HemeOnc Flashcards
1
Q
HCT criteria for diagnosing anemia in a male
A
< 41%
2
Q
Hgb criteria for diagnosing anemia in a male
A
< 13.5 g/dL
3
Q
HCT criteria for diagnosing anemia in a female
A
< 37%
4
Q
Hgb criteria for diagnosing anemia in a female
A
< 12 g/dL
5
Q
Patient with liver disease presents for his bimonthly LFTs. In addition to the LFT report, you see that the lab tech has noted inc. ferritin, dec. iron, and dec. RBC MCV. diagnosis?
A
Anemia of chronic disease
6
Q
While treating a chronic renal failure patient for her anemia with Erythropoietin, what should you be cautious of?
A
Watch her iron stores, since she’s likely on dialysis. We don’t want to overload the kidneys with iron.
7
Q
Condition of bone marrow failure that arises from injury to or suppression of the hematopoietic stem cell
A
Aplastic anemia
8
Q
Anemia involving pancytopenia, no abnormal cells on PBS, and hypocellular bone marrow
A
Aplastic anemia
9
Q
Most common cause of aplastic anemia
A
T-cell mediated autoimmune bone marrow failure - shuts down blood cell production
10
Q
Patient presents with weakness, fatigue, increased bleeding time, pallor, purpura, petechia, and seems to have had a series of infections in the last 6 months. Diagnosis?
A
Aplastic anemia
11
Q
Refractory aplastic anemia tx
A
IV Cyclophosphamide
12
Q
Treatment for severe aplastic anemia
A
-If 40, anti-thymocyte globulin + Cyclosporine or Tacrolimus
13
Q
How does antithymocyte globuline (ATG) work?
A
Works at the thymus to reduce T-cell production
14
Q
Why do we give steroid with anti-thymocyte globulin (ATG) treatment?
A
- to avoid serum sickness
- the globulin we’re giving is a foreign protein and we don’t want someone to have a massive reaction to it
- steroids reduce this chance
15
Q
Why is Vit. B12 important?
A
It’s a cofactor for steps in DNA synthesis, particularly in erythroid progenitor cells
16
Q
Macrocytic (Megaloblastic) anemia with macro-ovalocytes and hypersegmented neutrophils
A
- B12 or folate deficiency!
- Have to check levels of both
17
Q
Why can a decrease in intrinsic factor lead to a B12 deficiency?
A
Intrinsic factor is needed to absorb B12
18
Q
Vit. B12 deficiency might be associated with what other kind of anemia?
A
Pernicious anemia
19
Q
Severe B12 deficiency anemia can mimic what other type of anemia, and how can you tell them apart?
A
- Aplastic anemia
- MCV is large in B12 deficiency and normal in aplastic anemia
20
Q
Patient presents with red and swollen tongue, diarrhea, and anorexia. On exam, you note a loss of proprioception, balance, and vibratory sensation. Diagnosis?
A
-Vit. B12 deficiency anemia
21
Q
Peripheral blood smear with the following findings:
- elevated MCV
- Anisocytes and Poikilocytes
- Macro-ovalocytes
- hypersegmented neutrophils
A
Vitamin B12 or Folate deficiency anemia
22
Q
Patient presents with a red and swollen tongue, anorexia, and diarrhea. You suspect an anemia. What kind?
A
Folate deficiency
23
Q
Iron deficiency anemia is caused by _______ until proven otherwise
A
Bleeding
24
Q
Most common cause of anemia worldwide
A
Iron deficiency anemia
25
Where is 70-95% of our iron contained?
In Hgb
26
How much of our dietary iron is usually absorbed, and what can we increase it to?
- 10%
| - Can be increased to 20% if our body needs it
27
Patient presents with easy fatigability, heart palpitations, and tachypnea. She says she's had a weird craving to eat things that aren't food. You note tachycardia. Suspected diagnosis?
Iron deficiency anemia
28
Peripheral blood smear with:
- hypochromic RBCs
- target cells
- Elliptocytes
Iron deficiency anemia
29
What would you add with PO ferrous sulfate to decrease GI effects and increase iron absorption?
Vitamin C
30
Hereditary enzyme defect that causes episodic hemolytic anemia because of the decreased ability of RBCs to deal with oxidative stress
G6PD deficiency anemia
31
- X-linked recessive disorder in American black men or Mediterranean patients
- Episodic hemolysis
- Minimally abnormal peripheral blood smear
G6PD deficiency anemia
32
What types of cells might you see during an "episode" in a G6PD deficiency anemia patient?
- Increased retics
- blister/bite cells
- Heinz bodies
33
Why do we see Blister and Bite cells in G6PD deficiency anemia?
-Because the Hgb is crystallizing
34
Patient presents RUQ pain and the feeling of being slightly out of breath. While performing an ultrasound for suspected gallstones, you note pallor and jaundice. Underlying cause of symptoms?
Hemolytic anemia
35
PBS shows elevated retics and nRBCs. Increased plasma Hgb and indirect bilirubin also noted. Diagnosis?
Hemolytic anemia
36
Genetic mutation that causes Sickle Cell Anemia?
-A point mutation, changing a Valine for a Glutamine on the 6th spot of the beta hemoglobin chain
37
What can the hemolytic anemia that's associated with sickle cell anemia cause?
- Jaundice
- Gallstones
- Hepatosplenomegaly
- Poorly healing ulcers over the lower tibia
38
Patient presents with ulcerations over the lower tibia that won't seem to heal. On exam, you note jaundice and hepatosplenomegaly. You draw blood. What do you expect to find on the PBS?
- Sickle cells
- Reticulocytes
- Howell-Jolly bodies
- Target cells
39
Hgb F characteristics
- Very high affinity for oxygen
| - Can cause hypoxia in tissues
40
Hereditary disorder characterized by reduction in the synthesis of globin chains
Thalassemias
41
-Microcytosis disproportionate to the degree of anemia with elevated RBC count
Thalassemia
42
Classification of a thalassemia that has no clinical impact
Trait
43
Classification of a thalassemia that sometimes requires RBC transfusions, but otherwise has only a moderate clinical impact
Intermedia
44
Classification of a thalassemia that involves transfusion-dependence
Major
45
Type of genetic mutation that results in alpha-thalassemias
Gene deletions
46
Thalassemia that produces no change in the percentage distibution of Hgb A, A2, or F
Alpha
47
Type of genetic mutation that causes beta-thalassemias
Point mutations, which result in premature chain termination or in problems with RNA transcription
48
Beta null thalassemia
Absent globin chain production
49
Beta plus thalassemia
Reduced globin chain synthesis
50
What do the RBCs look like in a patient with a thalassemia?
Small (microcytic) and hypochromic
51
Which chromosome contains the genes with information for alpha globin chains?
Chromosome 16
52
Which chromosome contains the genes with information for beta globin chains?
Chromosome 11
53
Consequence of an alpha-thalassemia in which three genes are deleted
Hgb H
54
Consequence of an alpha-thalassemia in which 4 genes are deleted?
Hydrops fetalis (fluid overload causes fetal heart failure)
55
Consequence of an alpha-thalassemia in which 2 genes are deleted
"Trait"
56
Ethnicity of many patients with beta-thalassemias
Mediterranean
57
Anemia involving:
- MCV 60-75
- HCT: 28-40%
- PBS: microcytes, hypochromia, target cells, acanthocytes
- Electrophoresis: no increase in Hgb A2, F, and Hgb H is absent
Alpha-thalassemia trait (2 gene deletion)
58
Anemia involving:
- MCV: 60-70
- HCT 22-32%
- PBS: microcytes, hypochromia, target cells, poikilocytosis
- Electrophoresis: Hgb H
Alpha-thalassemia Hgb H disease (3 genes deleted)
59
Anemia involving:
- MCV: 55-75
- HCT: 28-40%
- PBS: hypochromia, microcytes, target cells, basophilic stippling
- Electrophoresis: Hgb A2 and Hgb F
Beta-thalassemia minor
60
Anemia involving:
- HCT: < 10%
- PBS: poikilocytosis, hypochromia, microcytes, target cells, basophilic stippling, nRBCs
- Electrophoresis: Hgb F is major Hgb present
Beta-thalassemia Major
61
Hgb H disease tx
- Folate supplementation
| - Avoid iron and oxidative drugs
62
Severe thalassemia tx
- Transfusions
- Folate supplementation
- Bone marrow transplant for beta major
63
Acquired myeloproliferative bone marrow disorder that causes overproduction of all three cell lines
Polycythemia vera
64
What causes polycythemia vera?
Mutation of the JAK2 gene
65
Patient presents after an episode of extreme itching after getting out of the shower. He says it's happened many times and that he's lately developed tinnitus, blurred vision, a HA, and fatigue. Suspicion?
Polycythemia vera
66
What might you find on physical exam of someone with polycythemia vera?
- Plethora (excess blood)
- Engorged retinal veins
- Palpable spleen
67
Major complication of polycythemia vera
- Thrombosis
| - Due to increased blood viscosity and abnormal platelet function
68
Is bleeding time of polycythemia vera increased or decreased?
Increased (paradoxical)
69
Hallmark lab finding of HCT > 54% in males and 51% in females
Polycythemia vera
70
What's associated with:
- Inc. RBC mass
- Inc. WBC and RBC count
- Basophilia and Eosinophilia
- Low erythropoietin
- Decreased iron stores
Polycythemia vera
71
Treatment of choice for polycythemia vera
Intentional phlebotomy
72
Malignancy of the hematopoietic progenitor cell; cells proliferate in an uncontrolled fashion and replace normal bone marrow elements
Acute Leukemia
73
- Short duration of fatigue, fever, bleeding
- Cytopenia or pancytopenia
- > 20% blasts in bone marrow (increased)
- Blast cells in peripheral blood
Acute Leukemia
74
Leukemia that occurs primarily in adults > 60yo and is associated with auer rod cells
AML
75
Leukemia that occurs primarily in children
ALL
76
13-yo WF presents with HA, dyspnea, and repetitive nosebleeds. PMH significant for menorrhagia and diffuse bone pain. Suspicion?
ALL
77
65yo WM presents with mucosal bleeding and diffuse pain in his bones and joints. He thinks it's arthritis, but you note no inflammation in joints and hypertrophy of his gums. What's your suspicion?
AML
78
6yo patient presents with small "bruises" on her body and bone pain. On physical exam, you note hepatosplenomegaly and enlarged lymph nodes. You draw blood and find a decrease in all three cell lines. Suspicion?
ALL
79
What medical treatment do you provide for adult patients after ALL remission has been achieved via combination chemo?
CNS prophylaxis, because some leukemic cells can sequester in the brain
80
Why do acute leukemia patients experience HA, confusion, and dyspnea?
They have an increase in circulating blasts, which can impair circulation
81
AML treatment
Anthracyclines + Cytarabine
82
Chromosomal abnormality associated with chronic myeloid leukemia?
Philadelphia chromosome
83
Myeloproliferative disorder characterized by overproduction of myeloid cells
Chronic myeloid leukemia
84
56 yo WM presents with complaint of fatigue and night sweats. Patient is slightly febrile (100.0'F) and has splenomegaly. You note very elevated WBCs on CBC with a left shift. Suspicion?
CML
85
PBS showing left shift, basophilia, eosinophilia and VERY elevated WBCs
AML
86
CML Treatment
- Imatinib mesylate
| - Dastinib and Nilotinib attack the Philadelpia chromosome directly
87
Clonal malignancy of B lymphocytes
CLL
88
Isolated lymphocytosis, fatigue or lymphadenopathy, hepatosplenomegaly, and immunosuppression
CLL
89
CLL staging: lymphocytosis only
0
90
CLL staging: anemia
III
91
CLL staging: organomegaly
II
92
CLL staging: lymphocytosis + lymphadenopathy
I
93
CLL staging: thrombocytopenia
IV
94
What lymphocyte lineage markers are associated with CLL?
CD19 and CD5
95
CLL Treatment
Fludarabine + Rituximab + Cyclophosphamide
96
Heterogenous group of cancers of lymphocytes that usually present as enlarged lymph nodes
Non-Hodgkin Lymphoma
97
Painless lymphadenopathy that spreads in a non-contiguous fashion whose diagnosis is made by a tissue exam
Non-Hodgkin Lymphoma
98
Patient presents with a high fever, weight loss, drenching night sweats, and large masses in her neck and inguinal area. You suspect infection, but upon palpation, the lymph nodes are non-tender. Suspicion?
Non-Hodgkin lymphoma
99
What might you see on a CXR of a Non-Hodgkin lymphoma patient?
Mediastinal mass
100
Prognostic marker associated with NHL
LDH
101
How do you make a diagnosis of Non-Hodgkin Lymphoma or Hodgkin lymphoma?
Tissue biopsy
102
NHL treatment
- Radiation
| - Combination chemo for aggressive lymphomas
103
Lymphocytic cancer associated with Reed-Sternberg cells
Hodgkin Lymphoma
104
Painless lymphadenopathy that spreads in a contiguous fashion
Hodgkin lymphoma
105
Typical age of Hodgkin lymphoma patients
- Bimodal age distribution
| - 20's, then again in the 50's
106
Patient presents with a high fever, weight loss, drenching night sweats, and large masses behind her ear, in her neck and under her arm (axilla). You suspect infection, but upon palpation, the lymph nodes are non-tender. Suspicion?
Hodgkin lymphoma
107
Non-tender lymphadenopathy, but sometimes associated with pain after ETOH consumption
Hodgkin lymphoma
108
Ann Arbor Hodgkin Lymphoma Stage I
One lymph node region is involved
109
Ann Arbor Hodgkin Lymphoma Stage II
Involvement of 2 lymph node ares on the same side of the diaphragm
110
Ann Arbor Hodgkin Lymphoma Stage III
Lymph node regions on either side of the diaphragm are involved
111
Ann Arbor Hodgkin Lymphoma Stage IV
disseminated disease with bone marrow or liver involvement
112
Hodgkin Lymphoma stage A
Lack of constitutional symptoms
113
Hodgkin Lymphoma Stage B
- > 10% weight loss over 6 months
| - Fever or night sweats
114
Hodgkin Lymphoma Stage IA tx
Radiation
115
Hodgkin Lymphoma Stages I and II tx
Short course of ABVD
116
Hodgkin Lymphoma Stages III or IV tx
Full course of ABVD
117
Malignancy of hematopoietic stems cells terminally differentiated as plasma cells
Multiple myeloma
118
Bone pain, monoclonal paraproteins in serum, clonal plasma cells in bone marrow, organ damage due to plasma cells
Multiple myeloma
119
67 yo WM presents with mucosal bleeding, nausea, visual disterbances, and hard deposits of tissue in his tongue. Suspicion?
Multiple myeloma
120
What kind of symptoms would you expect to find in a patient with multiple myeloma?
- pallor
- bone tenderness
- soft tissue masses
- amyloid symptoms (enlarged tongue, neuropathy, CHF, hepatomegaly)
- neurologic sings
121
Why might you find neurologic signs and symptoms in a patient with multiple myeloma?
-because amyloid deposits may compress the spinal cord
122
What might you expect to find on a PBS of a patient with multiple myeloma?
Rouleaux formations
123
Paraprotein on Serum Protein Electrophoresis (SPEP)
Multiple myeloma
124
If a patient had severe symptoms with multiple myeloma, what might you do if they were < 76 yo?
Autologous stem cell transplantation
125
Recurrent episodes of hemarthroses and arthorpathy
Hemophilia A or B
126
What should you look for in an older patient with a hemophilia?
HIV or Hep C from contaminated blood products
127
Congenital deficiency of coagulation factor VIII
Hemophilia A
128
Chance of developing an inhibitor antibody in hemophilia A
25%
129
Would PTT, PT, or BT be prolonged in hemophilia A?
PTT
130
Tx for hemophilia A
- Cryoprecipitate
- DDAVP (Desmopressin acetate)
- COX 2 inhibitor for arthritis
131
Congenital deficiency of F. IX
Hemophilia B
132
Chance of developing an inhibitory antibody in hemophilia B
< 5%
133
PTT, PT, or BT prolonged in hemophilia B?
PTT
134
Hemophilia B treatment
- FFP
- DDAVP not useful
- COX-2 inhibitor for arthritis
135
Hemophilia C deficiency in what clotting factor?
XI
136
Hemophilia A deficient in what clotting factor?
VIII
137
Hemophilia B deficient in what clotting factor?
F. IX
138
Autoimmune condition in which pathogenic antibodies bind platelets, resulting in an accelerated platelet clearance
Immune Thrombocytopenia (TTP)
139
Isolated thrombocytopenia
Immune thrombocytopenia
140
Treat immune thrombocytopenia if PLT count gets below what?
< 20,000 or 30,000
141
Initial treatment of immune thrombocytopenia
- Prednisone +/- IVIG
| - Anti-D
142
Treatment for severe immune thrombocytopenia
Splenectomy
143
Thrombocytopenia and microangiopathic hemolytic anemia
Thrombotic Thrombocytopenic Purpura
144
Cause of thrombotic thrombocytopenic purpura
- Autoantibody against vWFCP
- Congenital (vWFCP decreased)
- Pregnancy-associated antibody against vWFCP
145
Another thrombotic microangiopathy that's sometimes confused with thrombotic thrombocytopenic purpura
Hemolytic uremic syndrome
146
Signs and symptoms associated with thrombotic thrombocytopenic purpura
- anemia (pallor)
- bleeding
- neurologic problems
147
Thrombotic thrombocytopenic purpura treatment
- plasma exchange
- FFP
- blood transfusions
148
The most common inherited bleeding disorder
-Von Willebrand's Disease
149
Function of vWF
- bridges platelets together and tethers them to the subendothelial matrix at the site of vascular injury
- prolongs half-life of F. VIII
150
Type I vWF disease
-quantitative abnormality of vWF
151
Type 2 A or 2B vWF disease
-qualitative defect of vWF
152
Type 2M vWF disease
Defect in vWF that decreases binding to platelets
153
Type 2N vWF disease
Defect in vWF that decreases binding to V. FIII
154
Type 3 vWF disease
- undetectable levels of vWF
| - Rare
155
Signs and symptoms of vWF type 1
Mild-moderate platelet-type bleeding (skin and mucosal)
156
Type 2 vWF disease signs and symptoms
Moderate-severe bleeding
157
Mild-moderate bleeding; vWF activity and antigen are mildly depressed
Type I vWF disease
158
Moderate to severe bleeding; vWF antigen to activity is 2:1
Type 2A or 2B vWF disease
159
vWF disease platelet count
normal - decreased
160
vWF disease PT
normal
161
vWF disease PTT
Normal - prlonged
162
vWF disease bleeding time
prolonged
163
vWF disease fibrinogen time
normal
164
vWF disease tx
- DDAVP
- Antifibrinolytic therapy
- Cryoprecipitate if severe
