnal Hemoglobinuria for hemolysis and thrombosis. It inactivates C5 in the complement pathway and decreases red cell destruction.

A

Eculizumab

90
Q

What would you give prior to the initiation of Eculizumab in Paroxysmal Nocturnal Hemoglobinuria?

A

Meningococcal vaccine

91
Q

This disease acts as an autoimmune disorder in which the T cells attack the patient’s own marrow. It is confirmed by excluding all the causes of pancytopenia.

A

Aplastic anemia

92
Q

How would you treat aplastic anemia is patient is young?

A

Allogenic Bone Marrow Transplantation

93
Q

What is the treatment when a patient with aplastic anemia is too old for BMT (above 50) or there is no matched donor?

A

Antithymocyte globulin (ATG) and Cyclosporine

94
Q

This is an alternative to Cyclosporine in Aplastic Anemia treatment.

A

Tacrolimus

95
Q

This is an anti-CD52 agent that suppresses the T cells.

A

Alemtuzumab

96
Q

This disease is defined as the unregulated overproduction of all 3 cell lines, with red cell overproduction as the most prominent.

A

Polycythemia Vera

97
Q

What protein is mutated in Polycythemia Vera?

A

JAK2 protein

98
Q

What would you expect in the diagnosis of Polycythemia Vera in terms of hematocrit, platelet and white cell count, total red cell mass, oxygen levels, and erythropoietin levels.

A 
hematocrit - elevated above 60%
platelet and white cell count - elevated
total red cell mass - elevated
oxygen levels - normal
erythropoietin levels - low
99
Q

What is the most accurate test for Polycythemia Vera?

A

JAK2 Mutation

100
Q

What are your treatment options for 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
101
Q

If hyroxyurea fails to lower cell count in Polycythemia Vera, what would you give the patient?

A

Ruxolitinib - inhibitor of JAK

102
Q

This is a disease with markedly elevated platelet count above one million leading to both thrombosis and bleeding.

A

Essential Thrombocytosis

103
Q

What is the best initial therapy for Essential Thrombocytosis?

A

Hydroxyurea

104
Q

What is the cut-off platelet count that’ll tell you to start Hydroxyurea in a patient with Essential Thrombocytosis?

A

above 1.5 million and patient above 60

105
Q

This medication is used when there is red cell suppression from hydroxyurea.

A

Anagrelide

106
Q

This is a disease of older persons with a pancytopenia associated with a bone marrow showing marked fibrosis. Blood production shifts to the spleen and liver, which become markedly enlarged

A

Myelofibrosis

107
Q

What would you look for in the smear to confirm myelofibrosis?

A

Teardrop-shaped cells and nucleated red blood cells

108
Q

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.

A

Acute Leukemia

109
Q

What is the best initial test for Acute Leukemia?

A

Blood smear showing blasts

110
Q

What is the most accurate test for Acute Leukemia?

A

Flow Cytometry

111
Q

This is a characteristic of Acute Myelocytic Leukemia (AML)

A

Myeloperoxidase

112
Q

These are eosinophilic inclusions associated with AML.

A

Auer rods

113
Q

M3 (Promyelocytic leukemia) is prone to…

A

DIC

114
Q

What will you add in the chemotherapy regimen of those with M3 (Promyelocytic leukemia)?

A

All-trans-retinoic acid (ATRA)

115
Q

What will you add in the chemotherapy regimen of those with ALL?

A

Intrathecal Methotrexate

116
Q

This patient has persistently high WBC count that is all neutrophils. Pruritus is common after hot baths/showers, splenomegaly with early satiety, abdominal fullness, and left upper quadrant pain. It can also present with vague symptoms of fatigue, night sweats, and fever from hyper metabolic syndrome. What is the most likely diagnosis?

A

Chronic Myelogenous Leukemia

117
Q

What is the most accurate test for CML?

A

PCR or FISH to determine BCR-ABL (9:22 translocation)

118
Q

What is the best initial treatment for CML?

A

Tyrosine Kinase Inhibitors: Imatinib, Dasatinib, Nilotinib

119
Q

What is the most effective cure for CML?

A

Bone Marrow Transplant

120
Q

A 54-year-old man comes to the emergency department for shortness of breath, blurry vision, confusion, and priaprism. His WBC count is found to be 225,000/uL. 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.

121
Q

This is a preleukemic disorder presenting in older patients (over 60) with a pancytopenia despite a hypercellular bone marrow. Its characteristic abnormality is 5q deletion.

A

Myelodysplastic Syndrome

122
Q

This is the most distinct lab abnormality of Myelodysplastic Syndrome.

A

Pelger-Huet cells

123
Q

Patients with confirmed 5q deletion in MDS respond best to _______.

A

Lenalidomide

124
Q

What will you see in the Prussian blue stain of MDS?

A

Ringed Sideroblasts

125
Q

This is defined as the conversion of CLL into high-grade lymphoma (happens in 5% of patients).

A

Richter phenomenon

126
Q

This is a clonal proliferation of normal, mature-appearing B lymphocytes that function abnormally. It occurs over the age of 50 in 90% of those affected. Many are asymptomatic at presentation with only a markedly elevated white cell count.

A

Chronic Lymphocytic Leukemia

127
Q

What is the treatment for Stage 3 and 4 of CLL?

A

Fludarabine, Cyclophosphamide, Rituximab

128
Q

This disease presents in middle-aged men. B-cells with filamentous projections are seen on smear.

A

Hairy Cell Leukemia

129
Q

What is the best initial test for Hairy Cell Leukemia?

A

Smear

130
Q

What is the most accurate test for Hairy Cell Leukemia?

A

Immunotyping by Flow Cytometry

131
Q

How would you treat Hairy Cell Leukemia?

A

Cladribine or Pentostatin

132
Q

This is defined as proliferation of lymphocytes in the lymph nodes and spleen. It can affect any lymph node or organ that has lymphoid tissue. It is very similar to CLL. There is no Reed-Sternberg cells

A

Non-Hodgkin Lymphoma

133
Q

Patient has painless lymphadenopathy. Nodes are not warm, red, or tender. He also presents with ‘B’ symptoms such as fever, weight loss, and night sweats. What is the most likely diagnosis?

A

Non-Hodgkin/Hodgkin Lymphoma

134
Q

What is the best initial test for Non-Hodgkin/Hodgkin Lymphoma?

A

Excisional Biopsy

135
Q

High _____ levels correlate with worse severity of Non-Hodgkin/Hodgkin Lymphoma.

A

LDL

136
Q

How would you stage Non-Hodgkin/Hodgkin Lymphoma?

A
  • CT scan of the chest, abdomen, and pelvis

- Bone marrow biopsy

137
Q

How would you treat local disease (stage Ia and IIa) Non-Hodgkin/Hodgkin Lymphoma?

A

local radiation and small dose/course of chemotherapy

138
Q

How would you treat advanced disease (stage III and IV, any ‘B’ symptoms) of Non-Hodgkin?

A

Combination Chemotherapy with CHOP and Rituximab (an antibody against CD20)

C = cyclophosphamide
H = adriamycin (doxorubicin or 'hydroxydaunorubicin')
O = vincristine (oncovin)
P = prednisone
139
Q

This is a pathologic subtype of Hodgkin disease that has the best prognosis.

A

Lymphocyte predominant

140
Q

This is a pathologic subtype of Hodgkin disease that has the worst prognosis.

A

Lymphocyte depleted

141
Q

Non-Hodgkin/Hodgkin Lymphoma?

Disease centers around cervical area

A

Hodgkin Lymphoma

142
Q

Non-Hodgkin/Hodgkin Lymphoma?

Disease is disseminated

A

Non-Hodgkin

143
Q

Non-Hodgkin/Hodgkin Lymphoma?

Reed-Sternberg cells

A

Hodgkin Lymphoma?

144
Q

Non-Hodgkin/Hodgkin Lymphoma?

No Reed-Sternberg cells

A

Non-Hodgkin Lymphoma

145
Q

How would you treat local disease (stage Ia and IIa) of Non-Hodgkin Lymphoma?

A

Local radiation and small dose/course of chemotherapy

146
Q

How would you treat local disease (stage Ia and IIa) of Hodgkin Lymphoma?

A

Local radiation and small dose/course of chemotherapy

147
Q

How would you treat advanced disease (stage III and IV, any ‘B’ symptoms) of Hodgkin Lymphoma?

A

ABVD

A = adriamycin (doxorubicin)
B = bleomycin
V = vinblastine
D = dacarbazine
148
Q

What are the risks associated with radiation therapy?

A
  • solid tumors such as breast, thyroid, or lung cancer (screen for breast cancer after 8 years)
  • increases the chance of premature coronary artery disease
149
Q

Which of the following is the most useful to determine dosing of chemotherapy in Hodgkin Disease?

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’ use adriamycin if the ejection fraction is less than 50%.

150
Q

What is the adverse effect of Doxorubicin?

A

Cardiomyopathy

151
Q

What is the adverse effect of Vincristine?

A

Neuropathy

152
Q

What is the adverse effect of Bleomycin?

A

Lung Fibrosis

153
Q

What is the adverse effect of Cyclophosphamide?

A

Hemorrhagic cystitis

154
Q

What is the adverse effect of Cisplatin?

A

Renal and ototoxicity

155
Q

This is an abnormal proliferation of plasma cells. These plasma cells are unregulated in their production of useless immunoglobulin that is usually IgG or IgA.

A

Multiple Myeloma

156
Q

This is the most common presentation of myeloma

A

Bone pain

157
Q

What is the most common cause of death of Multiple Myeloma?

A

Renal Failure & Infection

158
Q

This is the first test done in Multiple Myeloma.

A

X-Ray will show lytic (punched out) lesions

159
Q

What will you expect in the bone marrow biopsy of Multiple Myeloma?

A

Greater than 10% plasma cells

160
Q

What electrolyte abnormality will you see in Multiple Myeloma?

A

Hypercalcemia

161
Q

What will you see on urine immunoelectrophoresis in Multiple Myeloma?

A

Bence-Jones protein

162
Q

What will you see in the smear in Multiple Myeloma?

A

Rouleaux formation

163
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-Jonces 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.

164
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 “M-spike” of immunoglobulin, 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.

165
Q

What is the best initial therapy for Multiple Myeloma?

A

Combination of Dexamethasone with Lenalidomide, Bortezomib, or both

166
Q

This is an alternative treatment for older patients with Multiple Myeloma who cannot tolerate adverse effects.

A

Melphalan

167
Q

What is the most effective therapy for Multiple Myeloma in those under age 70?

A

Autologous Bone Marrow Transplant with Stem Cell support

168
Q

This is the overproduction of IgM from malignant B cells leading to hyperviscosity. It presents with lethargy, blurry vision and vertigo, engorged blood vessels in the eye, mucosal bleeding, and raynaud phenomenon.

A

Waldenstrom Macroglobulinemia

169
Q

What is the best initial therapy for Waldenstrom Macroglobulinemia?

A

Plasmapheresis

170
Q

What are long term treatments you can consider for Waldenstrom Macroglobulinemia?

A

Rituximab or Prednisone Cyclophosphamide

171
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/uL.

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.

172
Q

Patient has an isolated case of thrombocytopenia (normal hematocrit, normal WBC count), and normal-sized spleen. What is the most likely diagnosis?

A

Immune Thrombocytopenic Purpura (ITP)

173
Q

What is the treatment for ITP with mild bleeding (platelet count <30,000)?

A

Glucocorticoids

174
Q

What is the treatment for ITP with severe bleeding (platelet count <10,000)?

A

IVIG, Anti-Rho (anti-D)

175
Q

What is the treatment for recurrent episodes of ITP that is steroid dependent?

A

Splenectomy

176
Q

What is the treatment for ITP if splenectomy or steroids is not effective?

A

Romiplostim or eltrombopag, rituximab, azathioprine, cyclosporine, mycophenolate

177
Q

Prior to splenectomy, what vaccines should you give to the patient?

A

Neisseria meningitidis
Haemophilus influenzae
Pneumococcus

178
Q

It is the most common inherited bleeding disorder.

A

Von Willebrand Disease

179
Q

This patient usually have bleeding related to platelets (epistaxis, gingival, gums) with a normal platelet count. It is markedly worsened after the use of aspirin. aPTT may be elevated.

A

Von Willebrand Disease

180
Q

This is the best initial therapy for VWD.

A

DDAVP (Desmopressin)

181
Q

What will you give if there is no response to Desmopressin in VWD?

A

Factor VIII replacement or VWF concentrate

182
Q

This usually present as delayed joint or muscle bleeding in a male child, since this condition is X-linked recessive.

A

Hemophilia

183
Q

What is the most accurate test for Hemophilia?

A

Specific assay for Factor VIII or IX

Medicine (15 decks)

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