Hematology Flashcards
1
Q
What symptom would you expect if the CBC shows a hematocrit of >30-35%?
A
None
2
Q
What symptom would you expect if the CBC shows a hematocrit of 25-30%?
A
Dyspnea (worse on exertion), fatigue
3
Q
What symptom would you expect if the CBC shows a hematocrit of 20-25%?
A
Lightheadedness, angina
4
Q
What symptom would you expect if the CBC shows a hematocrit of <20-25%?
A
Syncope, chest pain
5
Q
This is a component of CBC that determines the etiology of anemia.
A
Mean Corpuscular Volume
6
Q
Give 4 causes of low MCV?
A
Iron deficiency
Thalassemia
Sideroblastic anemia
Anemia of chronic disease
7
Q
At what level of hematocrit do you transfuse blood to a patient?
A
It depends!
- Is the patient SYMPTOMATIC (SOB, lightheaded, confused, syncope, hypotension, tachycardia, chest pain)? Transfuse
- IS the hematocrit VERY LOW (25-30) in an ELDERLY or one with HEART DISEASE? Transfuse
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
9
Q
This type of anemia can be either microcytic or macrocytic
A
Sideroblastic anemia
10
Q
Blood loss and hemolysis will raise/lower the reticulocyte count
A
raise
11
Q
Methotrexate causes:
a. macrocytic anemia
b. microcytic anemia
c. normocytic anemia
d. monocytic anemia
A
A
12
Q
Rheumatoid Arthritis causes:
a. macrocytic anemia
b. microcytic anemia
c. normocytic anemia
d. monocytic anemia
A
D
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
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
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
16
Q
This blood product as all Vitamin K factors used to reverse warfarin toxicity
A
Prothrombin Complex Concentrate (PCC)
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
18
Q
This type of anemia is an extremely common cause of microcytosis. Most patient with this trait alone are asymptomatic.
A
Thalassemia
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
20
Q
This type of anemia is associated with blood loss and menstruation
A
Iron Deficiency Anemia
21
Q
Target cells are most common in?
A
Thalassemia
22
Q
What is the most likely diagnosis if Iron studies reveals low ferritin?
A
Iron Deficiency Anemia
23
Q
What is the most likely diagnosis if Iron studies reveals high iron?
A
Sideroblastic anemia
24
Q
What is the most likely diagnosis if Iron studies are normal?
A
Thalassemia
25
What is the most accurate test for IDA?
Bone marrow biopsy
26
What is the most accurate test for Sideroblastic anemia?
Prussian blue staining for ringed sideroblasts
27
What is the most accurate test for Thalassemia?
Hemoglobin electrophoresis
28
What is the most accurate test for Alpha Thalassemia?
Genetic studies/DNA analysis
29
How would you treat IDA?
Replace iron with oral ferrous sulfate. If this is insufficienct, give intramuscular iron
30
How would you treat anemia of chronic disease?
Correct underlying disease
31
How would you treat sideroblastic anemia?
Correct the cause. Some patients respond to vitamin B6 or Pyridoxine replacement.
32
How would you treat Thalassemia?
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)
33
This type of anemia has the presence of hypersegmented neutrophils.
Megaloblastic anemia
34
These 3 factors are the only one that causes hypersegmentation of neutrophils.
B12 deficiency
Folate deficiency
Antimetabolite medications
35
What nutrient deficiencies will you expect from Celiac disease?
B12 deficiency
Folate deficiency
Iron deficiency
36
What is the most common symptom of B12 deficiency?
Peripheral neuropathy
37
B12 deficiency is associated with an increased/decreased methylmalonic acid level
increased
38
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. While both B12 and folate deficiency increase homocysteine levels, only B12 is associated with increased MMA.
39
How would you confirm Pernicious Anemia?
Anti-intrinsic factor and anti-parietal cell antibodies
40
B12 and Folate deficiency can cause?
Pancytopenia and Macrocytic anemia
41
Metformin is associated with what deficiency?
B12 deficiency
42
How would you treat Macrocytic Anemia?
Replace what is deficient. Folate replacement corrects the hematologic problems of B12 deficiency, but not the neurological problems.
43
Which of the following is a complication of B12 or folate replacement?
a. Seizures
b. Hemolysis
c. Hypokalemia
d. Hyperkalemia
e. Diarrhea
C. Extremely rapid cell production in the bone marrow causes hypokalemia.
44
This enzymes are needed to remove B12 from R-protein so it can bind with intrinsic factor.
Pancreatic enzymes
45
This is caused by a point mutation at position 6 of the beta globin chain: valine replaces glutamic acid
Sickle Cell Disease
46
What will you expect in the reticulocyte count of a patient with Sickle Cell Disease?
Always High
47
Acute vasoocclusive crisis of Sickle Cell Disease is caused by:
Hypoxia
Dehydration
Infection/fever
Cold temperatures
48
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?
Sickle Cell Disease
49
What is the best initial test for Sickle Cell Disease?
Peripheral Smear
50
What is the most accurate test for Sickle Cell Disease?
Hemoglobin Electrophoresis
51
These managements lower mortality in sickle cell disease
Hydroxyurea in prevention
| Antiobiotics with fever
52
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
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
53
What antibiotics can you give if patient with sickle cell disease has fever or a white cell count higher than usual?
Ceftriaxone
Levofloxacin
Moxifloxacin
54
When do you consider exchange transfusion in a patient with sickle cell disease?
If there is severe vasoocclusive crisis presenting with:
Acute chest syndrome
Priaprism
Stroke
Visual disturbance from retinal infarction
55
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. 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.
56
This is the only manifestation of sickle cell trait.
Isosthenuria - defect in the ability to concentrate the urine
No treatment
57
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.
Hereditary Spherocytosis
58
Patient presents with recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, family history of anemia or hemolysis, bilirubin gallstones. What is the most likely diagnosis?
Hereditary Spherocytosis
59
What is the most accurate test for Hereditary Spherocytosis?
eosin-5-maleimide flow cytometry
60
What will you expect in the diagnostic tests of Hereditary Spherocytosis?
- Low MCV
- Increased mean corpuscular hemoglobin concentration (MCHC)
- Negative Coombs test
61
What are your treatment options for Hereditary Spherocytosis?
1. Chronic folic acid replacement supports red cell production
2. Splenectomy stops the hemolysis but does not eliminate the spherocytes
62
What is the most accurate diagnostic test for Autoimmune (Warm or IgG) Hemolysis?
Coombs test
63
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
E
64
What is the best initial therapy for Autoimmune Hemolysis?
Glucorticoids (Prednisone)
65
How would you treat sever acute autoimmune hemolysis not responding to Prednisone?
IVIg
66
How would you treat recurrent episodes of Autoimmune Hemolysis?
Splenectomy
67
If splenectomy was not able to control the hemolysis in autoimmune hemolysis, what are your treatment options?
Rituximab
Azathioprine
Cyclophosphamide, or
Cyclosporine
68
These are antibodies against the red cell developing in association with Epstein-Barr virus, Waldenstrom macroglobulinemia, or Mycoplasma pneumoniae.
Cold aggltinins/ IgM antibodies
69
What is the most accurate test for Cold Agglutinin Disease?
Cold Agglutinin Titer
70
How would you treat a patient with Cold Agglutinin Disease?
1. Keep the patient warm
2. Administer rituximab and sometimes plasmapheresis
3. Cyclophosphamide, cyclosporine, or other immunosuppressive agents stop the production of the antibody
71
This is an X-linked recessive disorder leading to an inability to generate glutathione reductase and protect the red cells from oxidant stress.
Glucose 6-phosphate dehydrogenase deficiency
72
This is the most common oxidant stress in G6PD.
Infection
73
What are other causes of oxidant stress in G6Pd aside from infection?
```
Dapsone
Quinidine
Sulfa drugs
Primaquine
Nitrofurantoin
Fava beans
```
74
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?
G6PD deficiency
75
What will you see in the Methylene Blue staining for G6PD?
Heinz bodies
76
These 2 diseases are caused by deficiency of metalloproteinase ADAMTS 13.
Hemolytic Uremic Syndrome (HUS)
| Thrombotic Thrombocytopenic Purpura (TTP)
77
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
H
78
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
I
79
What treatment is contraindicated to HUS or TTP and would only worsen it?
Platelet Transfusion
80
If TTP or HUS is not related to drugs or diarrhea, how would you treat?
Steroids
81
How would you treat severe cases of HUS or TTP?
Plasmapheresis or plasma exchange
82
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.
Paroxysmal Nocturnal Hemoglobinuria
83
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?
Paroxysmal Nocturnal Hemoglobinuria
84
What gene is defective in Paroxysmal Nocturnal Hemoglobinuria?
phospatidylinositol class A (PIG-A)
85
If patient has defective phospatidylinositol class A (PIG-A), what will it lead to?
overactivation of the complement system (leads to hemolysis and thrombosis in a patient with PNH)
86
What is the most accurate test for Paroxysmal Nocturnal Hemoglobinuria?
decreased level of CD55 and CD59 through Flow Cytometry
87
This is the best initial test for hemolysis in Paroxysmal Nocturnal Hemoglobinuria?
Prednisone
88
This is the only method of cure for Paroxysmal Nocturnal Hemoglobinuria.
Bone Marrow Transplant
89
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.
Eculizumab
90
What would you give prior to the initiation of Eculizumab in Paroxysmal Nocturnal Hemoglobinuria?
Meningococcal vaccine
91
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.
Aplastic anemia
92
How would you treat aplastic anemia is patient is young?
Allogenic Bone Marrow Transplantation
93
What is the treatment when a patient with aplastic anemia is too old for BMT (above 50) or there is no matched donor?
Antithymocyte globulin (ATG) and Cyclosporine
94
This is an alternative to Cyclosporine in Aplastic Anemia treatment.
Tacrolimus
95
This is an anti-CD52 agent that suppresses the T cells.
Alemtuzumab
96
This disease is defined as the unregulated overproduction of all 3 cell lines, with red cell overproduction as the most prominent.
Polycythemia Vera
97
What protein is mutated in Polycythemia Vera?
JAK2 protein
98
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.
```
hematocrit - elevated above 60%
platelet and white cell count - elevated
total red cell mass - elevated
oxygen levels - normal
erythropoietin levels - low
```
99
What is the most accurate test for Polycythemia Vera?
JAK2 Mutation
100
What are your treatment options for Polycythemia Vera?
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
If hyroxyurea fails to lower cell count in Polycythemia Vera, what would you give the patient?
Ruxolitinib - inhibitor of JAK
102
This is a disease with markedly elevated platelet count above one million leading to both thrombosis and bleeding.
Essential Thrombocytosis
103
What is the best initial therapy for Essential Thrombocytosis?
Hydroxyurea
104
What is the cut-off platelet count that'll tell you to start Hydroxyurea in a patient with Essential Thrombocytosis?
above 1.5 million and patient above 60
105
This medication is used when there is red cell suppression from hydroxyurea.
Anagrelide
106
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
Myelofibrosis
107
What would you look for in the smear to confirm myelofibrosis?
Teardrop-shaped cells and nucleated red blood cells
108
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.
Acute Leukemia
109
What is the best initial test for Acute Leukemia?
Blood smear showing blasts
110
What is the most accurate test for Acute Leukemia?
Flow Cytometry
111
This is a characteristic of Acute Myelocytic Leukemia (AML)
Myeloperoxidase
112
These are eosinophilic inclusions associated with AML.
Auer rods
113
M3 (Promyelocytic leukemia) is prone to...
DIC
114
What will you add in the chemotherapy regimen of those with M3 (Promyelocytic leukemia)?
All-trans-retinoic acid (ATRA)
115
What will you add in the chemotherapy regimen of those with ALL?
Intrathecal Methotrexate
116
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?
Chronic Myelogenous Leukemia
117
What is the most accurate test for CML?
PCR or FISH to determine BCR-ABL (9:22 translocation)
118
What is the best initial treatment for CML?
Tyrosine Kinase Inhibitors: Imatinib, Dasatinib, Nilotinib
119
What is the most effective cure for CML?
Bone Marrow Transplant
120
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. In acute leukostasis reaction, it is more important to remove the excessive white cells from the blood than to establish a specific diagnosis.
121
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.
Myelodysplastic Syndrome
122
This is the most distinct lab abnormality of Myelodysplastic Syndrome.
Pelger-Huet cells
123
Patients with confirmed 5q deletion in MDS respond best to _______.
Lenalidomide
124
What will you see in the Prussian blue stain of MDS?
Ringed Sideroblasts
125
This is defined as the conversion of CLL into high-grade lymphoma (happens in 5% of patients).
Richter phenomenon
126
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.
Chronic Lymphocytic Leukemia
127
What is the treatment for Stage 3 and 4 of CLL?
Fludarabine, Cyclophosphamide, Rituximab
128
This disease presents in middle-aged men. B-cells with filamentous projections are seen on smear.
Hairy Cell Leukemia
129
What is the best initial test for Hairy Cell Leukemia?
Smear
130
What is the most accurate test for Hairy Cell Leukemia?
Immunotyping by Flow Cytometry
131
How would you treat Hairy Cell Leukemia?
Cladribine or Pentostatin
132
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
Non-Hodgkin Lymphoma
133
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?
Non-Hodgkin/Hodgkin Lymphoma
134
What is the best initial test for Non-Hodgkin/Hodgkin Lymphoma?
Excisional Biopsy
135
High _____ levels correlate with worse severity of Non-Hodgkin/Hodgkin Lymphoma.
LDL
136
How would you stage Non-Hodgkin/Hodgkin Lymphoma?
- CT scan of the chest, abdomen, and pelvis
| - Bone marrow biopsy
137
How would you treat local disease (stage Ia and IIa) Non-Hodgkin/Hodgkin Lymphoma?
local radiation and small dose/course of chemotherapy
138
How would you treat advanced disease (stage III and IV, any 'B' symptoms) of Non-Hodgkin?
Combination Chemotherapy with CHOP and Rituximab (an antibody against CD20)
```
C = cyclophosphamide
H = adriamycin (doxorubicin or 'hydroxydaunorubicin')
O = vincristine (oncovin)
P = prednisone
```
139
This is a pathologic subtype of Hodgkin disease that has the best prognosis.
Lymphocyte predominant
140
This is a pathologic subtype of Hodgkin disease that has the worst prognosis.
Lymphocyte depleted
141
Non-Hodgkin/Hodgkin Lymphoma?
Disease centers around cervical area
Hodgkin Lymphoma
142
Non-Hodgkin/Hodgkin Lymphoma?
Disease is disseminated
Non-Hodgkin
143
Non-Hodgkin/Hodgkin Lymphoma?
Reed-Sternberg cells
Hodgkin Lymphoma?
144
Non-Hodgkin/Hodgkin Lymphoma?
No Reed-Sternberg cells
Non-Hodgkin Lymphoma
145
How would you treat local disease (stage Ia and IIa) of Non-Hodgkin Lymphoma?
Local radiation and small dose/course of chemotherapy
146
How would you treat local disease (stage Ia and IIa) of Hodgkin Lymphoma?
Local radiation and small dose/course of chemotherapy
147
How would you treat advanced disease (stage III and IV, any 'B' symptoms) of Hodgkin Lymphoma?
ABVD
```
A = adriamycin (doxorubicin)
B = bleomycin
V = vinblastine
D = dacarbazine
```
148
What are the risks associated with radiation therapy?
- 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
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
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
What is the adverse effect of Doxorubicin?
Cardiomyopathy
151
What is the adverse effect of Vincristine?
Neuropathy
152
What is the adverse effect of Bleomycin?
Lung Fibrosis
153
What is the adverse effect of Cyclophosphamide?
Hemorrhagic cystitis
154
What is the adverse effect of Cisplatin?
Renal and ototoxicity
155
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.
Multiple Myeloma
156
This is the most common presentation of myeloma
Bone pain
157
What is the most common cause of death of Multiple Myeloma?
Renal Failure & Infection
158
This is the first test done in Multiple Myeloma.
X-Ray will show lytic (punched out) lesions
159
What will you expect in the bone marrow biopsy of Multiple Myeloma?
Greater than 10% plasma cells
160
What electrolyte abnormality will you see in Multiple Myeloma?
Hypercalcemia
161
What will you see on urine immunoelectrophoresis in Multiple Myeloma?
Bence-Jones protein
162
What will you see in the smear in Multiple Myeloma?
Rouleaux formation
163
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
D. Bence-Jones protein is detected by urine immunoelectrophoresis. The urine dipstick will detect only albumin.
164
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)
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
What is the best initial therapy for Multiple Myeloma?
Combination of Dexamethasone with Lenalidomide, Bortezomib, or both
166
This is an alternative treatment for older patients with Multiple Myeloma who cannot tolerate adverse effects.
Melphalan
167
What is the most effective therapy for Multiple Myeloma in those under age 70?
Autologous Bone Marrow Transplant with Stem Cell support
168
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.
Waldenstrom Macroglobulinemia
169
What is the best initial therapy for Waldenstrom Macroglobulinemia?
Plasmapheresis
170
What are long term treatments you can consider for Waldenstrom Macroglobulinemia?
Rituximab or Prednisone Cyclophosphamide
171
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
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
Patient has an isolated case of thrombocytopenia (normal hematocrit, normal WBC count), and normal-sized spleen. What is the most likely diagnosis?
Immune Thrombocytopenic Purpura (ITP)
173
What is the treatment for ITP with mild bleeding (platelet count <30,000)?
Glucocorticoids
174
What is the treatment for ITP with severe bleeding (platelet count <10,000)?
IVIG, Anti-Rho (anti-D)
175
What is the treatment for recurrent episodes of ITP that is steroid dependent?
Splenectomy
176
What is the treatment for ITP if splenectomy or steroids is not effective?
Romiplostim or eltrombopag, rituximab, azathioprine, cyclosporine, mycophenolate
177
Prior to splenectomy, what vaccines should you give to the patient?
Neisseria meningitidis
Haemophilus influenzae
Pneumococcus
178
It is the most common inherited bleeding disorder.
Von Willebrand Disease
179
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.
Von Willebrand Disease
180
This is the best initial therapy for VWD.
DDAVP (Desmopressin)
181
What will you give if there is no response to Desmopressin in VWD?
Factor VIII replacement or VWF concentrate
182
This usually present as delayed joint or muscle bleeding in a male child, since this condition is X-linked recessive.
Hemophilia
183
What is the most accurate test for Hemophilia?
Specific assay for Factor VIII or IX
