Hematology Flashcards
1
Q
all forms of anemia lead to
A
fatigue/loss of energy
2
Q
pt with severe anemia will have
A
SOB, lightheadedness, or confusion
3
Q
diseases with similar presentations to anemia:
A
- hypoxia - CO poisoning - methemoglobinemia - ischemic heart disease
4
Q
craving for ice or dirt, think
A
anemia
5
Q
physical exam findings on anemic patient
A
- pallor - flow murmur - pale conjunctiva
6
Q
physical exam findings in hemolytic anemia
A
- jaundice - scleral icterus
7
Q
in severe anemia, what is needed to exclude ischemia?
A
EKG
8
Q
best INITIAL test for anemia
A
CBC with peripheral smear
9
Q
additional initial tests for anemia
A
- reticulocyte count - haptoglobin - LDH - total and direct bilirubin - TSH and free T4 - B12/folate levels - iron studies - UA w/ microanalysis
10
Q
categorization of anemia is based on
A
MCV (mean corpuscular volume)
11
Q
what value may indicate if there’s a problem with the synthesis of Hb?
A
MCHC (mean corpuscular hemoglobin concentration)
12
Q
anemia categorized based on MCV is as follows:
A
- microcytic - macrocytic - normocytic
13
Q
based on MCHC anemia can be further categorized as follows:
A
- hypochromic - hyperchromic - normochromic
14
Q
- blood loss - THROMBOCYTOSIS what is the diagnosis?
A
IDA
15
Q
- RA - ESRD - any chronic infectious, inflammatory, or connective tissue disease what is the diagnosis?
A
ACD
16
Q
- very small MCV w/ few or no symptoms - TARGET cells what is the diagnosis?
A
thalassemia
17
Q
- alcoholic - INH - lead exposure what is the diagnosis?
A
sideroblastic anemia
18
Q
best INITIAL test for IDA, ACD, thalassemia, or sideroblastic anemia
A
iron studies
19
Q
results of iron studies in IDA
A
- low ferritin - high TIBC (total iron binding capacity) - low iron - low iron saturation - elevated RDW (red cell distribution width)
20
Q
results of iron studies in ACD
A
- HIGH ferritin (acute phase reactant) - low TIBC (total iron binding capacity) - low iron - normal or low iron saturation
21
Q
results of iron studies in thalassemia
A
NORMAL
22
Q
results of iron studies in sideroblastic anemia
A
HIGH IRON
23
Q
MOST ACCURATE test for IDA
A
bone marrow biopsy (don’t do this on CCS exam)
24
Q
MOST ACCURATE test for thalassemia
A
Hb electrophoresis (beta: elevated HbA2, and HbF) (alpha: normal)
25
MOST ACCURATE test for sideroblastic anemia
Prussian blue stain
26
best INITIAL treatment for IDA
ferrous sulfate PO
27
best INITIAL treatment for ACD
correct underlying cause
28
best INITIAL treatment for thalassemia
no treatment for trait
29
best INITIAL treatment for sideroblastic anemia
minor: pyridoxine (vitamin B6) major: remove toxin exposure
30
MOST ACCURATE test for ALPHA thalassemia
DNA sequencing
31
name the 4 types of microcytic anemia
1. IDA 2. ACD 3. thalassemia 4. sideroblastic anemia
32
causes of macrocytic anemia
- vitamin B12 deficiency - folate deficiency
33
which medication blocks B12 absorption?
metformin
34
B12 deficiency causes:
- hypersegmented neutrophils - peripheral neuropathy (can cause any neuro sx) - glossitis (smooth tongue) - diarrhea
35
folate deficiency causes:
- hypersegmented neutrophils only
36
labs seen in B12 defiicincy
- DECREASED reticulocyte count - elevated bilirubin - elevated LDH
37
mechanism of elevated indirect bilirubin and LDH in B12 deficiency
"ineffective erythropoiesis" RBCs are destroyed as they leave bone marrow
38
MOST ACCURATE tests for B12 and folate deficiencies
B12 and folate levels
39
next step if B12 deficiency is SUSPECTED, but B12 level is NORMAL
methylmalonic acid level
40
after finding low B12 level or elevated methylmalonic acid level, next best step to confirm etiology of B12 deficiency
antiparietal cell Ab and anti-intrinsic factor Ab
41
treatment for B12 and folate deficiencies
replace
42
first thing that improves after B12 replacement therapy
reticulocytes
43
last thing that improves after B12 replacement therapy
neurological abnormalities
44
name the hemolytic anemias (9)
1. sickle cell anemia 2. Hb sickle cell disease 3. autoimmune hemolysis 4. cold-induced hemolysis (cold agglutinins) 5. glucose-6-phosphate dehydrogenase (G6PD) deficiency 6. pyruvate kinase deficiency 7. hereditary spherocytosis 8. hemolytic uremic syndrome (HUS), and thrombotic thrombocytopenic purpura (TTP) 9. paroxysmal nocturnal hemoglobinuria (PNH)
45
all forms of hemolytic anemia present with
SUDDEN ONSET of weakness and fatigue
46
diagnostic testing for hemolysis shows the following
- elevated indirect bilirubin - elevated reticulocyte count - elevated LDH - decreased haptoglobin - spherocytes on smear
47
in hemolysis, why is indirect bilirubin elevated, and haptoglobin decreased?
- limited capacity of liver to glucuronidate indirect to DIRECT bilirubin - haptoglobin is a transport for indirect bilirubin which is quickly used up
48
what electrolyte should be monitored after treating vitamin B12 deficiency?
K+ (can be low)
49
- very severe chest, back, and thigh pain
sickle cell anemia
50
best INITIAL treatment for sickle cell anemia
- OXYGEN - HYDRATION - PAIN MEDICATIONS - ABX (ceftriaxone, levofloxacin, or moxifloxacin)
51
why is FEVER an EMERGENCY in a pt w/ sickle cell anemia?
NO SPLEEN
52
tests that should be ordered for pt with sickle anemia
- blood cultures - UA - reticulocyte count - CBC - CXR
53
if fever is present after treatment for sickle cell anemia, then?
physical examination
54
possible physical exam findings in sickle cell anemia: - HEENT - CVS - respiratory - abdomen - extremities - neurological
HEENT- retinal infarction CV- flow murmur from anemia respiratory- rales, or consolidation from infection or infarction abdomen- splenomegaly in children, absence of spleen in adults extremities- skin ulcers, aseptic necrosis of hip (found on MRI) neurological- stroke (current or previous)
55
when is exchange transfusion done in sickle cell anemia?
- eye: visual disturbance from retinal infarction - lung: pulmonary infarction leading to pleuritic chest pain and abnormal CXR - penis: priapism from infarction of prostatic plexus of veins - brian: stroke
56
what is the goal of exchange transfusion?
decrease Hb S to 30-40%
57
- sudden drop in hematocrit in pt with hemoglobinopathy - low reticulocyte count
- parvovirus B19 - folate deficiency
58
MOST ACCURATE test for parvovirus B19
PCR for parvovirus DNA
59
treatment for sickle cell crisis d/t parvovirus B19
transfusions and IVIG
60
sickle cell anemia patients should be discharged on:
1. folate 2. Pneumococcal vaccine 3. hydroxyurea, if crises occur \> 4x/year
61
MOA of hydroxyurea
increases % of Hb F
62
sickle cell disease manifestations
- visual disturbance (frequent) - hematuria - isosthenuria (inability to concentrate/dilute urine) - UTIs
63
sickle cell trait manifestations
- hematuria - isosthenuria - splenic vein thrombosis can occur with severe hypoxia
64
look for other autoimmune diseases in history, or medications
autoimmune hemolysis
65
diagnostic tests for autoimmune hemolysis
- elevated LDH - elevated indirect bilirubin - elevated reticulocyte count - decreased haptoglobin
66
peripheral smear will show what in autoimmune hemolysis
spherocytes
67
MOST ACCURATE diagnostic test for autoimmune hemolysis
Coombs test
68
best INITIAL treatment for autoimmune hemolysis
steroids
69
treatment if RECURRENT episodes of hemolysis
rituximab
70
MOST EFFECTIVE treatment if RECURRENT episodes of hemolysis
splenectomy
71
mechanism of spherocytes in autoimmune hemolysis
Abs attack RBC membranes which are biconcave turning them into spheres
72
in autoimmune hemolysis, steroids and splenectomy only work on?
IgG Abs "warm antibodies"
73
if severe hemolysis does not respond to steroids or repeated blood transfusions use
IVIG
74
a response to IVIG predicts a response to
splenectomy
75
cold-induced hemolysis (cold agglutinins); look for what in the history?
- mycoplasma - Epstein-Barr virus
76
in cold-induced hemolysis Coombs test will be?
NEGATIVE
77
in cold-induced hemolysis complement test will be?
POSITIVE
78
treatment for in cold-induced hemolysis
rituximab
79
will steroids, splenectomy, or IVIG work for cold-induced hemolysis?
NO
80
MOA of rituximab
MAB against CD20R on lymphocytes which make Abs
81
syphilis causes cold agglutinins with?
IVIG
82
- sudden onset hemolysis - X-linked - oxidant stress causes acute hemolysis - MC oxidant stress = infection - sulfa, primaquine, dapsone, fava beans
glucose-6-phosphate dehydrogenase (G6PD) deficiency
83
best INITIAL test for glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Heinz body test - bite cells
84
Heinz bodies are
collection of oxidized, precipitated Hb embedded in RBC membrane
85
bite cells are
pieces of RBC membrane removed by spleen
86
MOST ACCURATE test for glucose-6-phosphate dehydrogenase (G6PD) deficiency
G6PD LEVEL, BUT ONLY AFTER 2 MONTHS HAVE PASSED from acute episode (otherwise, may be falsely normal)
87
treatment for glucose-6-phosphate dehydrogenase (G6PD) deficiency
avoid oxidant stress
88
- sudden onset hemolysis - NOT provoked (unclear cause)
pyruvate kinase deficiency
89
- recurrent episodes of hemolysis - splenomegaly - bilirubin gallstones - elevated mean corpuscular Hb concentration (MCHC)
hereditary spherocytosis
90
MOST ACCURATE test for hereditary spherocytosis
osmotic fragility test
91
treatment for hereditary spherocytosis
splenectomy
92
mechanism of hereditary spherocytosis
genetic loss of ankyrin and spectrin in RBC membrane
93
hemolytic uremic syndrome (HUS) diagnosis is based on IT'S IN THE NAME!!!
- intravascular hemolysis (Autoimmune hemolysis) - elevated BUN and creatinine (Renal failure) - thrombocytopenia (Thrombocytopenia)
94
thrombotic thrombocytopenic purpura (TTP) diagnosis is based on
- intravascular hemolysis - elevated BUN and creatinine - thrombocytopenia AND - fever - neurological abnormalities
95
never use what in HUS or TTP?
platelets
96
what is LOW in TTP?
ADAMTS-13 level
97
treatment for HUS/TTP
- some cases resolve on their own - severe cases: plasmapheresis
98
what makes HUS worse?
antibiotics
99
mechanism of HUS/TTP
ADAMTS-13 = metalloproteinase that breaks down VWF to RELEASE platelets from one another if VWF is NOT dissolved, platelets form abnormally long strands and RBCs break down as they pass through
100
- pancytopenia - recurrent episodes of dark urine, especially in morning - MCC of death = large vessel venous thrombosis
paroxysmal nocturnal hemoglobinuria (PNH)
101
paroxysmal nocturnal hemoglobinuria (PNH) can transform into
- aplastic anemia - acute myelogenous leukemia (AML)
102
MOST ACCURATE test for paroxysmal nocturnal hemoglobinuria (PNH)
CD55 and CD59 Abs (aka, decay accelerating factor)
103
best INITIAL treatment for paroxysmal nocturnal hemoglobinuria (PNH)
glucocorticoids
104
treatment for transfusion-dependent pts with severe paroxysmal nocturnal hemoglobinuria (PNH)
eculizumab
105
MOA of eculizumab
inhibits C-5 and prevents complement activation
106
how can HELLP syndrome be distinguished from DIC?
HELLP has NORMAL coagulation studies
107
- SOB for no clear reason - clear lungs on exam - normal CXR
methemoglobinemia
108
methemoglobinemia is
Hb locked in OXIDIZED state that cannot pick up O2
109
possible drugs that can cause methemoglobinemia
- nitroglycerin - amyl nitrate - nitroprusside - dapsone - any anesthetic (ends in -CAINE; lidocaine/bupivacaine/tetracaine)
110
in methemoglobinemia, what color is the blood?
BROWN
111
treatment for methemoglobinemia
methylene blue
112
transfusion reactions: case 1 - 20 minutes after a pt receives a blood transfusion, the pt becomes SOB - there are transient infiltrates on CXR - all symptoms resolve spontaneously
transfusion-related acute lung injury (TRALI), or "leukoagglutination reaction"
113
transfusion reactions: case 2 - as soon as a pt receives a transfusion, he becomes HYPOTENSIVE, SOB, TACHYCARDIC - LDH, and bilirubin levels are NORMAL
IgA deficiency
114
transfusion reactions: case 3 - during a transfusion, a pt becomes hypotensive and tachycardic - has back and chest pain - has dark urine - LDH, and bilirubin are ELEVATED - haptoglobin is LOW
ABO incompatibility
115
transfusion reactions: case 4 - a few days after a transfusion, pt becomes jaundiced - hematocrit does NOT rise with transfusion - generally without symptoms
minor blood group incompatibility
116
transfusion reactions: case 5 - a few hours after a transfusion, pt becomes FEBRILE with a rise in temperature of about 1 degree - no evidence of hemolysis
febrile nonhemolytic reaction
117
- present w/ signs of pancytopenia: 1. fatigue 2. bleeding 3. infections d/t functional immunodeficiency
acute leukemia
118
best INITIAL test for acute leukemia
peripheral smear showing blasts
119
Auer rods are associated with?
acute myeloid leukemia (AML)
120
most important prognostic finding in acute leukemia
cytogenetic abnormalities (specific karyotype abnormalities)
121
if pt is a high risk for relapse of acute leukemia after chemotherapy, pt should have what after chemotherapy induces remission?
bone marrow transplantation
122
best INITIAL treatment for acute leukemia
chemotherapy - idarubicin, or daunorubicin AND - cytosine arabinoside
123
what treatment should be added for acute promyelocytic leukemia (M3 AML) to the initial treatment?
all trans retinoic acid (ATRA)
124
what treatment should be added for acute lymphocytic leukemia (ALL) to the initial treatment?
intrathecal methotrexate
125
M3, acute promyelocytic leukemia is associated with
disseminated intravascular coagulation (DIC)
126
- SOB, confusion, and blurry vision - white cell count \> 100,000
leukostasis = sludging of blood vessels of brain, eyes, and lungs
127
treatment for leukostasis
leukapheresis
128
what should be added in a pt with leukostasis to lower the white cell count?
hydroxyurea
129
- elderly pt w/ pancytopenia - elevated MCV - low reticulocyte count - macroovalocytes - "Pelger-Huet cell" (neutrophil w/ 2 lobes) - NORMAL B12 level - small # of blasts (not enough to be considered acute leukemia)
myelodysplasia
130
mild, slow progressive preleukemia syndrome
myelodysplasia
131
myelodysplasia may progress to
acute leukemia
132
MCC of death in myelodysplasia
infection or bleeding
133
main treatment for myelodysplasia
transfusions as needed
134
only thing that increases survival in myelodysplasia
azacytidine
135
decreases transfusion dependence in myelodysplasia
lenalidomide
136
name the myeloproliferative disorders (6)
1. chronic myelogenous leukemia (CML) 2. chronic lymphocytic leukemia (CLL) 3. hairy cell leukemia 4. myelofibrosis 5. polycythemia vera 6. essential thrombocythemia
137
- elevated white cell count - predominantly NEUTROPHILS - splenomegaly (common)
chronic myelogenous leukemia (CML)
138
of all forms of myeloproliferative disorders, which has the highest risk of transforming into acute leukemia?
untreated chronic myelogenous leukemia (CML)
139
mechanism of early satiety in CML and CLL
d/t splenomegaly compressing stomach
140
diagnostic test for chronic myelogenous leukemia (CML)
elevated neutrophil count with LOW LAP score
141
MOST ACCURATE test for chronic myelogenous leukemia (CML)
Philadelphia chromosome by PCR, or BCR/ABL by fluorescence in situ hybridization (FISH)
142
best INITIAL treatment for chronic myelogenous leukemia (CML)
imatinib
143
curative treatment for chronic myelogenous leukemia (CML)
bone marrow transplantation
144
- pts \> 50 y/o w/ elevated white cell count described as "normal appearing lymphocytes" - often asymptomatic
chronic lymphocytic leukemia (CLL)
145
best INITIAL test for chronic lymphocytic leukemia (CLL)
peripheral blood smear shows "SMUDGE" cells (ruptured nuclei of lymphocytes)
146
stages for chronic lymphocytic leukemia (CLL)
stage 0 = elevated white cell count alone stage 1 = enlarged lymph nodes stage 2 = spleen enlargement stage 3 = anemia stage 4 = low platelets
147
mechanism of infection and hemolysis in chronic lymphocytic leukemia (CLL)
- lymphocytes produce abnormal/insufficient immunoglobulins - insufficient immunoglobulins = infection - abnormal immunoglobulins = thrombocytopenia/hemolyis
148
treatment for chronic lymphocytic leukemia (CLL)
based on disease stage
149
treatment for chronic lymphocytic leukemia (CLL): stages 0 and 1
no treatment needed
150
treatment for chronic lymphocytic leukemia (CLL): stages 2-4
fludarabine, and rituximab and cyclophosphamide
151
which treatment is most likely to extend survival in treatment for chronic lymphocytic leukemia (CLL)?
fludarabine
152
- middle-aged pt (50's) - pancytopenia - massive splenomegaly
hairy cell leukemia
153
MOST ACCURATE test for hairy cell leukemia
smear showing HAIRY cells and immunophenotyping
154
best INITIAL treatment for hairy cell leukemia
cladribine (2-CDA)
155
- pancytopenia - splenomegaly - NORMAL TRAP level
myelofibrosis
156
key feature seen on PBS in myelofibrosis
teardrop-shaped cells
157
finding on bone marrow biopsy in myelofibrosis
fibrosis and JAK2 mutation
158
best INITIAL treatment for myelofibrosis if bone marrow transplant is NOT possible
lenalidomide/thalidomide
159
curative treatment for myelofibrosis
bone marrow transplantation
160
inhibits Janus kinase
ruxolitinib
161
- headache - blurred vision - dizziness - fatigue - pruritis, after hot bath/shower - splenomegaly
polycythemia vera
162
key to diagnosis in polycythemia vera
MARKEDLY HIGH hematocrit in the ABSENCE of hypoxia with a LOW MCV (erythropoietin level will be low)
163
diagnostic tests for polycythemia vera
- CBC - ABG to r/o hypoxia - erythropoietin level (low) - JAK2 mutation (97% sensitive)
164
best INITIAL treatment for polycythemia vera
phlebotomy
165
used to lower the cell count in polycythemia vera
hydroxyurea
166
treatment for thrombocytopenia in polycythemia vera
anagrelide
167
other treatment that should be given for polycythemia vera
aspirin
168
- markedly elevated platelet count - presents w/ headache, visual disturbance, and pain in hands
essential thrombocythemia
169
MCC of death in essential thrombocythemia
thrombosis and bleeding
170
treatment for essential thrombocythemia
hydroxyurea
171
treatment for essential thrombocythemia if pt is thrombosing
aspirin
172
what other lab values are elevated in polycythemia vera?
- B12 - LAP (leukocyte alkaline phosphatase) score
173
name the plasma cell disorders (4)
1. multiple myeloma 2. monoclonal gammopathy of unknown significance (MGUS) 3. Waldenstrom's macrogammaglobulinemia 4. aplastic anemia
174
- bone pain caused by fracture occurring under normal use (most frequent presentation)
multiple myeloma
175
MCC of death from MM
- infection: pts effectively immunodeficient - renal failure
176
initial tests for MM
- skeletal survey - serum protein electrophoresis (SPEP) - urine protein electrophoresis (UPEP) - peripheral smear - elevated Ca++ level - B2 microglobulin level - elevated BUN/creatinine
177
skeletal survey findings in MM
punched out osteolytic lesions (osteoblastic lesions suggest metastatic prostate cancer)
178
serum protein electrophoresis (SPEP) findings in MM
elevated monoclonal Ab (usually IgG) levels (20% are IgA)
179
urine protein electrophoresis (UPEP) findings in MM
Bence-Jones protein
180
peripheral smear findings in MM
"rouleaux" formation of blood cells (mean platelet volume (MPV) is elevated bc cells stick together)
181
cause of elevated Ca++ level in MM
d/t osteoLYTIC lesions
182
prognostic indicator in MM
B2 microglobulin level
183
mechanism of renal failure in MM
- hypercalcemia = nephrocalcinosis - hyperuricemia = directly toxic to kidney tubules - Bence-Jones protein clog up glomeruli and are also toxic to kidney tubules - amyloid occurs in myeloma
184
reverses renal dysfunction in MM
bortezomib
185
single MOST SPECIFIC test in MM
bone marrow biopsy (detects high numbers of plasma cells (10%))
186
treatment for MM
melphalan and steroids
187
most effective treatment for MM
autologous stem cell bone marrow transplantation (pts
188
other treatment considerations for MM: - hypercalcemia - bone fractures - renal failure - anemia - infection
- hydration/diuresis - bisphosphonates - hydration - erythropoietin - vaccinations
189
MGUS presents w/
ASYMPTOMATIC elevation of IgG on SPEP
190
treatment for MGUS
none
191
Waldenstrom's macrogammaglobulinemia presents w/
hyperviscosity from IgM overproduction
192
how does Waldenstrom's macrogammaglobulinemia present?
- blurred vision - confusion - headache - enlarged LN's/splenomegaly
193
best INITIAL test for Waldenstrom's macrogammaglobulinemia
- serum viscosity level (will be markedly increased) AND - SPEP (elevated IgM level)
194
best INITIAL treatment for Waldenstrom's macrogammaglobulinemia, if SYMPTOMATIC
plasmapheresis
195
further treatment for Waldenstrom's macrogammaglobulinemia
- rituximab - fludarabine - chlorambucil
196
hemolytic uremic syndrome (HUS) is associated w/
E. coli 0157:H7
197
- ticlopidine predisposes to this
thrombotic thrombocytopenic purpura (TTP)
198
- pancytopenia w/ no identified etiology
aplastic anemia
199
best possible treatment for aplastic anemia
bone marrow transplantation
200
if bone marrow transplantation is not possible, treatment for aplastic anemia
antithymocyte globulin and cyclosporine
201
- enlarged LNs (most commonly cervical area) - +/- "B" symptoms (fever, weight loss, night sweats)
lymphoma
202
lymphoma: starts at the neck and spreads centrifugally away from center
Hodgkin's lymphoma (HD)
203
lymphoma: widespread disease
non-Hodgkin's lymphoma (NHL)
204
best INITIAL test for HD and NHL
excisional LN biopsy
205
the major difference between HD and NHL, is HD has
Reed-Sternberg cells
206
tests needed for staging HD and NHL
- CXR - CT scans w/ contrast of chest, abdomen, pelvis, and head - bone marrow biopsy
207
staging of HD and NHL
stage 1: single LN group stage 2: 2 LN groups on ONE side of diaphragm stage 3: LNs on BOTH sides of diaphragm stage 4: widespread disease
208
HD presents in what stages?
80-90% in stages 1 or 2
209
NHL presents in what stages?
80-90% in stages 3 or 4
210
lymphoma treatment: localized disease (stages 1 and 2) WITHOUT "B" symptoms
radiation and lower-dose chemotherapy
211
lymphoma treatment: advanced disease (stages 3 and 4)
chemotherapy ONLY
212
chemotherapy treatment for HD
ABVD - Adriamycin (doxorubicin) - bleomycin - vinblastine - dacarbazine
213
chemotherapy treatment for NHL
CHOP - cyclophosphamide - hydroxyadriamycin - Oncovin (vincristine) - prednisone
214
if anti-CD20 Ag is present in NHL, what should be added?
rituximab (adds efficacy to CHOP)
215
name the coagulation disorders (4ish)
1. von Willebrand's disease (VWD) 2. idiopathic thrombocytopenic purpura (ITP) 3. uremia-induced platelet dysfunction 4. clotting factor deficiencies (factors 8, 9, 11, 12)
216
- bleeding from platelet dysfunction (superficial bleeding: skin and mucosal surfaces) - epistaxis - bleeding worse w/ aspirin use - NORMAL platelet count - elevated aPTT in up to 50% of pts
von Willebrand's disease (VWD)
217
MOST ACCURATE tests for von Willebrand's disease (VWD)
- ristocetin cofactor assay - von Willebrand's factor (VWF) level
218
first-line treatment for von Willebrand's disease (VWD)
desmopressin
219
MOA of desmopressin
releases subendothelial stores of VWF and factor 8
220
treatment for von Willebrand's disease (VWD) if desmopressin is ineffective
factor 8 replacement
221
- platelet-type bleeding - platelet count
idiopathic thrombocytopenic purpura (ITP)
222
diagnostic tests for idiopathic thrombocytopenic purpura (ITP)
- peripheral smear - sonogram - bone marrow - Abs to glycoprotein 2b/3a receptor
223
finding of peripheral smear in idiopathic thrombocytopenic purpura (ITP)
large platelets
224
finding of sonogram in idiopathic thrombocytopenic purpura (ITP)
normal spleen size
225
finding of bone marrow biopsy in idiopathic thrombocytopenic purpura (ITP)
increased # of megakaryocytes
226
most important step in idiopathic thrombocytopenic purpura (ITP)
initiating treatment
227
- platelet count of 5,000 - epistaxis and petechiae - intracranial hemorrhage and melena what is the best initial step?
IVIG
228
idiopathic thrombocytopenic purpura (ITP) treatment: platelet count \> 50,000
NO treatment
229
idiopathic thrombocytopenic purpura (ITP) treatment: platelet count
prednisone
230
idiopathic thrombocytopenic purpura (ITP) treatment: platelet count
IVIG aka Rhogam
231
idiopathic thrombocytopenic purpura (ITP) treatment: RECURRENT episodes
splenectomy
232
idiopathic thrombocytopenic purpura (ITP) treatment: no response to splenectomy
- romiplostim - eltrombopag
233
uremia prevents platelets from working properly by
preventing degranulation
234
- renal failure - normal platelet count w/ platelet-type bleeding
uremia-induced platelet dysfunction
235
the ristocetin test and VWF level will be what in uremia-induced platelet dysfunction
NORMAL
236
best INITIAL treatment for uremia-induced platelet dysfunction
desmopressin, dialysis, and estrogen
237
the first test to determine difference between clotting factor deficiency and a factor inhibitor Ab
mixing study (aPTT will correct to normal w/ clotting factor deficiency)
238
- woman present w/ bleeding into her thigh after minor trauma - aPTT is prolonged, and PT is normal - mixing study does NOT correct the aPTT what is the diagnosis?
factor 8 Ab
239
clotting factor deficiencies: joint bleeding or hematoma in a MALE child
factor 8 deficiency
240
treatment for factor 8 deficiency: minor and major (
- desmopressin - factor 8 replacement
241
clotting factor deficiencies: - joint bleeding or hematoma - less common than factor 8 deficiency
factor 9 deficiency
242
treatment for factor 9 deficiency
factor 9 replacement
243
clotting factor deficiencies: rare bleeding w/ trauma or surgery
factor 11 deficiency
244
factor 11 deficiency treatment
fresh frozen plasma w/ bleeding episodes
245
clotting factor deficiencies: no bleeding
factor 12 deficiency
246
treatment for factor 12 deficiency
no treatment needed
247
best INITIAL test for all the clotting factor deficiencies
mixing study
248
test after mixing study for all the clotting factor deficiencies
check the specific factor
249
how does heparin-induced thrombocytopenia present?
drop in platelets of at least 50%
250
when does heparin-induced thrombocytopenia occur?
a few days AFTER start of heparin
251
is HIT exclusive to heparin only?
NO!! any form of heparin; this is an allergic reaction
252
MC clinical manifestation of HIT
THROMBOSIS
253
best INITIAL tests for HIT
- platelet factor 4 Abs - heparin-induced antiplatelet Abs
254
best INITIAL therapy for HIT
- STOP THE HEPARIN - use a direct thrombin inhibitor (argatroban, lepirudin, fondaparinux)
255
thrombophilia/hypercoagulable states: - venous or arterial thrombosis - elevated aPTT w/ normal PT - spontaneous abortion - false positive VDRL
lupus antiphospholipid syndrome - lupus anticoagulant - anticardiolipin Abs
256
best INITIAL test for lupus antiphospholipid syndrome
mixing study
257
MOST ACCURATE test for lupus anticoagulant
Russel viper venom test
258
treatment for lupus antiphospholipid syndrome
heparin followed by warfarin
259
thrombophilia/hypercoagulable states: - skin necrosis w/ warfarin use - venous thrombosis
protein C deficiency
260
test for protein C deficiency
protein C level
261
treatment for protein C deficiency
heparin followed by warfarin
262
thrombophilia/hypercoagulable states: - MCC of thrombophilia - venous thrombosis
factor V Leiden thrombophilia
263
test for factor V Leiden thrombophilia
factor V Leiden mutation test
264
treatment for factor V Leiden thrombophilia
heparin followed by warfarin
265
thrombophilia/hypercoagulable states: - no change in aPTT with IV heparin bolus - venous thrombosis
antithrombin deficiency
266
test for antithrombin deficiency
antithrombin 3 level
267
treatment for antithrombin 3 deficiency
large amounts of heparin or direct thrombin inhibitor followed by warfarin
268
mechanism of factor 5 thrombophilia
mutated factor 5 cannot be inactivated by protein C (factor 5 mutation functions like protein C deficiency)
269
Blood transfusion: Anaphylaxis occurs when?
In the first few seconds to minutes and occurs with kids who get blood tranfusion with IgA deficiency
270
Blood transfusion:Acute hemolytic reaction occurs when?
When patient recieves a tranfusion which is not cross matched and occurs within an hour. you will often find coombs test positive and UA showing hemoglobinuria.
271
Blood transfusion:Febrile nonhemolytic reaction occurs when?
When patient recieves blood that which is not Leukoreduced(WBC removed-which also removes cmv and reduces HLA alloimmunization) these reactions usually occur within 1 to 6 hours of tranfusion.
272
Why do you never use whole blood?
Whole blood contains numerous leukocytes and increase the risk of febrile reactions.
273
What are irradiated RBCS?
They are irradiated for bone marrow transplant recipients, Immuno deficient, or blood products from 1deg or 2nddeg relatives
274
Whats are washed rbcs?
Washed For those with Iga deficiency and prevent autoimmunie hemolytic anemia
275
What are leukoreduced RBCS?
For thos who are chronically transfused, CMV at risk patients, potential transplant patient and previously febrile nonhemolytic reaction in PMH patients
