Hematology exam review (summer) Flashcards
(342 cards)
1
Q
Hgb number in anemia?
A
Male <12
Female <15
2
Q
Where is iron absorbed?
A
Duodenum
3
Q
What conditions is iron absorbed?
A
Acidic conditions
4
Q
Which iron study reflects total body stores of iron? Normal value?
A
Serum Fe
>30 is normal
5
Q
Men and non-menstural women need how much iron/day?
A
1mg/day
6
Q
Menstural women need how much iron/day?
A
3-4mg/d
7
Q
Preggers women need how much iron/day?
A
2-5mg/d
8
Q
Normal Recitulocyte Count range?
A
0.5-2%
9
Q
What does Reticulocyte Count measure?
A
Bone marrow function.
10
Q
High Reticulocyte Count means what type of cells being produced?
A
Immature cells
11
Q
Normal Reticulocyte Index Count range?
A
2-3
12
Q
Normal Serum B12? Deficiency?
A
Normal >210
Deficiency <170
13
Q
Normal RBC Folic Acid level? Deficiency?
A
Normal >140
Deficiency <140
14
Q
What is #1 MC anemia?
A
Iron Deficiency Anemia
15
Q
MC cause of Iron Deficiency Anemia?
A
BLEEDING (menstural or GI)
16
Q
How often screen 12-18 y/o F (non-preggers) for Iron Deficiency Anemia?
A
q5-10 year
17
Q
When to first screen preggers for Iron Deficiency Anemia?
A
At first prenatal visit
18
Q
When to screen men and post-menopausal F for Iron Deficiency Anemia?
A
No screening
19
Q
What very weird craving in Iron Deficiency Anemia?
A
Pagophagia (ice craving)
20
Q
What happens to tongue, nails, and corners of mouth in severe Iron Deficiency Anemia?
A
Brittle nails, nail spooning (koilonychia), and cheilosis of mouth corners
21
Q
How much iron lost from menstural bleed?
A
1mg/day
22
Q
How much iron lost from preggers, delivery, and nursing?
A
1g/d
23
Q
How much iron lost per 100mL whole blood?
A
50mg Fe per 100mL whole blood
24
Q
CBC and Iron Deficiency Anemia?
A
Hgb low, RBC low
25
TIBC up or down in Iron Deficiency Anemia?
Up. More binding places for iron due to not enough iron.
26
Serum Fe, Serum Iron, and % Sat in Iron Deficiency Anemia?
DOWN
27
Two essential lab tests for Iron Deficiency Anemia?
Hgb <12 or 15, Serum Fe <30
28
MCV and MCHC levels in Iron Deficiency Anemia?
Microcytic <80
| Hypochromic <27
29
TOC for Iron Deficiency Anemia?
Ferrous Sulfate 325mg TID x1-2 mo until normal CBC
30
When to do CBCs in Iron Deficiency Anemia?
3 weeks (half better), 2 months (normal), 6 months (normal and stores rebuilt)
31
Tx of Iron Deficiency Anemia months 3-6?
325mg PO qd to rebuild stores
32
2 things which increase iron absorption?
1. Empty stomach
| 2. Orange juice
33
SEs of Ferrous Sulfate?
Nausea, constipation, diarrhea, dark stool. Reduce dose.
34
When to use IV iron in Iron Deficiency Anemia?
Can't absorb iron orally. IBD, gastric bypass, etc.
35
What is reduced in Thalassemia?
Reduced production of alpha or beta blobin chains
36
Alpha Thalassemia reduction of what?
Reduced alpha globin production
37
Who gets Alpha-1-Thalassemia? (two)
MC in S.E. Asia and China
38
Geneotype in Alpha-1-Thalassemia?
Heterozygous. Each parent missing one gene.
| alpha ____/alpha ____
39
Who gets Alpha-2-Thalassemia?
MC black
40
Geneotype in Alpha-2-Thalassemia?
Homozygous. One parent missing each.
| alpha alpha/____ ____
41
Genotype in Alpha Thalassemia Intermedia (aka Hgb H Dz)?
alpha ___/___ ___
42
Genotype of Alpha Thalassemia Majora (aka Hydrops Fetalis)?
No alpha globin at all
| ___ ___ / ___ ___
43
MCV in Alpha Thalassemia?
Microcytic
44
What does electrophoresis show in Alpha Thalassemia trait?
Normal Hgb.
45
What does electrophoresis show in Alpha Thalassemia Intermediate (Hgb H Dz)?
10-40% HgbH
46
S/Sx of Alpha Thalassemia trait?
No clinical S/Sx.
47
Tx of Alpha Thalassemia trait? Life expectency?
No tx. Normal expectency.
48
S/Sx of Alpha Thalassemia Intermediate (Hgb H Dz)?
Pallor, splenomegaly, fatigue, weakness
49
TIBC normal/increased/decreased in Alpha Thalassemia?
Normal or increased
50
Iron normal/increased/decreased in Alpha Thalassemia?
Normal or increased
51
Tx for Alpha Thalassemia Intermediate (Hgb H Dz)?
Folic Acid 1mg PO
52
What to avoid with Alpha Thalassemia Intermediate (Hgb H Dz)?
Avoid oxidative (sulfa) drugs, avoid iron
53
When to tranfuse PRBC in Alpha Thalassemia Intermediate (Hgb H Dz)? Goal Hgb?
If severely low Hgb. Goal 9-10.
54
Tx for refractory Alpha Thalassemia Intermediate (Hgb H Dz)? (Hint: take something out)
Splenectomy
55
When needed if getting regular transfusions for Alpha Thalassemia?
Iron chelation with Deferastrox 20-30mg/kg/d
56
Type of counseling needed for Alpha Thalassemia?
Genetic
57
Genetic bases of Beta Thalassemia?
Point delection on chromosome 11
58
Which globin chains are affected in Beta Thalassemia?
Beta-globin chains
59
What happens to Beta-globin chains in Beta Thalassemia?
Replaced with Beta-like chains (Delta and Gamma)
60
Beta Thalassemia MC in which population? (Hint: 2)
Mediterranian and Middle Eastern
61
Genotype of Beta Thalassemia Minor (aka Trait)? (Hint: two)
B/B+ or B/B˚
62
What percent are Beta-chains reduced in Beta Thalassemia Minor/Trait?
50%
63
S/Sx of Beta Thalassemia Minor/Trait?
ASx
64
CBC shows what in Beta Thalassemia Minor/Trait?
Clinically insignificant microcytic anemia
65
Electrophoresis shows what in Beta Thalassemia Minor/Trait?
HgbA2 4-8%
66
Tx required in Beta Thalassemia Minor/Trait?
No tx required
67
Counseling in Beta Thalassemia Minor/Trait?
Genetic counseling
68
Genotype of Beta Thalassemia Intermedia?
B+/B+
| homozyous
69
When does Beta Thalassemia Intermedia begin to manifest?
6mo old when HgbF goes away
70
Where are bone deformities in Beta Thalassemia Intermedia?
Face
71
What type of fractures in Beta Thalassemia Intermedia?
Pathologic
72
Spleen and liver in Beta Thalassemia Intermedia?
Hepatosplenomegaly
73
When does severe anemia start in Beta Thalassemia Intermedia?
6 months
74
Three types of hemoglobin in Beta Thalassemia Intermedia?
HgbA 30%
HgbA2 10%
HgbF 6-100%
75
What does Peripheral Smear show in Beta Thalassemia Intermedia?
Bsophillic Stipling
76
Beta Thalassemia Majora aka?
Cooley Anemia
77
Growth and deformity in Beta Thalassemia Majora/Cooley Anemia?
Stunted growth, bone deformity
78
Spleen and liver in Beta Thalassemia Majora/Cooley Anemia?
Hepatosplenomegaly and jaundice
79
MCV in Beta Thalassemia Majora/Cooley Anemia?
Severe low
80
Majority of Hgb type in Beta Thalassemia Majora/Cooley Anemia?
HgbF
81
Tx of choice in Beta Thalassemia Majora/Cooley Anemia?
Allogeneic stem cell transplant
82
How often to transfuse PRBC in Beta Thalassemia Majora/Cooley Anemia?
Weekly
83
What to avoid in Beta Thalassemia Majora/Cooley Anemia?
Oxidative meds (sulfa, iron)
84
How to treat iron overload?
Deferoxamine
85
Prophyria due to what?
No porpherin ring in RBC
86
Skin in Prophyria?
Very pale skin
87
Light exposure does what in Prophyria?
Burns, blisters
88
Teeth in Prophyria?
Vampire-like teeth
89
Anemia of Chronic Dz due to what?
Conditions which reduce iron uptake
90
EPO response in Anemia of Chronic Dz?
Down
91
S/Sx in Anemia of Chronic Dz?
Fatigue, weak, dizzy
92
Hepcidin levels in Anemia of Chronic Inflammation?
Increased!
93
Serum Iron levels in Anemia of Chronic Inflammation?
Low
94
Serum Fe levels in Anemia of Chronic Inflammation?
Normal or inc
95
RBC life and mass in Anemia of Chronic Dz?
Decreased
96
Tx for Anemia of Chronic Dz?
Tx underlying condition
| Folic Acid and/or Iron def
97
When to transfuse PRBC in Anemia of Chronic Dz?
If severe anemia
98
What med to give in Hgb <10 in Anemia of Chronic Dz? Goal Hgb?
Recombinant EPO until 10-12
99
Shape of Sideroblast in Sideroblastic Anemia?
Ringed sideroblast.
100
Where is ringed sideroblast found in Sideroblastic Anemia?
Bone Marrow
101
Iron levels in Sideroblastic Anemia?
Too much iron. Can't get into RBC.
102
4 causes of acquired Sideroblastic Anemia?
1. Cu deficiency
2. INH
3. Linezolid
4. Chloramphenicol
103
Tx for INH acquired Sideroblastic Anemia?
B6 to fix
104
TIBC in Sideroblastic Anemia?
DOWN
105
SI, SFe, and % Sat in Sideroblastic Anemia?
Up!
106
Dx for Sideroblastic Anemia?
Bone marrow biopsy
107
Why splenectomy in congenital Sideroblastic Anemia?
Tertiary RBC production center.
108
Lead Toxcity does what to iron binding?
Decreases iron binding in proporphyrin ring
109
Chromic in Lead Toxcity?
Hypochromic
110
What type of cells seen on peripheral smear in Lead Toxcity?
Basophillic Stipiling
111
Basophillic Stipiling seen in what type of toxicity?
Lead Toxcity
112
Tx for Lead Toxcity?
Chelate
113
MCV in Alcoholic Anemia?
Macrocytic 100-110
114
How much EtOH per day to cause Alcoholic Anemia?
80g/day (1 bottle of wine)
115
Tx for Alcoholic Anemia? When resolves?
Stop drinking. Resolves in 2-4 months.
116
Myelodysplastic Syndromes are due to an inappropriately low response to what?
Reticulocytes
117
Which population has 2-3x higher instance of Aplastic Anemia?
East Asian
118
Ages of Aplastic Anemia?
Biphasic. 10-25, >60.
119
Aplastic Anemia due to injury of what?
Pluripotent stem cells in BM. Impairs proliferation and differentiation.
120
What is low in Aplastic Anemia?
Low PLT, RBCs, WBCs
121
Causes of Aplastic Anemia?
Viruses, drugs, chemo, radiation, toxins
122
What does CBC show in Aplastic Anemia?
Pancytoanemia
123
What does Peripheral Smear show in Aplastic Anemia?
Look normal but reduced number
124
Reticulocyte Count/Index increased or decreased in Aplastic Anemia?
Decreased
125
Tx for Aplastic Anemia under 20 y/o?
Allogenic hepatopoietic stemm cell transplant
126
Tx for Aplastic Anemia under 50 y/o?
Immunisuppressive therapy
127
Most common type of Aplastic Anemia?
Fanconi Anemia
128
Fingers and Fanconi Anemia?
Sindactyly (fused digits)
129
Where is B12 absorbed?
Terminal Ileum
130
What is B12 a cofactor for synthesis of? (Hint: 2)
1. Nucleic acid synthesis
| 2. Myelin synthesis
131
How long are B12 stores good for?
3 years
132
Causes of B12 anemia?
Vegans, Pernicious Anemia, Chron's DZ, EtOH
133
Pernicious Anemia what type immune disease?
Autoimmune.
134
What is destroyed in Pernicious Anemia by Abs?
Parietal cells and intrinsic factor
135
MCV in Pernicious Anemia?
>115. Megaloblastic anemia.
136
Blood smear in Pernicious Anemia?
Hypersegmented neurophils and macro-ovalcytes.
137
Sx in Pernicious Anemia?
Anemia, pale, neuro symptoms (vibration, proprioception, memory)
138
When do Sx develop in Pernicious Anemia?
About 3 years
139
Normal Serum B12? When deficient?
Normal >210
| Deficient <170
140
Tx for Pernicious Anemia?
VitB12 100mcg SC/IM qd x1wk, then 1/wk, then monthly for life
141
When do check CBC and B12 levels in Pernicious Anemia?
1-2 wk, 2 mo, then periodic indefinitely
142
What is the natural form of Folic Acid called?
Folate
143
Daily intake of Folic Acid is preggers or lactating?
600-800mcg
144
Folic Acid absorbed where?
Duodenum and jejunum
145
Folic Acid in what foods?
Fruits and veggies
146
How long are stores of Folic Acid?
2-3 months
147
MC cause of Folic Acid decifiency?
Inadequate intake. (Then EtOH abuse.)
148
MCV in Folic Acid decifiency?
Megaloblastic. MCV >115.
149
When do Sx appear in Folic Acid decifiency?
4-5 months
150
Tx for Folic Acid decifiency?
Folic Acid 1mg PO qd x1-4mo or until hematologic recovery
151
Expected response from tx in Folic Acid decifiency?
Rapid improvement and sense of well-being
152
When will see reticulocytosis after tx in Folic Acid decifiency?
5-7d
153
Sickle Cell is autosomal recessive or dominant?
Recessive
154
Hgb type in Sickle Cell? Which Hgb completely absent?
HgbS
| HgbA absent.
155
AA substitution in Sickle Cell? In which globin chain?
Glutamic Acid becomes Valine in Beta-globin chain.
156
When do HgbS change into and do poorly when deoxygenated?
Poorly soluable. Turn into sickle shape.
157
HgbS RBC lifespan?
10-20 days.
| Normal HgbA is 100 days.
158
What does Sickle Cell cause in microcirculation?
Recurrent vasoocclusion
159
What is a Sickle Cell Crisis? What causes it?
Acute episode with many sickle cells. Due to Infection, hypoxia, dehydration, acidosis, exercise at high altitude
160
What bacteria type due to bad spleen in Sickle Cell?
Encapsulated (pneomococcal, h flu, meningitis)
161
What is Chest Syndrome in Sickle Cell?
Lung injury and infarction due to occlusion
162
DX for Sickle Cell?
Hgb electrophoresis. Shows HgbS.
163
Daily tx for Sickle Cell?
Folic Acid 1mg PO qd
164
What to avoid in Sickle Cell?
Precipitation factors
165
What to give in acute crisis in Sickle Cell?
IV hydration, oxygen, and lots of analgesia for pain.
166
Tx for intractable pain or severe chest syndrome in Sickle Cell?
Exchange transfusion
167
What does Hydroxyurea do for Sickle Cell?
Increased production of HgbF
168
Tx for children with Sickle Cell?
Allogenic hematopoietic stem cell transplant
169
G6PD deficiency MC in?
Back males
170
What do G6PD enzymes do?
Maintain RBC membrane from oxidative stress
171
Does G6PD deficiency cause immune or non-immune hemolytic anemia?
Non-immune
172
G6PD deficiency due to what?
X-linked mutation. RBC damage triggered by Oxidative drugs, infection, or fava beans.
173
How often is hemolytic anemia in G6PD deficiency? (Constant, episodic, rarely, never?)
Episodic
174
What drugs to avoid in G6PD deficiency?
Oxidative drugs.
| Bactrim, Dapsone (TB and Leprosy, antimalarials, INH, etc.
175
Coombs test in G6PD deficiency?
Negative
176
When will G6PD enzyme assay be low in G6PD deficiency?
During or after hemolysis
177
Peripheral Smear shows what type of cells in G6PD deficiency?
Shistocytes with bite or blister fragments
178
Tx for G6PD deficiency?
Self-limiting. Avoid oxidating drugs and fava beans. Transfuse PRBC when severe or symptomatic anemia.
179
Hemolytic Anemia do to what?
RBC destruction
180
Do Petechia and Purpura blance?
No
181
What does increased indirect bilirubin cause?
Jaundice (yellowed eyes and skin)
182
Dark urine due to what?
Increased bilirubin
183
Direct Coombs tests for what?
RBCs coated with Ab or complement
184
Indirect Coombs tests for what?
Ab against in RBCs in serum
185
MC Hemolytic Anemia?
Hereditary Sperocytosis
186
Hereditary Sperocytosis due to defect where in RBCs?
Membrane defect
187
Hereditary Sperocytosis and coombs test?
Negative! Not an immune issue!
188
Peripheral smart in Hereditary Sperocytosis?
Spherocytes
189
Tx for Hereditary Sperocytosis?
Supportive care and Folic Acid. Transfuse PRBC as needed. Splenectomy if severe.
190
Autoimmune Hemolytic Anemia cause?
IgG Ab bind to RBC membrane at room temp and activate complement to attack RBC membrane. "Warm".
191
Which Coombs test in Autoimmune Hemolytic Anemia?
Positive direct coombs!
192
Tx for Autoimmune Hemolytic Anemia?
Steroids to blunt immune system. Splenectomy to remove site of RBC destruction. Plasma pheresis and transfusion.
193
What is Leukemia a malignancy of?
WBCs
194
Leukemia causes bone marrow to produce what number of WBCs?
Increased # of RBCs
195
What happens to cell lines in Leukemia?
Pancytopenia (Neutropenia, anemia, thromboytopenia)
196
Meyloid line gives rise to which WBCs?
Neutrophila
Eosinophils
Basophils
197
Which are the "granulocytes"?
Neutrophila
Eosinophils
Basophils
198
Lymphoid line gives rise to which WBCs?
B and T Lymphocytes
199
Which leukemia developes fast and has signs/symptoms- Acute or Chronic?
Acute leukemia
200
Speed of chronic leukemias? Sx?
Slow/insidious. ASx until progession or blast crisis.
201
Chronic Lymphocytic Leukemia (CLL) MC in who? (color and age)
White males, >65
202
Chronic Lymphocytic Leukemia malignancy of which WBC?
B Lymphocyte cells
203
Chronic Lymphocytic Leukemia S/Sx? Speed?
Slow onset. Often ASx.
204
Dx for Chronic Lymphocytic Leukemia?
>5000 absolute B-Lymphocutes in peripheral smear >3mo
205
Tx for Chronic Lymphocytic Leukemia?
Only tx if +sx.
206
Cure for Chronic Lymphocytic Leukemia?
Allogeneic stem cell t/p
207
What is the MC childhood malignancy?
Acute Lymphocytic Leukemia "ALL"
208
Acute Lymphocytic Leukemia malignancy of which WBCs?
Clonal proliferation of immature B and T Lymphocytes in BM.
209
Onset of Acute Lymphocytic Leukemia?
Rapid onset
210
S/Sx in Acute Lymphocytic Leukemia?
Fever, fatigue, pallor
211
Anemic picture in Acute Lymphocytic Leukemia?
Pancytopenia/Anemia
212
DX for Acute Lymphocytic Leukemia?
BM Biopsy showing hypercellular >20% blasts
213
When to transfuse PRBC in Acute Lymphocytic Leukemia?
Hgb <8
214
Tx for Acute Lymphocytic Leukemia?
Chemo.
215
When to do BM transplant in ALL?
If relapses
216
Philadelphia Chromosome in which leukemia?
Chronic Myeloid Leukemia
217
Chronic Myeloid Leukemia malignant proliferatin of which WBC line?
Granulocytic/Meyloid line (N.E.B)
218
Chronic Myeloid Leukemia MC in what age?
>50 y/o
219
Speed of onset in Chronic Myeloid Leukemia?
Slow
220
Chronic Myeloid Leukemia asx until what?
Blastic crisis
221
WBC number in Chronic Myeloid Leukemia?
>100,000
222
Age of granulocytes in Chronic Myeloid Leukemia?
Increased number of old granulocytes with low or no leukocyte alk phos
223
Tx for Chronic Myeloid Leukemia?
Treat anemias. TKIs.
224
WHat is MC leukemia in adults?
Acute Myeloid Leukemia
225
What percent of blasts in BM with Acute Myeloid Leukemia?
>20% blasts in BM
226
Time of onset in Acute Myeloid Leukemia?
Rapid onset
227
Bleeding in Acute Myeloid Leukemia?
Mucocutaneous bleeding. Petechiae and ecchymosis.
228
Acute Myeloid Leukemia and gingiva
Gingivitis
229
Peripheral smear shows what kind of cells in Acute Myeloid Leukemia?
Auer Rods
230
Auer Rods in which leukemia?
Acute Myeloid Leukemia
231
Tx for Acute Myeloid Leukemia?
Chemo + bone marrow transplant
232
Lymphoma is a malignancy of which tissues?
Lymphatic tissue
233
2 MC lymphatic tissues?
Lymphnodes, spleen
234
2 types of Lymphoma?
1. Hodgkins Lymphoma
| 2. Non-Hodgkins Lymphoma
235
Reed-Sternberg Cells seen in which Lymphoma?
Hodgins Lymphoma
236
MC age for Non-Hodgkins Lymphoma?
>50 y/o
237
2 types of Non-Hodgkins Lymphoma?
1. Indolent
| 2. Aggressive
238
S/Sx of Indolent Non-Hodgkins Lymphoma?
Slow, waxing/waning s/sx. Hepatosplenomegaly. Painless lymphadenopathy. B Symptoms.
239
Is the lymphadenopathy in Lymptoms painful or painless?
PAINLESS
240
What are the B-symptoms in Lymphoma?
Fever, night sweats, weight loss
241
S/Sx of Aggressive Non-Hodgkins Lymphoma?
Acute, rapidly growing painless lymphedenopathy. Hepatosplenomegaly. Extranodal involvement on GI, skin, CNS
242
Dx of Non-Hodgkins Lymphoma?
Lymph node and tissue biopsy
243
What test to stage Non-Hodgkins Lymphoma?
PET/CT
244
Stage 1 Lymphoma how many lymph nodes?
Single lymph node
245
Stage 2 Lymphoma how many lymph nodes?
≥2 lymph nodes on same side of diaphragm
246
Stage 3 Lymphoma how many lymph nodes?
Both sides of diaphragm
247
Stage 4 Lymphoma how many lymph nodes?
In extra-lymphatic sites (liver, lungs, B.M.)
248
Hodgkins Lymphoma MC in people with what other dz?
HIV
249
Ages who get Hodgkins Lymphoma?
25-30, >55 y/o
250
Type of cells shown on excisional biopsy of Hodgkins Lymphoma?
Reed-Sternberg Cells
251
Multiple Myeloma is malignant proliferation of which cells?
Plasma cells d/t overproduction of monoclonal immunoglobulin
252
Multiple Myeloma d/t over production of what?
Monoclonal immunoglobulin (esp IgA and IgG) -> increased plasma cells
253
Where do plasma cells accumulate in Hodgkins Lymphoma?
Bone Marrow
254
MC bone malignancy?
Multiple Myeloma
255
Multiple Myeloma age of onset?
4-6th decade of life
256
Exposure to what two big things increase risk of Multiple Myeloma?
Radiation and pesticide exposure
257
Where is pain in Multiple Myeloma?
Bone pain. MC in spine and ribs.
258
What is bone pain due to in Multiple Myeloma?
Boney lytic lisons
259
What "formation" seen on CBC in Multiple Myeloma?
Roulex Formation (RBCs sticking together)
260
Calcium and Multiple Myeloma?
Increased serum Ca2+ d/t lytic lesions
261
ESR in Multiple Myeloma?
Increased
262
Tx for Hypercalcemia and pathologic fx in Multiple Myeloma?
Bisphosphonates
263
Tx for Multiple Myeloma?
Hematopoietic stem cell transplant + chemo
264
Hemophilia A is a deficiency of which factor?
Factor 8
265
Which pathway does Hemophilia A affect?
Instrinsic pathway
266
Lack of Factor 8 causes what to not form?
Thrombin
267
MC hemophilia?
Hemophilia A
268
Where does bleeding MC occur in Hemophilia A?
Weight-bearing joints. "Hemarthosis".
269
Hemophilia A and aPTT time?
Prolonged!
270
How to dx Hemophilia A?
Very low F8 levels. <10IU.
271
How to treat mild Hemophilia A?
Demopressin. Releases stored Factor 8 from endothelium
272
Emergent tx for Hemophilia A?
IV Fresh frozen Plasma
273
Office treatment for severe Hemophilia A?
Recombinant Factor 8
274
Who gets Hemophilia A?
Males only. X-linked.
275
Hemophilia B due to lack of which factor?
Factor 9 deficiency
276
Where is bleeding in Hemophilia B?
Deep tissue bleeding
277
Hemophilia A and B bleed more or longer?
More, not longer.
278
Hemophilia B and aPTT?
Prolonged PTT
279
Office tx of Hemophilia B?
Factor 9 infusion
280
Emergent tx of Hemophilia B?
IV FFP
281
Hemophilia C due to which Factor?
Factor 11 decifiency
282
Disseminated Intravascular Coagulation due to sort of activation?
Pathological activation of coagulation system
283
What does pathological activation of coagulation system lead to in DIC?
Widespread microthrombi which use up coagulation proteins
284
Widespread microthrombi which use up coagulation proteins leads to what in DIC?
Severe thrombocytopenia and bleeding
285
What caues the pathological activation in DIC?
Infx, malignancy, OB issues, trauma/burns, rattlesnake bites
286
When to transfuse platlets in DIC?
If <20k
287
How to replace coag factors in DIC?
IV FFP
288
How to replace Fibrinogen in DIC?
Cryopercipitate
289
What to use for thrombosis in DIC?
Heparin
290
Polycythemia Vera causes what to be overproduced?
RBCs
291
Viscosity in Polycythemia Vera?
Increased
292
Polycythemia Vera age of onset?
60 y/o
293
What is Erythromelalgia in Polycythemia Vera?
Burning pain in hands and feet d/t increased PLT number and aggregation. Red/blue skin color.
294
Aquagenic Pruritus and Polycythemia Vera?
Itching after warm water exposure
295
Hgb, HCT, WBC, and PLT levels in Polycythemia Vera?
Increased
296
EPO levels in Polycythemia Vera?
Decreased!
297
Goal of tx in Polycythemia Vera?
Decreased thrombotic complications. Give 81mg ASA.
298
Phlebotomy target in Polycythemia Vera?
<45%
299
Idiopathic Thrombocytopenia immune or non-immune?
Autoimmune. Ab destroy megakaryocutes.
300
Idiopathic Thrombocytopenia MC in?
Kids s/p viral infection
301
Where is bleeding in Idiopathic Thrombocytopenia
Gingival and mucosal bleed
302
Rash in Idiopathic Thrombocytopenia?
Purpuric rash with petechiae.
303
Tx in Idiopathic Thrombocytopenia?
High dose glucocorticoids to blunt immune response.
304
Tx of Idiopathic Thrombocytopenia if severe?
IVIG
305
Tx of refractory Idiopathic Thrombocytopenia?
Splenomegaly
306
Thombotic Thombocytopenic Purpura due to what being destroyed?
Enzyme ADAMTS12
307
What does enzyme ADAMTS13 do?
Cuts large vonWillibrant Factor
308
What happens to RBCs in Thombotic Thombocytopenic Purpura?
Damaged due to microthombi. Causes intravasular hemolysis and shistocyte formation.
309
Pentad of S/Sx in Thombotic Thombocytopenic Purpura?
1. Bruising and purpura
2. Jaundice
3. Neuro problems (HA, AMS, CVA, hallucinations)
4. Renal failure
5. Fever
310
PLT count in Thombotic Thombocytopenic Purpura?
Very low PLT count
311
TOC in Thombotic Thombocytopenic Purpura?
Early plasmapheresis qd
312
2 major roles of von Willenbrand Factor?
1. Mediates adhesion of PLTs to site of vascular injuey
| 2. Binds and stabilize Factor 8
313
What is MC herediatary coagulation abnormality?
von Willenbrand Disease
314
von Willenbrand Disease d/t what?
Deficiency of vWF
315
MC type of von Willenbrand Disease?
Type 1= decreased vWF levels
316
S/Sx of Type 1 von Willenbrand Disease?
Asx or mild
317
Type 2 vW Disease due to what?
Quality defect of vWF
318
Type 3 vW Disease due to?
Complete absence of vWF -> decreased Factor 8 levels
319
Where bleed and bleeding time in von Willenbrand Disease?
Mucosal bleed- epistaxis, gingival, and menorrhagia.
| Increased bleeding time.
320
PTT normal or prolonged in vW Disease?
Prolonged
321
Tx for moderate vW Disease?
Desmopressin
322
Tx for severe/Type 3 vW Disease
Virus inactivated vW Factor or cryopercipitate/FFP
323
PPX for surgery in vW Disease?
vWF-containine F8 concentrates
324
Heparin Induced Thrombocytopenia due to?
UFH exposure. Massive PLT decrease.
325
Type 1 HIT within how many hours of UFH explosure?
48h
326
Type 1 HIT immune or non-immune?
Non-immune
327
What will happen to PLTs in Type 1 HIT with continued exposure?
Normalize with continued UFH exposure
328
What happens to PLTs in Type 2 HIT?
PLTs activated after UFH exposure. CVA/MI/DVT/PE.
329
HIT risk decreased with which other heparin?
LMWH
330
Dx of Heparin Induced Thrombocytopenia?
HIT antibodies
331
Tx for HIT?
D/C UFH immediately
332
What to use instead of UFH for Warfarin bridge if have HIT?
Direct Thrombin Inhibitor (Dargatroban, Lepiridu)
333
When safe to switch to Warfarin in HIT if using Direct Thrombin Inhibitor?
When PLT >150,000
334
Tranfuse PLT in HIT?
NO! Risk Thrombosis!
335
HIT more than 100 days ago. OK to use UFH?
Yes
336
Warfarin's MOA?
Decreases Vitamin K dependent clotting factors and decreases Vitamin K reserves
337
Warfarin indicated for?
DVT, PE, CVA, Afib, valve replacement
338
Warfarin dose?
2-10mg PO qd
339
Goal INR with Warfarin?
2-3
340
Warfarin's inhibitors?
Vitamin K PO or IV.
| Prothrombin Complex Concentrate > FFP
341
Warfarin CI'd with which 2 classes of anticoagulants?
1. IIa inhibitors
| 2. Xa inhibitors
342
Warfarin PT education
- Inform all medical and dental providers
- Soft toothbrush
- No heavy contact sports
- No huge increase in leafy greens (contain VitK, can decrease INR)
- No new multivitamins, OTCs, or herbal supplements
- No preggers
