Hematology Flashcards
1
Q
generalized anemia symptoms
A
fatigue, malaise, loss of energy
2
Q
severe anemia symptoms
A
short of breath, lightheadedness, confusion
3
Q
anemia symptoms
A
pallor
flow murmur (I/VI or II/VI systolic murmur)
pale conjuctiva
4
Q
for anemia, there are no uneque physical findings to specify diagnosis, so what do you do?
A
order CBC
general appearance, CV, chest, extremities, HEENT
5
Q
diagnostic tests for anemia
A
CBC with peripheral smear (paying mind to MCV and MCHC)
reticulocyte count, haptobloin, LDH, total and direct bilirubin, TSH with T4, B12/folate, iron
Urinalysis with microscopic analysis
chest exam (if shortness of breath)
EKG (for severe anemia to exclude eschemia)
6
Q
best initial test for anemia
A
CBC with peripheral smear
7
Q
MCV
A
mean corpuscular volume
may clarify whether anemia is microcytic, macrocytic, or normocytic
8
Q
MCHC
A
mean corpuscular hemoglobin concentration
may reveal whether there is a problem with the synthesis of hemoglobin
hypochromic, hyperchromic, or normochromic
9
Q
what has similar presentations to anemia?
A
hypoxia
carbon monoxide poisoning
methemoglobinemia
ischemic heart disease
10
Q
microcytic anemia with: blood loss, elevated platelet count - diagnosis
A
iron deficiency
11
Q
microcytic anemia with: blood loss, elevated platelet count - best initial diagnostic test?
A
iron studies:
- low ferritin
- high TIBC
- low Fe
- low Fe sat
- elevated RDW
12
Q
microcytic anemia with: blood loss, elevated platelet count - most accurate diagnostic test?
A
bone marrow biopsy - but do not do
13
Q
microcytic anemia with: blood loss, elevated platelet count - best initial therapy?
A
prescribe ferrous sulfate orally
14
Q
microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - diagnosis
A
anemia of chronic disease
15
Q
microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - best initial diagnostic test?
A
iron studies:
- high ferritin
- low TIBC
- low Fe
- normal or low Fe sat
16
Q
microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - most accurate diagnostic test?
A
no specific diagnostic test
17
Q
microcytic anemia with: rheumatoid arthritis, ESRD, or any chronic infectious, inflammatory, or connective tissue disease - best initial therapy?
A
correct underlying disease
EPO only with renal failure
18
Q
microcytic anemia with: very small MCV with few or no symptoms, target cells - diagnosis?
A
thalassemia
19
Q
microcytic anemia with: very small MCV with few or no symptoms, target cells - best initial diagnostic test?
A
iron studies:
- normal
20
Q
microcytic anemia with: very small MCV with few or no symptoms, target cells - most accurate diagnostic test?
A
hemoglobin
electrophoresis
beta: elevated HgA2, HgF
alpha: normal
21
Q
microcytic anemia with: very small MCV with few or no symptoms, target cells - what is the best initial therapy?
A
no treatment for trait
22
Q
microcytic anemia: alcoholic isoniazid, lead exposure - diagnosis?
A
sideroblastic anemia
23
Q
microcytic anemia: alcoholic isoniazid, lead exposure - best initial diagnostic test?
A
iron studies:
- high Fe
24
Q
microcytic anemia: alcoholic isoniazid, lead exposure - most accurate diagnostic test?
A
prussian blue stain of marrow (shows ringed sideroblasts)
25
microcytic anemia: alcoholic isoniazid, lead exposure - best initial therapy?
major: remove the toxin exposure
minor: prescribe pyridoxine replacement
26
most accurate diagnosis of alpha thalassemia
DNA sequencing
27
diagnostic tests for anemia:
iron studies/profile (Fe, Fe sat, ferritin, TIBC)
| bone marrow biopsy
28
macrocytic anemia causes
B12 or folate deficiency
29
B12 deficiency findings:
```
peripheral neuropathy
least common: dementia
resolve with treatment if present for a short time
glossitis
diarrhea
```
30
Folate deficiency findings
no neurologic problems
31
diagnostic testing of macrocytic anemia
```
CBC with peripheral blood smear (hypersegmented neutrophils and oval cells)
bilirubin, LDH (commonly elevated)
decreased reticulocyte
oval cells on peripheral smear
B12, folate (most accurate)
```
32
what is low in B12 deficiency?
reticulocytes
33
what to do if you suspect B12 deficiency, but B12 is normal?
order a methylmalonic acid level
| homocysteine levels go up in both vitamin B12 deficiency and folate deficiency
34
next best test after finding a low B12 or elevated methylmalonic acid?
confirm etiology with antiparietal cell and anti-intrinsic factor antibodies
- confirm pernicious anemia as etiology (allergy to parietal cells)
35
Schilling test
older way to confirm etiology
36
why does B12 deficiency raise LDH and indirect bilirubin?
destroying red blood cells early as they come out of the bone marrow
"ineffective erythropoiesis" and is why retic count is low
37
what happens after B12 replacement therapy?
reticulocytes will improve first (in iron deficiency)
| neurological abnormalities will improve last
38
presentations of hemolytic anemia
sudden onset of weakness and fatigue associated with anemia
39
hemolysis shows the following in labs:
```
elevated indirect bilirubin level
elevated reticulocyte count
elevated LDH
decreased haptoglobin level
spherocytes on smear
```
40
what else gives spherocytes?
autoimmune hemolysis
41
mechanism of lab abnormalities in hemolysis
when cells are destroyed, they release indirect bilirubin. the liver has limited capacity to glucuronidate it into direct (water-soluble) bilirubin. indirect bili never gets into the urine because it's attached to albumin and cannot be filtered. haptoglobin is a transport protein for newly released indirect bili and is rapidly used up during hemolysis. LDH increases from any form of tissue breakdown and is extremely nonspecific
42
what to order in hemolysis
peripheral smear, LDH, bilirubin level, reticulocyte count, haptoglobin level
43
what to watch out for when treating B12 deficiency?
low potassium
44
what do you see in intravascular hemolysis?
abnormal peripheral smear (schistocytes, helmet cells, fragmented cells)
hemoglobinuria
45
sickle cell presentation
acute severe pain in the chest, back, and thighs
46
diagnostic tests for sickle cell anemia
peripheral smear
LDH, indirect bili, reticulocytes: elevated
hemoglobin electrophoresis
47
best initial test for sickle cell
peripheral smear - showing sickles
48
most accurate test for sickle cell
hemoglobin electrophoresis
49
initial management of sickle cell:
oxygen, fluids, analgesics, and antibiotics
| complete physical exam
50
physical exam findings in sickle cell
HEENT: retinal infarction
CV: flow murmur from anemia
abdomen: splenomegaly in children; absence of spleen in adults
chest: rales or consolidation from infection or infarction
extremities: skin ulcers (unclear etiology), aseptic necrosis of hip (found on MRI)
neurological: stroke, current or previous
51
best next step for sickle
oxygen, hydration with normal saline continuously and pain medication
if fever, give ceftriaxone, levofloxacin, or moxifloxacin with the first screen (more important than waiting for results of testing)
52
answer for a sickler with a fever
blood cultures, urinalysis, reticulocyte count, CBC, and chest x-ray along with oxygen, hydration, IVF, and antibiotics
53
when is the answer 'exchange transfusion' in sickle cell disease?
eye: visual disturbance from retinal infarction
lung: pulmonary infarction leading to pleuritic pain, pulmonary, hypertension, and abnormal x-ray
penis: priapism from infarction of prostatic plexus of veins if local drainage does not work
brain: stroke
54
most common cause of osteomyelitis in sickle cell disease
salmonella
| * requires a biopsy
55
if a patient is on folate replacement therapy, what is the diagnosis?
parvovirus B19
| - invades the marrow and stops production of cells at the level of the pronormoblast
56
most accurate diagnostic test for parvo
PCR for DnA
| - more accurate than IgM or IgG
57
treatment of parvo
transfusions and IV immunoglobulins
58
further management of parvovirus
folate replacement
vaccinations: pneumococcal (13-PV and 23-PV), hemophilus influenzae, meningococcal
hydroxyurea to prevent further crisis (if >3 per year)
voxelotor or crizanlizumab to prevent pain crises when hydroxyurea is not working
59
what is voxelotor
HgS polymerization inhibitor
| increases oxygen-carrying capacity
60
what is crizanlizumab
inhibits P-selectin inhibitor
| controls platelet aggregation with RBCs
61
mechanism of hydroxyurea in sickle cell disease
increases the percentage of hemoglobin that is hemlogobin F, or fetal hemoglobin
increased fetal hemoglobin dilutes the sickle hemoglobin and decreases the frequency of painful crises
62
hemoglobin sickle cell disease
mild version of sickle cell disease with fewer crises
visual disturbances are frequent
no painful crises
renal problems are the only significant manifestation - hematuria, isosthenuria (inability to concentrate or dilute the urine), and UTIs
no specific tx of hemoglobin SC disease
63
autoimmune hemolysis
clues:
look for SLE or rheumatoid arthritis
CLL, lymphoma, or medications (PCNs, alpha-methyldopa, quinine, or sulfa drugs)
64
diagnostic testing of autoimmune hemolysis
LDH, indirect bilirubin level, and reticulocyte count (all elevated)
haptoglobin level can be decreased in both intravascular and extravascular
peripheral smear - spherocytes
Coombs test
65
most accurate test of autoimmune hemolysis
Coombs test
66
treatment of autoimmune hemolysis
steroids (prednisone)
if ineffective: splenectomy
rituximab works on IgG and IgM
67
warm antibodies
IgG
| only IgG antibodies respond to steroids and splenectomy
68
cold-induced hemolysis (cold agglutinins)
```
mycoplasma or EBV in hx
standard Coombs is negative
complement test is positive
treatment: rituximab
if fails, try bendamustine
```
69
mechanism of spherocytes in autoimmune hemolysis
normal RBC is biconcave. when antibodies attack the RBC membrane, they pull pieces out of it, which decreases the surface area and turns the RBC into a sphere
it takes more surface area to maintain biconcave disc than a sphere
70
rituximab
antibody against CD20 receptor on lymphocytes
CD20+ make antibodies
improves disease (and rheumatoid arthritis) by removing antibody-producing cells
71
G6PD deficiency
sudden onset of severe hemolysis
| X-linked
72
most common form of oxidative stress in G6PD
infection
73
causes of oxidative stress in G6PD
```
oxidizing drugs (sulfa, primaquine, dapsone)
fava bean ingestion
```
74
best initial diagnostic test for suspected G6PD
Heinz body test - bite cells
75
most accurate test for G6PD
G6PD level - but only after 2 months have passed
76
Heinz bodies and bite cells
collections of oxidized, precipitated hemoglobin embedded in the RBC membrane
bite cells appear when pieces of RBC membrane have been removed by spleen
77
tx of G6PD
no specific treatment
| avoid triggers
78
pyruvate kinase deficiency
presents same as G6PD in terms of hemolysis
not provoked by meds or fava beans
unsure the provocation
79
hereditary spherocytosis presentation
recurrent episodes of hemolysis
splenomegaly
bilirubin gallstones
elevated MCHC
80
all with chronic hemolysis (sickle cell, spherocytosis) need:
lifelong folate replacement
81
most accurate diagnostic test for suspected hereditary spherocytosis
eosin-5-maleimide (EMA)
| more accurate than osmotic fragility
82
treatment for hereditary spherocytosis
splenectomy
- prevents hemolysis since cells are destroed in spleen
give folate
83
hereditary spherocytosis genetics
loss of both ankyrin and spectrin in RBC membrane, which are the basis of the cytoskeleton that maintains the RBC membrane in its biconcave disc
84
HUS cause
E.coli 0157:H7 in history
85
TTP cause
medication use, such as ticlopidine
86
diagnosis of HUS based on
intravascular hemolysis with abnormal smear
elevated BUN and creatinine
thrombocytopenia
87
diagnosis of TTP based on
```
intravascular hemolysis with abnormal smear
elevated BUN and creatinine
thrombocytopenia
fever
neurological abnormalities
```
88
what to never use in HUS/TTP
platelets
89
what test should be ordered in HUS/TTP?
ADAMTS-13 level - decreased in TTP
90
PT/aPTT in HUS and TTP
normal
91
treatment for HUS/TTP
plasmapheresis for severe cases
HUS w/o infection = eculizumab (antibody against complement C5)
caplicizumab (ab against VWF acts like ADAMTS-13 and removes VWF and stops platelet aggregation)
92
what not to use in tx of HUS
antibiotics
| platelet transfusion
93
what should you do before starting eculizumab?
vaccinate for meningococcus
| - complement deficiency predisposes to meningococcal infection
94
mechanism of HUS/TTP
ADAMTS-13 is a metalloproteinase that breaks down von Willebrand factor (VWF) to release platelets from one another. When VWF is not dissolved, the platelets form abnormaly prolonged strands that serve as a barrier to RBCs, which run into the strands and break down/are destroyed. plasmapheresis in tx of severe TTP is to replace ADAMTS-13
giving platelets increases the size of the abnormal platelet strands
95
paroxysmal nocturnal hemoglobinuria
presents with pancytopenia and recurrent episodes of dark urine, particularly in the morning
96
most common cause of death in paroxysmal nocturnal hemoglobinuria
large vessel venous thrombosis, such as portal vein thrombosis
97
most accurate diagnostic test of paroxysmal nocturnal hemoglobinuria
CD 55 and CD 59 antibody (decay accelerating factor)
98
treatment of paroxysmal nocturnal hemoglobinuria
glucocorticoids
| eculizumab for transfusion-dependent patients with severe illness
99
unprovoked portal or hepatic vein (budd-chiari) thrombosis?
look for JAK2 mutation and CD55/59
100
long term consequence of paroxysmal nocturnal hemoglobinuria
aplastic anemia or acute myelogenous leukemia (AML)
101
methemoglobinemia
occurs when the blood is locked in an oxidized state and cannot pick up oxygen
102
methemoglobinemia symptoms
shortness of breath for no clear reason, with clear lungs on exam and a normal chest xray
look for brown blood
103
what to look for when suspecting methemoglobinemia
drugs: nitroglycerin, amyl nitrate, nitroprusside, dapsone, or any anesthetic drugs that end in -caine
104
treatment of methemoglobinemia
methylene blue
105
twenty minutes after a patient receives a blood transfusion, the patient becomes short of breath. there are transient infiltrates on the chest x-ray. all symptoms resolve spontaneously
TRALI - presents with acute SOB from antibodies in the donor blood against recipient white cells
no treatment
106
as soon as a patient receives a transfusion, he becomes hypotensive, short of breath, and tachycardic. LDH and bilirubin levels are normal
IgA deficiency - presents with anaphylaxis
| in the future, use blood donations from an IgA-deficient donor or washed red cells
107
during a transfusion, a patient becomes hypotensive and tachycardic. she has back and chest pain and there is dark urine. LDH and bilirubin are elevated and the haptoglobin level is low
ABO incompatibility
108
a few days after a transfusion, a patient becomes jaundiced. the hematocrit does not rise with transfusion, and he is generally without symptoms
minor blood group incompatibility to Kell, Duffy, Lews, or Kidd antigens or Rh incompatibility presents with delayed jaundice
109
a few hours after a transfusion, a patient becomes febrile with a rise in temperature of about 1 degree. there is no evidence of hemolysis
febrile nonhemolytic reaction
| reactions against donor white cell antigens
110
acute leukemia symptoms
signs of pancytopenia: fatigue, bleeding and infections from white cells that don't work
have functional immunodeficiency
111
what would you see on bone marrow biopsy for AML
auer rods
112
best initial test if suspecting leukemia
peripheral smear showing blasts
113
treatment for acute myelogenous leukemia
chemotherapy with idarubicin and cytosine arabinoside (best initial therapy)
114
treatment for acute promyelocytic leukemia (M3 leukemia)
add all trans retinoic acid to idarubicin and cytosine arabinoside
arsenic trioxide is extremely effective when combined with ATRA
115
treatment for acute lymphocytic leukemia
add intrathecal methotrexate
116
most important prognostic finding in acute leukemia
cytogenetic abnormalities, such as specific karyotype
117
what is associated with acute promyelocytic leukemia
DIC
118
treatment of leukostasis
leukapheresis and hydroxyurea
119
elderly patient with pancytopenia, elevated MCV, low reticulocyte count, and macroovalocytes and normal B12, <20% blasts
myelodysplasia
120
pelger-huet cell
neutrophil with two lobes
121
myelodysplasia
mild, slowly progressive preleukemia syndrome
may progress to acute leukemia
most common cause of death is infection or bleeding
122
treatment of myelodysplasia
largely supportive
transfusions as needed
specific therapies: azacitidine (increases survival), decitabine, lenalidomide (for those with 5q minus syndrome)
luspatercept (erythroid maturation stimulant): helps RBCs grow, reducing the frequency of transfusions
123
chronic myelogenous leukemia (CML)
elevated white count that is predominantly neutrophils
splenomegaly
early satiety
124
mechanism of early satiety in CML and CLL
spleen anatomically right on top of the stomach and compresses it, which makes a person feel full right after eating
125
diagnostic testing for CML
elevated neutrophil count with low leukocyte alkaline phosphatase (LAP) score
- rx high wbc from infection give elevated LAP score
- LAP is up in normal cells, not CML
philadelphia chromosome by PCR of blood or BCR/ABL by FISH (most accurate test)
126
most accurate test for CML
philadelphia chromosome by PCR of blood or BCR/ABL by FISH
127
treatment for CML
imatinib
which leads to 90% hematologic remissionwith no major adverse effects
if not effective, try dasatinib and nilotinib
128
wrong answers for CML
interferon -> much less effective, causes uncomfortable, flu-like symptoms
hydroxyurea -> never makes the philadelphia chromosome negative
busulfan -> never right for anything, unless the exam asks what causes pulmonary fibrosis
129
when would you use interferon in CML?
in pregnant patients
130
what myeloproliferative disorder has the highest risk of transformation into acute leukemia?
CML
131
chronic leukocytic leukemia (CLL)
exclusively age >50 with elevated white count that is described as "normal appearing lymphocytes"
- often asymptomatic
132
diagnostic testing for CLL
peripheral blood smear: smudge cells (ruptured nuclei of lymphocytes) - best initial diagnostic test
133
best initial diagnostic test for suspected CLL
peripheral blood smear
134
stages of CLL
```
stage 0: elevated white cell count alone
stage 1: enlarged lymph nodes
stage 2: enlarged spleen
stage 3: anemia
stage 4: low platelets
```
135
mechanism of infection and hemolysis in CLL
the lymphocytes in CLL produce abnormal or insufficient immunoglobulins
- when IgG produced is abnormal, it is inappropriately directed against RBCs or platelets, causing immune thrombocytopenia or hemolysis
- when IgG supply is insufficient, it leads to infection
136
treatment for CLL
based on stage of the disease
137
treatment for early stages CLL
stage 0 and stage 1
| no therapy required
138
treatment for more advanced stages of CLL
fludarabine + rituximab (an antibody against CD20) to extend survival
chlorambucil is less effective
139
first line therpay for CLL
there is no clear first line therapy
140
when is venetoclax the answer for treatment of CLL?
when there is CLL that fails initial therapy and there is a 17p deletion
141
venetoclax
increases apoptosis in CLL when there is a 17p deletion
142
hairy cell leukemia
pancytopenia
massive splenomegaly
middle-aged patient (50s)
143
hairy cell leukemia peripheral smear
hairy cells and immunophenotyping (or flow cytometry)
| most accurate test
144
treatment of hairy cell leukemia
cladiribine (2-CDA)
145
myelofibrosis
presents with pancytopenia, massive splenomegaly in a middle-aged patient but a normal TRAP level
146
myelofibrosis peripheral smear
teardrop shaped cells
147
myelofibrosis bone marrow biopsy
fibrosis
148
myelofibrosis genetics
JAK2 mutation
149
myelofibrosis treatment
bone marrow transplantation can be curative
| if not possible, ruxolitinib or fedratinib (inhibits JAK2)
150
polycythemia vera (Pvera)
elevated RBCs
headache, blurred vision, dizziness, fatigue
pruritus, often after hot bath or shower due to release of histamine from basophils
splenomegaly
151
what to order in Pvera
B12, LAP and a CBC
152
most accurate diagnostic test in pvera
CBC
| - markedly high hct in the absence of hypoxia with a low MCV
153
lab findings in Pvera
```
low erythropoietin
poss elevated white cell and plt
elev B12 and LAP
high hct -> thrombosis (order ABG to exclude hypoxia as cause)
JAK2 mutation
```
154
treatment for Pvera
phlebotomy
hydroxyuria - lower cell count
daily aspirin
anagrelide or ruxolitinib if hydroxyurea fails
155
essential thrombocytopenia
```
found in asymp pt on CBC
markedly elevated plt
headache, visual disturbace, pain in the hands (erythromyalgia)
thrombosis and bleeding
CALR mutation
```
156
treatment of essential thrombocytopenia
hydroxyurea to lower plt count
| daily aspirin
157
multiple myeloma presentation
bone pain caused by a fracture occurring under normal use
158
causes of death in MM
most common are infection
- immunodeficient
and renal failure
159
initial diagnostic testing for suspected MM
skeletal survey - punched out or osteolytic lesions are suggestive of metastatic prostate
serum protein electrophoresis (SPEP) - elevated monoclona antibodies (usually IgG)
urine protein electrophoresis (UPEP) - Bence-Jones protein
peripheral smear - rouleaux formation of blood cells, mpv elevated
calcium - elevated (osteolytic lesions)
beta 2 microglobulin level - prognostic indicator
BUN and Cr - detect the frequent occurrence of renal insufficiency; bortezomib reverses renal dysfunction
160
other testing for MM
serum free light chain (FLC) ratio 100:1 highly consistent
| low AG - IgG is cation and raises the chloride level, narrowing the gap
161
mechanism of renal failure in myeloma
hypercalcemia -> nephrocalcinosis
hyperuricemia -> directly toxic to kidney tubules
Bence-Jones protein clogs up glomeruli and is toxic to kidney tubules
amyloid occurs in myeloma
162
most specific test in MM
bone marrow biopsy
| high number of plasma cells
163
treatment for MM
no single clear treatment
melphalan and steroids
consider adding thalidomide, lenalidomide, bortezomib, or daratumumab
- give ppx against clotting when using these agents
- bort high risk neuro complications
- darat can be used in adults unable to receive other therapies
164
most effective therapy in MM
autologous stem cell bone marrow trnasplantation
| - reserved for young patients (<70) with advanced disease
165
things to remember to treat with MM
```
hypercalcemia (hydration)
bone fractures (bisphosphonates)
renal failure (hydration)
anemia (erythropoietin)
ppx against infections (pneumovax, flu)
```
166
smolding myeloma
10-60% of bone marrow is plasma cells
high M-spike on serum protein electrophoresis (SPEP)
urine monoclonal protein level is elevated
FLC ratio is increased
no CRAB organ damage (no hypercalcemia, renal failure, anemia, or bone lesions)
167
tx smolding myeloma
no specific therapy, close follow up
168
monoclonal gammopathy of unknown significants (MGUS)
```
asymptomatic elevation of IgG on SPEP
elevated total protein, >70
peripheral neuropathy
10% plasma cells
no treatment
```
169
waldenstrom macroglobulinemia
hyperviscosity from IgM overproduction
blurry vision, confusion, and headache
enlarged nodes and spleen
170
best initial test if suspecting waldenstrom macrogloblinemia
serum viscosity level
- markedly increased
SPEP
- elevated IgM
171
treatment for waldenstrom macroglobulinemia
plasmapheresis if symptomatic
| for further treatment, same as CLL: fludarabine, chlorambucil, or rituximab
172
aplastic anemia
pancytopenia with no identified etiology
173
best treatment for aplastic anemia
```
if young (<50) with a match: BMT
if >50 and no match: use antithymocyte globulin and cyclosporine
```
174
causes of aplastic anemia
most are idiopathic
| chronic Hep B and C can cause it
175
lymphoma presentation
enlarged lymph nodes, most commonly cervical
176
hodgkin disease
spreads centrifugally away from the center, starting at the neck
177
non-hodgkin lymphoma
presents as widespread disease
178
B symptoms in lymphoma
fever
weight loss
night sweats
179
major difference between hodgkin's and non-hodgkin's
HD has reed-sternberg cells
180
best initial test for HD and NHL
excisional lymph node biopsy
181
after biopsy of lymph nodes, what's next?
staging
stage 1: single lymph node group
stage 2: two lymph node groups on one side of the diaphragm
stage 3: lymph node involvement on both sides of the diaphragm
stage 4: widespread disease
182
when does HD usually present?
80-90% present with stages I and II
183
when does NHL usually present?
80-90% present with stages III and IV
184
how to stage lymphoma?
chest x-ray, CT scan with contrast (chest, abdomen, pelvis, head), and bone marrow biopsy
185
wrong answers for diagnostic tests in lymphoma:
```
needle biopsy (useful for infections, but not sufficient for lymphoma)
lymphangiogram or ex lap of abdomen
```
186
treatment of lymphoma
```
localized disease (stage I and II) without B symptoms: radiation and low-dose chemotherapy
more advanced stages (stage III and IV): chemotherapy exclusively
```
187
chemo for HD
adriamycin (doxorubicin), bleomycin, vinblastine, davarbacine (ABVD)
188
treatment of chemo-induced nausea
```
5HT inhibitors (ondansetron, granisetron, palonosetron, dolasetron) = best initial
glucocorticoids: dexamethasone
neurokinin-1R antagonists: aprepitant, rolapitant, netupitant) - use if QT prolongation
```
189
VWD presentatin
epistaxis and/or petechiae
| superficial bleeding from the skin and mucosal surfaces
190
VWD findings
normal plt count
| elevated aPTT because VWF destabilizes factor VIII
191
most accurate test in suspected VWD
ristocetin cofactor assay and VWF level
192
treatment of VWD
desmopressin or DDAVP is first line - releases subendeothelial stores of VWF and factor VIII, which will stop the bleeding
if not effective, use factor VIII replacement
if both ineffective, use recombinant VWF
193
mechanism of ristocetin testing
acts as an artificial endothelial lining
if VWF present, platelets will stick to it
ristocetin is a functional test of VWF activity
194
ITP
platelet bleeding with plt <10k-30k
195
platelet-type bleeding
```
petechiae
epistaxis
purpura
gingiva
vaginal
```
196
factor-type bleeding
hemarthrosis
| hematoma
197
diagnostic testing of ITP
peripheral smear - large platelets
sonogram - normal spleen size
bone marrow - increased megakaryocytes
198
treatment for ITP if plt >50k
no treatment
199
treatment for ITP if plt <50k with minor bleeding
prednisone
200
treatment for ITP if plt <10-20k with serious bleeding
IVIG or RhoGAM
201
treatment for ITP if recurrent episodes
splenectomy
| rituximab
202
treatment for ITP if no response to splenectomy
avatrombopag, romiplostim, eltrombopag
203
what doesn't help ITP?
plasmapheresis because antibodies are already stuck to the platelets
204
uremia-induced platelet dysfunction
uremia by itself prevents platelets from working properly, they do not granulate
205
findings in uremia-induced platelet dysfunction
normal platelet count with platelet-type bleeding in a patient with renal failure
ristocetin test and VWF level will be normal
206
treatment for uremia-induced platelet dysfunction
ddavp, dialysis, estrogen
207
glanzmann thrombasthenia and bernard soulier
platelet-bleeding despite normal plt count and normal VWF
| diagnosed by platelet studies
208
platelet study in glanzmann thrombasthenia
like being on abciximab personality
209
platelet study in bernard-soulier
giant platelets
210
treatment of glanzmann thrombasthenia and bernard soulier
ddavp - releases subendothelial VWF and factor VIIIa
tranexamic and epsilon amino caproic acid inhibit fibrinolysis and plasminogen - acute bleeding
recombinant factor VIIIa
estrogen upregulates VWF
211
clotting factor deficiencies
factor VIII
factor IX
factor XI
factor XII
212
joint bleeding or hematoma in a male child
factor VIII
213
diagnostic test in factor VIII
mixing study firsts, then specific factor level
214
treatment of factor VIII
severe deficiency: factor VIII replacement
| minor replacement: DDAVP
215
joint bleeding or hematoma
| less common
factor IX
216
diagnostic test for Factor IX
mixing study first, then specific factor level
217
treatment of factor IX
factor ix replacement
218
rare bleeding with trauma or surgery
factor XI
219
diagnostic test of factor XI
mixing study first, then specific factor level
220
treatment of factor XI
FFP with bleeding episodes
221
no bleeding
factor XII
222
diagnostic test for factor XII
mixing study first, then specific factor level
223
treatment for factor XII
no treatment
224
factor VIII lab findings
prolonged aPTT and bleeding
225
treatment of factor VIII
recombinant factor VIII
| ddavp
226
what is in prothrombin complex concentrate
reverses warfarin
contains vitamin-k dependent factors: factors II, VII, XI, and X and protein C and S
works faster than FFP and vitK
227
heparin-induced thrombocytopenia
reduced platelets manifesting a few days after the start of heparin
228
most common presentation of HIT
thrombosis - venous thromboses 3x more common than arterial thromboses
229
best initial diagnostic test in HIT
platelet factor 4 antibodies or heparin-induced, antiplatelet antibodies
230
most accurate test in HIT
serotonin release assay
231
treatment of HIT
stop heparin
use fondaparinux or a DOAC
if fonda not avail, choose bivalirudin or argatroban
if both, choose fonda
232
venous or arterial thrombosis
elevated aPTT and normal PT
spontaneous abortion
false positive VDRL
antiphospholipid syndromes
233
diagnostic test for antiphospholipid syndromes
mixing study first
| russel viper venom test is most accurate for lupus anticoagulant
234
treatment of antiphospholipid syndromes
heparin followed by warfarin
| INR target 2-3, lifelong
235
skin necrosis with the use of warfarin
| venous thrombosis
protein C deficiency
236
diagnostic test in protein c deficiency
protein c level
237
treatment for protein c deficiency
DOAC
238
most common cause of thrombosis
| venous thrombosis
factor V leiden mutation
239
diagnostic test for factor V leiden
factor V mutation test
240
treatemnt for factor V leiden
DOAC
241
no change in aPTT with a bolus of IV heparin
| venous thrombosis
antithrombin deficiency
242
diagnostic test for antithrombin deficiency
level of antithrombin III
243
treatment of antithrombin deficiency
DOAC or warfarin, lifelong therapy may be required
