Heme Onc Flashcards
1
Q
you suspect an old guy has folate deficiency and anemia from his tea and toast diet, but your workup will proceed as follows
A
CBC with MCV then if indications persist as workup continues: peripheral smear b12 and folate methyl malonic acid
2
Q
a mediterranean man takes primaquine for malaria prophylaxis and develops a jaundiced anemia… sounds like…
A
G6PD deficiency or
Pyruvate Kinase deficiency
(hemolysis)
3
Q
MCV
bili
LDH
haptoglobin
in hemolysis
A
MCV normal
Bili elevated
LDH elevated
haptoglobin low
in hemolysis
4
Q
peripheral smear in folate or b12 defiiency
A
Hypersegmented Neutrophils with Macrocytic Red Blood Cells
impaired DNA synthesis causes delayed division and hypermaturation in other ways…
5
Q
homeless guy with macrocytic anemia and burning feet with poor hygeine probably from what and not what
A
probably from Folate deficiency (depleted in 1 month) and Tinea
NOT B12 deficiency and Subacute Combined Degeneration of the Cord which takes longer to develop
6
Q
thrombocytopenia with anemia in a homeless guy probably a product of…
A
alcoholic liver disease and maybe early cirrhosis
-decreased thrombopoetin and increased sequestration in liver…. macrocytic nonmegaloblastic anemia…
7
Q
when megaloblastic anemia with equivocal Folate and B12, next step…
A
Methyl Malonic Acid level
-elevated in B12 deficiency, normal in folate deficiency
8
Q
deferoxamine
use
moa
A
for iron overload
(e.g. sickle cell pt with transfusions)
binds trivalent ions Ferric (Fe3-) for excretion in urine
9
Q
beta thalassemia peripheral smear
A
small target cells
10
Q
how does subacute combined degeneration of the spinal cord from b12 deficiency present
A
lower extremity neurological symptoms - motor (weakness and hyperreflexia, upgoing babinski), DCML (proprioception vibratory position sense)
(ALS is preserved)
dementia
11
Q
how to get B12 deficiency, 3 ways
A
Crohn’s (terminal ileum inflammation, no B12 absorption)
Pernicious Anemia (antibodies against parietal cells destroy so no intrinsic factor or antibodies against intrinsic factor)
Strict Avoidance of All Anmial Products
12
Q
weakness of large muscles
but improves with use
no effect on sensation
from autoimmune attack against presynaptic calcium channels
diagnosis
A
Lambert-Eaton Syndrome
13
Q
alcoholic with good nutrition has Hb 10 and MCV 120, what do you see on peripheral smear?
A
NON-Megaloblastic anemia
-Large red blood cells only, no hypersegmented neutrophils
(good nutrition, not folate or b12 deficiency)
14
Q
B12 aka
A
cobalamin
15
Q
how does gastrectomy predispose to b12 deficiency
treat
A
no Parietal cells, no IF intrinsic factor, no B12 absorption
treat with Intramuscular B12 Injections
16
Q
what aspects of subacute combined degeneration of the spinal cord from b12 deficiency are reversible vs non-reversible
A
dementia is partially reversible
spinal cord symptoms are irreversible… motor (weakness and hyperreflexia, upgoing babinski), DCML (proprioception vibratory position sense)
17
Q
Anemia
Low MCV
Normal iron studies
Normal Hb electrophoresis
Dx
A
Alpha Thalassemia
beta thal had abnormal electrophoresis
18
Q
Anemia
mcv, reticulocute count, ldh normal
Elevated Ferritin low tibc low iron
Dx
A
Anemia of Chronic Inflammatory Disease
19
Q
symptomatic (microcytic?) anemia without improvement despite normal or elevated iron studies
suspect
pathogenesis
what biopsy
A
suspect Sideroblastic Anemia
problem of Mitochondria
Ringed Sideroblasts on Bone Marrow Biopsy
20
Q
Hb 10
Small cells on peripheral smear
Next test
Ddx
A
FERRITIN differentiates microcytic anemias- Low - iron deficiency anemia High - anemia of chronic disease Normal - thalassemia
(serum iron level more variable less reliable, low in both ida and acd)
21
Q
other than folate and b12 deficiencies, what else can cause macrocytic anemia
A
Alcohol
Liver Disease
22
Q
if you see an Elevated Iron in a Microcytic (low MCV) Production Anemia (low Retic) the next step is ____
you suspect ____
A
next step Bone Marrow Biopsy
suspect Sideroblastic Anemia
congenital Or part of an infiltrative malignancy… myrlidysplastic syndrome…
23
Q
Hb cutoff for EPO for anemia caused by renal disease
A
Hb v10 caused by renal disease give EPO
24
Q
Two times you should go for bone marrow biopsy for Anemia
A
serum Iron is High (sideroblastic anemia)
Pancytopenia (infiltrative)
25
etiology of hereditary spherocytosis
diagnose
treat
defective RBC CELL MEMBRANE PROTEIN
-Spectrin, Ankyrin, Band 4.1
diagnose with smear, osmotic fragility test
treat with Folate supplementation most importantly.... splenectomy if refractory and asymptomatic (still spherocytes but less hemolysis)
26
```
TF
TMP SMX (bactrim) is a sulfa antibiotic that can reveal G6PD deficiency
```
T
| induces oxidative stress
27
pathogenesis of paroxysmal nocturnal hemoglobinuria
deficiency in PIG-A gene
| ...GDI anchor fails and RBCs become succeptible to Complement Fixation
28
24yo with paroxysms of dark urine in the morning with hemosiderin rather than blood, abdominal pain,
diagnosis
course
pathogenesis
diagnose
Paroxysmal Nocturnal Hemoglobinuria
course indolent and not a bother till infection exacerbates
caused by PIG-A GENE DEFICIENCY, GDI anchor fails and RBCs become succeptible to COMPLEMENT FIXATION
FLOW CYTOMETRY to diagnose -- CD55 and CD59 deficiencies
29
other than folate and b12 deficiencies, what else can cause macrocytic anemia
Alcohol
| Liver Disease
30
transfusion, non-cardiogenic pulmonary edema, and fever is ___
TRALI
| transfusion-related lung injury
31
treat acute chest syndrome in sickle cell crisis
exchange transfusion
32
workup for hemolysis
Smear
Electrophoresis
Coombs test
33
etiology of hereditary spherocytosis
diagnose
treat
defective RBC CELL MEMBRANE PROTEIN
-Spectrin, Ankyrin, Band 4.1
diagnose with smear, osmotic fragility test
treat with splenectomy (still spherocytes but less hemolysis)
34
why Hydroxyurea in SS?
increases HbF production
35
Pt with CML now has AML (blast crisis) with image of blast cell with auer rods (M3 vairant)
treatment?
ATRA for AML Blast Crisis Blast Cells Auer Rods (M3 variant)
ALL-TRANS RETINOIC ACID
36
Ig's in cold vs warm autoimmune hemolytic anemia
IgM in COLD Agluttinin disease
IgG in WARM Agluttinin disease
37
Hb electrophoresis is useful for...
B thal
| SS
38
what tends to run out first, iron stores or folate stores
Folate stores run out first
39
treat
```
CML
AML M3 blasts Auer Rods
AML not m3 no auers
ALL kid, blasts
CLL
```
CML - IMATINIB
AML M3 blasts Auer Rods - ATRA
AML not m3 no auers - ARA-C Cytarabine and IDARUBICIN
ALL kid, blasts - ARA-C Cytarabine (intrathecal) , MTX, CYCLOPHOSPHAMIDE, DOXORUBICIN,
VINCRISTINE
CLL - often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB
40
what comes before splenectomy for ITP?
ITP stands for?
steroids before splenectomy for ITP
Immune/Idiopathic Thrombocytopenic Purpura
41
treat CLL
often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB
42
anti-nausea excalating ladder for chemo
promethazine (safe)
metoclopramide (can cause tardive dyskinesia)
ondansetron - potent for chemo... but used off label for everything
dexamethasone - pulsatile, prior to chemo
43
treat AML not m3 variant without auer rods
Ara-C and Idarubicin
| for AML not m3 no auer rods
44
treat ALL - kid, blasts on smear, needs intrathecal prophylaxis
Ara-C
MTX methotrexate
Cyclophosphamide
Doxorubicin
for ALL
45
treat CML
mechanism
Imatinib
| TK inhibitor targeting Philadelphia chromosome translocation BCR-ABL
46
treat
```
CML
AML M3 blasts Auer Rods
AML not m3 no auers
ALL kid, blasts
CLL
```
CML - IMATINIB
AML M3 blasts Auer Rods - ATRA
AML not m3 no auers - ARA-C and IDARUBICIN
ALL kid, blasts - ARA-C, MTX, CYCLOPHOSPHAMIDE, and DOXORUBICIN
CLL - often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB
47
adult
otherwise healthy, asymptomatic
found WBC 45
with neutrophil predominance
diagnosis?
treatment?
mechanism
CML
Imatinib
TK inhibitor targeting Philadelphia chromosome translocation BCR-ABL
48
treat CLL
often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB
49
anti-nausea excalating ladder for chemo
promethazine (safe)
metoclopramide (can cause tardive dyskinesia)
ondansetron - potent for chemo... but used off label for everything
dexamethasone - pulsatile, prior to chemo
50
old guy with massive WBC with Lymphocytes is
CLL
51
ATRA to treat this leukemia
M3 AML variant
| blasts with AUER Rods
52
kid floridly symptomatic with bone pain fever and pancytopenia, this is
ALL
53
not super old adult floridly symptomatic with bone pain fever pancytopenia this is
AML
54
consumption of platelets, microangiopathic hemolytic anemia (shitocytes on smear), renal failure, neurological impairment
in woman think
in kid think
woman - TTP thrombotic thrombocytopenic purpura
kid - HUS with diarrheal illness or uncooked hamburger EColi O157:H7
55
kid, rash caused by infection with autoimmune response via IgA and thrombocytopenia and fever
diagnosis?
what causes the rash?
Henoch-Schonlein Purpura
rash caused by IgA deposition not thrombocytopenia
56
Imatinib treats this leukemia
CML
| TK inhib targeting BCR-ABL tanslocation on Philly chromosome
57
Plasmapheresis used for which leukemia
why
Plasmapheresis for CLL
to treat hyperviscosity syndrome
58
ATRA to treat this leukemia
M3 AML variant
| blasts with AUER Rods
59
when to suspect aplastic anemia in a kid
parvovirus slapped cheek erythematous rash syndrome of 5ths disease and pancytopenia
60
parvovirus slapped cheek erythematous rash syndrome of 5ths disease and pancytopenia
think...
aplastic anemia
61
consumption of platelets, microangiopathic hemolytic anemia (shitocytes on smear), renal failure, neurological impairment
in woman think
in kid think
woman - TTP thrombotic thrombocytopenic purpura
kid - HUS with diarrheal illness or und
62
what is the chemo for NON-Hogkin's lymphoma
R-CHOPR = Radiation and CHOPR
Cyclophosphamide, (hydroxy)Doxorubicin, (oncovorin)Vincristine, Prednisone, Rituximab.... the mnemonic is a combo of generic and commercial....
63
chemo for Hodgkin lymphoma
ABVD
Adriamycin/Doxorubicin Bleomycin, Vinblastine, Dacarbazine
BEACOPP if bad/bulky - Bleomycin Etoposide Adriamycin/Doxorubicin Cyclophosphomide Oncovorin/Vincristine Procarbazine Prednisone
64
chemo for Hodgkin vs Non-Hodgkin lymphoma
Hodkin - ABVD - ADRIAMYCIN/Doxorubicin BLEOMYCIN VinBlastine DAVARBAZINE
BEACOPP if bad/bulky - Bleomycin Etoposide Adriamycin/Doxorubicin Cyclophosphomide Oncovorin/Vincristine Procarbazine Prednisone
Non-Hodgkin - R-CHOPR (R-CDVPR) - RADIATION - CYCLOPHOSPHAMIDE Doxorubicin, VinCristine PREDNISONE RITUXIMAB
--- Hodgkin ABCD's (ABVbD)... Non-Hodgkin needs R CHOPR R CDVcPR
65
when to get bone marrow biopsy in workup of lymphoma
when is it unnecessary
to RULE OUT METS - aka if stage IIA or Better (multiple nodes same side of diaphragm or better)
unnecessary if IIB or Worse (multiple nodes same side of diaphragm with B symptoms or worse) because GETTING CHEMO ANYWAY
66
chemo side effect man for lymphoma drugs
Vincristine and Vinblastine cause Peripheral Neuropathy
Bleomycin causes Pulmonary Fibrosis
Doxorubicin (Adriamycin) and Daunorubicin cause dose-dependent irreversible Cardiomyopathy (decreased EF)
Cisplatin causes Ototoxicity and Neprhotoxicity
Cyclophosphamide causes Hemorrhagic Cystitis -- bladder sits on the biCyclo
67
classic side effect of dacarbazine
no classic side effect of DaCarbazine
DoxoRubicin and DaunoRubicin cause dose-dependent irreversible Cardiomyopathy (decreased EF)
68
what is the chemo for NON-Hogkin's lymphoma
CHOP-R = CHOP and Radiation
| Cyclophosphamide, Vincristine, Prednisone, Rituximab.... the mnemonic is a combo of generic and commercial....
69
chemo for Hodgkin lymphoma
ABVD
| Adriamycin/Doxorubicin Bleomycin, Vinblastine, Dacarbazine
70
College kid with sore throat, tender lymphadenopathy and lymphocytosis on CBC with diff... next step excisional biopsy or heterophile antibody?
Heterophile Antibody
Do Not Biopsy Tender (Reactive) Nodes -- infection/autoimmune NOT LYMPHOMA
and Mono actually has Lymphocytosis (mononuclear as opposed to multi-lobed granulocytes) not monocytosis, so this is classic for mono
71
when to get bone marrow biopsy in workup of lymphoma
when is it unnecessary
to RULE OUT METS - aka if stage IIA or Better (multiple nodes same side of diaphragm or better)
unnecessary if IIB or Worse (multiple nodes same side of diaphragm with B symptoms or worse) because GETTING CHEMO ANYWAY
72
Paclitaxel chemo key side effects
Nausea and Vomiting, big offender
also Peripheral Neuropathy like Vincristine and Vinblastine
73
50yo woman with multiple myeloma has good resoponse to chemo, now best to get bone marrow transplant or just continue chemo?
bone marrow transplant
| she is younger than 70
74
man with blurred vision, fatigue, bleeding gums,
M-spike, lymphocytes in bone marrow, hyperviscocity syndrome... rouleaux formation on smear, peripheral neuropathy, alterned mental status
diagnosis
treatment
Waldenstrom Macroglobulinemia
Plasmapheresis most urgently for viscosity
then chemo, and transplant if v70
75
man with blurred vision, fatigue, bleeding gums,
M-spike, lymphocytes in bone marrow, hyperviscocity syndrome... rouleaux formation on smear, peripheral neuropathy, alterned mental status
diagnosis
pathogenesis
Waldenstrom Macroglobulinemia
bone marrow infiltration with lymphocytes and hyper IgM production - IgM is a pentamer, very sticky, hyperviscocity -- fatigue blurred vision altered mental status peripheral neuropathy, some gingival bleeding from impaired platelet function
76
compare presentations
multiple myeloma
mgus
waldenstrom macroglobulinemia
mm - CRAB hypercalcemia, renal failure, anemia, bone pain
mgus - asymptomatic but labs suggesting early multiple myeloma (protein gap, M spike, plasmacytosis on bone marrow biopsy but v10%) and has potential to convert to mm
waldenstrom macroglobulinemia - hyperviscocity syndrome - fatigue blurred vision altered mental status peripheral neuropathy, some gingival bleeding from impaired platelet function... hyper IgM pentamers very sticky
77
CD markers for Reed Sternberg Hodgkin Lymphoma cells
CD15 and CD30 in Reed Sternberg Hodgin Lymphoma cells
78
pt in ICU, starts bleeding from lines, low plt, low hb, elevated inr pt ptt, elevated d-dimer, low fibrinogen
diagnosis? DIC TTP ITP?
treatment?
DIC - pt in ICU, starts bleeding from lines, low plt, low hb, elevated inr pt ptt, elevated d-dimer, low fibrinogen
treat with blood products until critical illness cleared
TTP would be anemia, thrombocytopenia, ams, but normal fibrinogen and d-dimer... and critical illness would follow TTP no precede it
ITP is isolated to platelets with profoundly low platelets, diagnosis of exclusion
79
DIC vs TTP vs ITP
how related to clinical illness
plt, hb, inr pt ptt ddimer fibrinogen
DIC - oozing from lines after icu admission for critical illness, low plt hb high inr pt ptt high ddimer low fibrinogen
TTP precedes critical illness and icu admission with ams, low plt hb but normal fibrinogen and ddimer
ITP isolated to profoundly low plts, diagnosis of exclusion
80
man with blurred vision, fatigue, bleeding gums,
M-spike, lymphocytes in bone marrow, hyperviscocity syndrome
diagnosis
treatment
Waldenstrom Macroglobulinemia
Plasmapheresis most urgently for viscosity
then chemo, and transplant if v70
81
man with blurred vision, fatigue, bleeding gums,
M-spike, lymphocytes in bone marrow, hyperviscocity syndrome... rouleaux formation on smear, peripheral neuropathy, alterned mental status
diagnosis
pathogenesis
Waldenstrom Macroglobulinemia
bone marrow infiltration with lymphocytes and hyper IgM production - IgM is a pentamer, very sticky, hyperviscocity -- fatigue blurred vision altered mental status peripheral neuropathy, some gingival bleeding from impaired platelet function
82
TF
| plts and hb low in Von Willebrand Disease
F
| deranged Factors in VWD and platelet bleeding but normal hb and plt count
83
deep bleed predisposition (hemarthroses, bruising) generally suggests
superficial bleeding (mucosa gums nose vagina) generally suggests
deep joints bruising - factor deficiency
superficial gums nose vagina - platelet deficiency
84
25yo female with heavy periods and gingival bleeding
think
treat
think VWD von willebrand disease
treat with DDAVP...
85
DIC vs TTP vs ITP
how related to clinical illness
plt, hb, inr pt ptt ddimer fibrinogen
DIC - oozing from lines after icu admission for critical illness, low plt hb high inr pt ptt high ddimer low fibrinogen
TTP precedes critical illness and icu admission with ams, low plt hb but normal fibrinogen and ddimer
ITP isolated to profoundly low plts, diagnosis of exclusion
86
indication for platelet transfusion
absolute
if bleeding
v20 absolute
v50 actively bleeding
for plt transfusion
87
TF
| ITP has low hb high ddimer and low fibrinogen
F
| ITP limited to really low plts but normal hb ddimer fibrinogen... diagnosis of exclusion
88
TF
| HIT causes a factor deficiency
F
| just platelet deficiency in HIT, not factors
89
deep bleed predisposition (hemarthroses, bruising) generally suggests
superficial bleeding (mucosa gums vagina) generally suggests
deep joints bruising - factor deficiency
superficial gums vagina - platelet deficiency
90
25yo female with heavy periods and gingival bleeding
think
treat
think VWD von willebrand disease
treat with DDAVP...
91
how does cirrhosis contribute to bleeding
how would you evaluate this
decreased production of factors 2 7 9 10 c and s, TPO, portal pressure causing splenic sequestration of platelets
eval with INR, Plt count, albumin (idea of synthetic function)
92
indication for platelet transfusion
absolute
if bleeding
v20 absolute
v50 actively bleeding
for plt transfusion
93
old guy after 10 day hospitalization with limited nutrition gets bruising and hematuria with elevated INR but normal hb and plts
think
vitamin K deficiency
old guy after 10 day hospitalization with limited nutrition gets bruising and hematuria with elevated INR but normal hb and plts
94
TF
| HIT causes a factor deficiency
F
| just platelet deficiency in HIT, not factors
95
hemarthrosis in a child you can pretty much guess ___
confirm with abnormal ___ that corrects with ___
Hemophilia A or B (factor 8 or 9 deficiency)
confirm with abnormal PTT that corrects with mixing
96
how to reverse warfarin
how to treat brain bleed on warfarin
warfarin reversal - give vitamin K... eg if not actively bleeding and INR mildly elevated...
treat brain bleed on warfarin - FFP fresh frozen plasma... even IV K takes to long to take effect
97
von willebrand disease is a platelet disorder but may present as a factor __ deficiency
VWD is platelet disorder that may present as factor 8 deficiency (vwf stabilizes factor 8)
98
when is cryoprecipitate the treatment for bleeding
Cryoprecipitate in DIC when Fibrinogen is low (give platelets when platelets are low in DIC)
99
when to give Cryoprecipitate vs FFP in DIC
in DIC
Cryoprecipitate when Fibrinogen low
FFP when plts low
100
what does abnormal coag panel that corrects with mixing study suggest
correction with mixing suggests factor deficiency not inhibition (eg by lupus anticoagulant)
101
hemarthrosis in a child you can pretty much guess ___
Hemophilia A
102
Bernard Soulier is a ___ disorder
Bernard Soulier is a Platelet disorder
103
shistocytes mean MAHA which means one of 3 diagnoses
how do coags and patient age help you figure it out
shitocytes - MAHA - DIC or TTP/HUS
normal PT/PTT means no factors consumed not DIC
TTP if adult
HUS if kid
104
TF
| abnormal PT/PTT in HUS
F
| autoimmune Hyalin clots consume Platelets, not factors
105
lead poisoning causes ___ anemia
lead poisoning Macrocytic anemia
106
ortho postop pt recovering in hospital on LMWH gets DVT, treat
Lepirudin - thrombin inhibitor reserved for HIT
transition to Coumadin for 6 mos after stabilized
107
treat ITP
ITP
IV Prednisone first attempt
IV Ig if severe bleeding and steroids fail
splenectomy if refractory disease
108
before thinking ITP in asymptomatic patient with super low platelets, rule out
lab error
109
shistocytes mean MAHA which means one of 3 diagnoses
how does patient age and coags help you figure it out
shitocytes - MAHA - DIC or TTP/HUS
normal PT/PTT means no factors consumed not DIC
TTP if adult
HUS if kid
110
_ _ _ presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen
TTP
presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen
111
"teardrop cells" on peripheral smear think
myelofibrosis
when "teardrop cells" on peripheral smear
112
ortho postop pt recovering in hospital on LMWH gets DVT, treat
Lepirudin - thrombin inhibitor reserved for HIT
113
25 yo F asymptomatic with plts 55 likely ITP
manage
f/u plts in 1 week
no steroids, ivig, or splenectomy if not symptomatic/bleeding
no platelet transfusion if ^40 and not bleeding...
114
_ _ _ is "not anything else but low platelets" - no other lab abnorms
ITP is "not anything else but low platelets" - no other lab abnorms
115
treat HIT
stop the heparin
start -troban... dabigatran, argatroban, etc
send hit panel
116
_ _ _ presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen
TTP
presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen
117
"teardrop cells" on peripheral smear think
myelofibrosis
when "teardrop cells" on peripheral smear
118
kid 2-10yo, infections, lymphadenopathy, splenomegaly. anemia, thrombocytopenia, neutropenia, lymphoblasts on smear with PAS positive aggregats and positive TdT staining
diagnosis
ALL
| acute lymphblastic leukemia
119
TdT is expressed only in...
TdT only in Pre B and Pre T Lymphoblasts
120
```
TTP
mnemonic for symptoms
lab findings
pathogenesis
treat
NEVER give __, why
```
_ _ _ presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms... low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen
autoimmune ADAMTS-13 deficiency, not there to cleave vWF multimers, MAHA (hyalin clots, shistocytes, plt and hb consumption but not pt ptt inr fibrinogen ddimer derangements)
treat with Plasmapheresis
Never give platelets, it will worsen maha
121
treat HIT
stop the heparin
start -troban... dabigatran, argatroban, etc
send hit panel
122
most common cause of thrombophilia
what to think if young woman with DVT history of miscarriages and family history of lupus
Factor V Leiden is most common cause of thrombophilia
AntiPhospholipid Antibody Syndrome (Lupus) if young woman hinting at autoimmune
123
most common complication of sickle cell trait
painless hematuria - sickling in renal medulla
124
prolonged PT or PTT?
vit k deficiency
hemophilia A
vit k def - PT prolongued.... PTT too if severe
hemophilia A (factor 8) PTT prolongued
125
TdT is expressed only in...
TdT only in Pre B and Pre T Lymphoblasts
126
PAS vs Peroxidase positivity Lymphoblasts vs Myeloblasts
PAS lymphoblasts pre B and T
Peroxidase myeloblasts
127
TF
| lymphadenopathy and splenomegaly in ALL
T
| ... not just lymphomas...
128
```
neoplasm of mature B cells
EBV associated
mandibular or abdominal mass
high mitotic index
starry sky histology
agressive
responds well to chemo
```
diagnosis
Burkitt Lymphoma
129
anemic baby with hypochromic, microcytic RBCs and target cells think
thalassemia, alpha or beta
130
prolonged PT or PTT?
vit k deficiency
hemophilia A
vit k def - PT prolongued.... PTT too if severe
hemophilia A (factor 8) PTT prolongued
131
why would 8yo Sickler have Howell-Jolly Bodies on peripheral smear?
auto-Splenic Infarct by 5yo in sickle cell disease
spleen usually removes nuclear remnants of red blood cells (small purple dots in RBCs - Howell Jolly Bodies)
132
basophilic stippling on peripheral smear think
thalassemia
lead
heavy metal poisoning
with basophilic stippling on peripheral smear (ribosomal precipitates)
133
TF
| helmet cells / shistocytes on smear in sickle crisis
F
helmets / shistocytes = MAHA = traumatic extrinsic hemolysis = DIC TTP/HUS
sickle hemolysis is intrinsic, non-traumatic
134
pathogensis of anemia of prematurity
treatment
impaired EPO production
short RBC lifespan
iatrognic blood draws
limit bood draws
iron supplementation
transfuse as necessary
135
TF
| Anisocytosis on peripheral smear in 8 month old suggests iron deficiency anemia
T
iron deficiency the most common cause of anemia in kid after 6mos old
Anisocytosis (rbc's of unequal sizes) often the earliest finding on peripheral smear
136
kid post infection, antibiotics, radiation, insecticide, or toxin with anemia and profound hypocellularity on bone marrow biopsy
diagnosis
Acquired Aplastic Anemia
137
kid with pancytopenia, congenital hyperpigmentation of the trunk, intertriginous areas, cafe-au-lait spots, short stature, upper limb abnormalities, hypogonadism, skeletal anomalies, eye or eyelid changes, renal malformations... thrombocytopenia, then neutropenia, then anemia...
diagnosis
Fanconi Anemia
138
acute severe anemia in sickler kid
ddx? how does age and reticulocyte count inform? pathopys?
aplastic crisis - low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 infection suddenly halts erythropoiesis
splenic sequestration crisis - high retic and not yet autosplenectomy (v5yo)
-vasoocclusion and pooling in spleen
139
severe anemia Hb v6 and low retics in sickle cell crisis kid ^5yo
diagnosis
most common cause
aplastic crisis
low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 infection suddenly halts erythropoiesis
140
iron deficiency is rare in kids before but common after how old
iron deficiency rare before 6 mos (mom supplied stores)
common after 6 mos
141
blood iron and ferritin levels in kid with thalassemia
iron and ferritin high in thalassemia from increased rbc turnover
142
TIBC and ferritin in anemia of chronic disease
```
TIBC decreased (hide iron)
Ferritin increased (inflammatory marker)
```
in anemia of chronic disease
143
acute severe anemia in sickler kid
ddx? how does age and reticulocyte count inform? pathopys?
aplastic crisis - low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 suddenly halts erythropoiesis
splenic sequestration crisis - high retic and not yet autosplenectomy (v5yo)
-vasoocclusion and pooling in spleen
144
severe anemia Hb v6 and low retics in sickle cell crisis kid ^5yo
diagnosis
most common cause
aplastic crisis
low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 infection suddenly halts erythropoiesis
145
what does plasma exchange accomplish
what 2 heme onc disorders is it used for
plasma exchange Removes Toxic Substances from plasma
used for TTP and HUS
146
EPO level in polycythemia vera
| why
EPO low in PCV
because pathogenesis is almost always a Constitutively Active JAK2 Mutation that induces erythropoiesis without EPO (normally hypoxia stims ep stims jak2)
147
ESR level in polycythemia vera
ESR low in PCV
| high rbc to firbin ratio, lower esr... esr elevates with inflammation typically
148
what kind of transfusion reaction caused by recipient anti-IgA vs IgE and Mast cells
anti-IgA -Anaphylactic transfusion reaction (angioedema hypotension sob...shock)
IgE and Mast cells - Urticarial/Allergic transfusion reaction (rash)
149
DVT wihth high plasma homocysteine, treat
heparin to warfarin
Pyridoxine (B6), Folate, and B12 (if low) supplementation (cofactors for homocysteine metabolism)
150
what does plasma exchange accomplish
what 2 heme onc disorders is it used for
plasma exchange Removes Toxic Substances from plasma
used for TTP and HUS
151
short and long term complications of ivc filter both involve thrombus formation... at different locations - which ones
short - thrombus at venipuncture site
long - thrombus accumulation around filter in ivc
152
"amnestic antibody response" causes what kind of transfusion reaction
delayed hemolytic transfusion reaction
153
what kind of transfusion reaction caused by recipient anti-IgA vs IgE and Mast cells
anti-IgA -Anaphylactic transfusion reaction (angioedema hypotension sob...shock)
IgE and Mast cells - Urticarial/Allergic transfusion reaction (rash)
154
DVT wihth high plasma homocysteine, treat
heparin to warfarin
Pyridoxine (B6), Folate, and B12 (if low) supplementation (cofactors for homocysteine metabolism)
155
ITP
| pathogenesis
IgG autoantibodies against platelet glycoproteins - destroy platelets and inhibit megakaryocyte platelet destruction... presenting as low plts in young woman and maybe some bleeding but otherwise normal labs etc
156
short and long term complications of ivc filter both involve thrombus formation... at different locations - which ones
short - thrombus at venipuncture site
long - thrombus accumulation around filter in ivc
157
old lady with recurrent PNA and URTIs and osteolytic rib fractures and mild anemia
diagnosis
multiple myeloma
158
most common infections with multiple myeloma
| why
PNAs
URTIs
ineffective antibodies - hypogammaglobulinemia... and if advanced maybe replacing other immune cells
159
TF
| hypogammaglobulinemia with multiple myeloma
T
| antibodies produced are ineffective
160
ITP
| pathogenesis
IgG autoantibodies against platelet glycoproteins - destroy platelets and inhibit megakaryocyte platelet destruction
