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Medicine > Heme Onc > Flashcards

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
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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)

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3
Q

MCV
bili
LDH
haptoglobin

in hemolysis

A

MCV normal
Bili elevated
LDH elevated
haptoglobin low

in hemolysis

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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…

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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

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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…

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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

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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

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9
Q

beta thalassemia peripheral smear

A

small target cells

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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

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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

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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

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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)

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14
Q

B12 aka

A

cobalamin

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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

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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)

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17
Q

Anemia
Low MCV
Normal iron studies
Normal Hb electrophoresis

Dx

A

Alpha Thalassemia

beta thal had abnormal electrophoresis

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18
Q

Anemia
mcv, reticulocute count, ldh normal
Elevated Ferritin low tibc low iron

Dx

A

Anemia of Chronic Inflammatory Disease

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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

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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)

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21
Q

other than folate and b12 deficiencies, what else can cause macrocytic anemia

A

Alcohol

Liver Disease

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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…

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23
Q

Hb cutoff for EPO for anemia caused by renal disease

A

Hb v10 caused by renal disease give EPO

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24
Q

Two times you should go for bone marrow biopsy for Anemia

A

serum Iron is High (sideroblastic anemia)

Pancytopenia (infiltrative)

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25
Q

etiology of hereditary spherocytosis

diagnose
treat

A

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)

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26
Q 
TF
TMP SMX (bactrim) is a sulfa antibiotic that can reveal G6PD deficiency
A

T

induces oxidative stress

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27
Q

pathogenesis of paroxysmal nocturnal hemoglobinuria

A

deficiency in PIG-A gene

…GDI anchor fails and RBCs become succeptible to Complement Fixation

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28
Q

24yo with paroxysms of dark urine in the morning with hemosiderin rather than blood, abdominal pain,

diagnosis
course
pathogenesis
diagnose

A

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

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29
Q

other than folate and b12 deficiencies, what else can cause macrocytic anemia

A

Alcohol

Liver Disease

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30
Q

transfusion, non-cardiogenic pulmonary edema, and fever is ___

A

TRALI

transfusion-related lung injury

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31
Q

treat acute chest syndrome in sickle cell crisis

A

exchange transfusion

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32
Q

workup for hemolysis

A

Smear
Electrophoresis
Coombs test

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33
Q

etiology of hereditary spherocytosis

diagnose
treat

A

defective RBC CELL MEMBRANE PROTEIN
-Spectrin, Ankyrin, Band 4.1

diagnose with smear, osmotic fragility test

treat with splenectomy (still spherocytes but less hemolysis)

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34
Q

why Hydroxyurea in SS?

A

increases HbF production

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35
Q

Pt with CML now has AML (blast crisis) with image of blast cell with auer rods (M3 vairant)

treatment?

A

ATRA for AML Blast Crisis Blast Cells Auer Rods (M3 variant)

ALL-TRANS RETINOIC ACID

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36
Q

Ig’s in cold vs warm autoimmune hemolytic anemia

A

IgM in COLD Agluttinin disease

IgG in WARM Agluttinin disease

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37
Q

Hb electrophoresis is useful for…

A

B thal

SS

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38
Q

what tends to run out first, iron stores or folate stores

A

Folate stores run out first

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39
Q

treat

CML
AML M3 blasts Auer Rods
AML not m3 no auers
ALL kid, blasts
CLL
A

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

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40
Q

what comes before splenectomy for ITP?

ITP stands for?

A

steroids before splenectomy for ITP

Immune/Idiopathic Thrombocytopenic Purpura

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41
Q

treat CLL

A

often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB

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42
Q

anti-nausea excalating ladder for chemo

A

promethazine (safe)

metoclopramide (can cause tardive dyskinesia)

ondansetron - potent for chemo… but used off label for everything

dexamethasone - pulsatile, prior to chemo

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43
Q

treat AML not m3 variant without auer rods

A

Ara-C and Idarubicin

for AML not m3 no auer rods

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44
Q

treat ALL - kid, blasts on smear, needs intrathecal prophylaxis

A

Ara-C
MTX methotrexate
Cyclophosphamide
Doxorubicin

for ALL

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45
Q

treat CML

mechanism

A

Imatinib

TK inhibitor targeting Philadelphia chromosome translocation BCR-ABL

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46
Q

treat

CML
AML M3 blasts Auer Rods
AML not m3 no auers
ALL kid, blasts
CLL
A

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

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47
Q

adult
otherwise healthy, asymptomatic
found WBC 45
with neutrophil predominance

diagnosis?
treatment?
mechanism

A

CML

Imatinib
TK inhibitor targeting Philadelphia chromosome translocation BCR-ABL

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48
Q

treat CLL

A

often no treatment, FLUDRABINE, CYCLOPHOSPHAMIDE, RITUXIMAB

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49
Q

anti-nausea excalating ladder for chemo

A

promethazine (safe)

metoclopramide (can cause tardive dyskinesia)

ondansetron - potent for chemo… but used off label for everything

dexamethasone - pulsatile, prior to chemo

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50
Q

old guy with massive WBC with Lymphocytes is

A

CLL

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51
Q

ATRA to treat this leukemia

A

M3 AML variant

blasts with AUER Rods

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52
Q

kid floridly symptomatic with bone pain fever and pancytopenia, this is

A

ALL

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53
Q

not super old adult floridly symptomatic with bone pain fever pancytopenia this is

A

AML

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54
Q

consumption of platelets, microangiopathic hemolytic anemia (shitocytes on smear), renal failure, neurological impairment

in woman think

in kid think

A

woman - TTP thrombotic thrombocytopenic purpura

kid - HUS with diarrheal illness or uncooked hamburger EColi O157:H7

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55
Q

kid, rash caused by infection with autoimmune response via IgA and thrombocytopenia and fever

diagnosis?
what causes the rash?

A

Henoch-Schonlein Purpura

rash caused by IgA deposition not thrombocytopenia

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56
Q

Imatinib treats this leukemia

A

CML

TK inhib targeting BCR-ABL tanslocation on Philly chromosome

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57
Q

Plasmapheresis used for which leukemia

why

A

Plasmapheresis for CLL

to treat hyperviscosity syndrome

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58
Q

ATRA to treat this leukemia

A

M3 AML variant

blasts with AUER Rods

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59
Q

when to suspect aplastic anemia in a kid

A

parvovirus slapped cheek erythematous rash syndrome of 5ths disease and pancytopenia

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60
Q

parvovirus slapped cheek erythematous rash syndrome of 5ths disease and pancytopenia
think…

A

aplastic anemia

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61
Q

consumption of platelets, microangiopathic hemolytic anemia (shitocytes on smear), renal failure, neurological impairment

in woman think

in kid think

A

woman - TTP thrombotic thrombocytopenic purpura

kid - HUS with diarrheal illness or und

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62
Q

what is the chemo for NON-Hogkin’s lymphoma

A

R-CHOPR = Radiation and CHOPR
Cyclophosphamide, (hydroxy)Doxorubicin, (oncovorin)Vincristine, Prednisone, Rituximab…. the mnemonic is a combo of generic and commercial….

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63
Q

chemo for Hodgkin lymphoma

A

ABVD
Adriamycin/Doxorubicin Bleomycin, Vinblastine, Dacarbazine

BEACOPP if bad/bulky - Bleomycin Etoposide Adriamycin/Doxorubicin Cyclophosphomide Oncovorin/Vincristine Procarbazine Prednisone

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64
Q

chemo for Hodgkin vs Non-Hodgkin lymphoma

A

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

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65
Q

when to get bone marrow biopsy in workup of lymphoma

when is it unnecessary

A

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
Q

chemo side effect man for lymphoma drugs

A

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
Q

classic side effect of dacarbazine

A

no classic side effect of DaCarbazine

DoxoRubicin and DaunoRubicin cause dose-dependent irreversible Cardiomyopathy (decreased EF)

68
Q

what is the chemo for NON-Hogkin’s lymphoma

A

CHOP-R = CHOP and Radiation

Cyclophosphamide, Vincristine, Prednisone, Rituximab…. the mnemonic is a combo of generic and commercial….

69
Q

chemo for Hodgkin lymphoma

A

ABVD

Adriamycin/Doxorubicin Bleomycin, Vinblastine, Dacarbazine

70
Q

College kid with sore throat, tender lymphadenopathy and lymphocytosis on CBC with diff… next step excisional biopsy or heterophile antibody?

A

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
Q

when to get bone marrow biopsy in workup of lymphoma

when is it unnecessary

A

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
Q

Paclitaxel chemo key side effects

A

Nausea and Vomiting, big offender

also Peripheral Neuropathy like Vincristine and Vinblastine

73
Q

50yo woman with multiple myeloma has good resoponse to chemo, now best to get bone marrow transplant or just continue chemo?

A

bone marrow transplant

she is younger than 70

74
Q

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

A

Waldenstrom Macroglobulinemia

Plasmapheresis most urgently for viscosity
then chemo, and transplant if v70

75
Q

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

A

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
Q

compare presentations

multiple myeloma
mgus
waldenstrom macroglobulinemia

A

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
Q

CD markers for Reed Sternberg Hodgkin Lymphoma cells

A

CD15 and CD30 in Reed Sternberg Hodgin Lymphoma cells

78
Q

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?

A

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
Q

DIC vs TTP vs ITP
how related to clinical illness
plt, hb, inr pt ptt ddimer fibrinogen

A

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
Q

man with blurred vision, fatigue, bleeding gums,
M-spike, lymphocytes in bone marrow, hyperviscocity syndrome

diagnosis
treatment

A

Waldenstrom Macroglobulinemia

Plasmapheresis most urgently for viscosity
then chemo, and transplant if v70

81
Q

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

A

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
Q

TF

plts and hb low in Von Willebrand Disease

A

F

deranged Factors in VWD and platelet bleeding but normal hb and plt count

83
Q

deep bleed predisposition (hemarthroses, bruising) generally suggests

superficial bleeding (mucosa gums nose vagina) generally suggests

A

deep joints bruising - factor deficiency

superficial gums nose vagina - platelet deficiency

84
Q

25yo female with heavy periods and gingival bleeding

think
treat

A

think VWD von willebrand disease

treat with DDAVP…

85
Q

DIC vs TTP vs ITP
how related to clinical illness
plt, hb, inr pt ptt ddimer fibrinogen

A

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
Q

indication for platelet transfusion

absolute

if bleeding

A

v20 absolute

v50 actively bleeding

for plt transfusion

87
Q

TF

ITP has low hb high ddimer and low fibrinogen

A

F

ITP limited to really low plts but normal hb ddimer fibrinogen… diagnosis of exclusion

88
Q

TF

HIT causes a factor deficiency

A

F

just platelet deficiency in HIT, not factors

89
Q

deep bleed predisposition (hemarthroses, bruising) generally suggests

superficial bleeding (mucosa gums vagina) generally suggests

A

deep joints bruising - factor deficiency

superficial gums vagina - platelet deficiency

90
Q

25yo female with heavy periods and gingival bleeding

think
treat

A

think VWD von willebrand disease

treat with DDAVP…

91
Q

how does cirrhosis contribute to bleeding

how would you evaluate this

A

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
Q

indication for platelet transfusion

absolute

if bleeding

A

v20 absolute

v50 actively bleeding

for plt transfusion

93
Q

old guy after 10 day hospitalization with limited nutrition gets bruising and hematuria with elevated INR but normal hb and plts

think

A

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
Q

TF

HIT causes a factor deficiency

A

F

just platelet deficiency in HIT, not factors

95
Q

hemarthrosis in a child you can pretty much guess ___

confirm with abnormal ___ that corrects with ___

A

Hemophilia A or B (factor 8 or 9 deficiency)

confirm with abnormal PTT that corrects with mixing

96
Q

how to reverse warfarin

how to treat brain bleed on warfarin

A

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
Q

von willebrand disease is a platelet disorder but may present as a factor __ deficiency

A

VWD is platelet disorder that may present as factor 8 deficiency (vwf stabilizes factor 8)

98
Q

when is cryoprecipitate the treatment for bleeding

A

Cryoprecipitate in DIC when Fibrinogen is low (give platelets when platelets are low in DIC)

99
Q

when to give Cryoprecipitate vs FFP in DIC

A

in DIC
Cryoprecipitate when Fibrinogen low
FFP when plts low

100
Q

what does abnormal coag panel that corrects with mixing study suggest

A

correction with mixing suggests factor deficiency not inhibition (eg by lupus anticoagulant)

101
Q

hemarthrosis in a child you can pretty much guess ___

A

Hemophilia A

102
Q

Bernard Soulier is a ___ disorder

A

Bernard Soulier is a Platelet disorder

103
Q

shistocytes mean MAHA which means one of 3 diagnoses

how do coags and patient age help you figure it out

A

shitocytes - MAHA - DIC or TTP/HUS

normal PT/PTT means no factors consumed not DIC

TTP if adult

HUS if kid

104
Q

TF

abnormal PT/PTT in HUS

A

F

autoimmune Hyalin clots consume Platelets, not factors

105
Q

lead poisoning causes ___ anemia

A

lead poisoning Macrocytic anemia

106
Q

ortho postop pt recovering in hospital on LMWH gets DVT, treat

A

Lepirudin - thrombin inhibitor reserved for HIT

transition to Coumadin for 6 mos after stabilized

107
Q

treat ITP

A

ITP
IV Prednisone first attempt
IV Ig if severe bleeding and steroids fail
splenectomy if refractory disease

108
Q

before thinking ITP in asymptomatic patient with super low platelets, rule out

A

lab error

109
Q

shistocytes mean MAHA which means one of 3 diagnoses

how does patient age and coags help you figure it out

A

shitocytes - MAHA - DIC or TTP/HUS

normal PT/PTT means no factors consumed not DIC

TTP if adult

HUS if kid

110
Q

_ _ _ presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen

A

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
Q

“teardrop cells” on peripheral smear think

A

myelofibrosis

when “teardrop cells” on peripheral smear

112
Q

ortho postop pt recovering in hospital on LMWH gets DVT, treat

A

Lepirudin - thrombin inhibitor reserved for HIT

113
Q

25 yo F asymptomatic with plts 55 likely ITP

manage

A

f/u plts in 1 week

no steroids, ivig, or splenectomy if not symptomatic/bleeding

no platelet transfusion if ^40 and not bleeding…

114
Q

_ _ _ is “not anything else but low platelets” - no other lab abnorms

A

ITP is “not anything else but low platelets” - no other lab abnorms

115
Q

treat HIT

A

stop the heparin
start -troban… dabigatran, argatroban, etc
send hit panel

116
Q

_ _ _ presents as a FAT RN fever anemia thrombocytopenia renal failure neuro symptoms, low platelets and hemoglobin and shistocytes, normal inr pt ptt fibrinogen

A

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
Q

“teardrop cells” on peripheral smear think

A

myelofibrosis

when “teardrop cells” on peripheral smear

118
Q

kid 2-10yo, infections, lymphadenopathy, splenomegaly. anemia, thrombocytopenia, neutropenia, lymphoblasts on smear with PAS positive aggregats and positive TdT staining

diagnosis

A

ALL

acute lymphblastic leukemia

119
Q

TdT is expressed only in…

A

TdT only in Pre B and Pre T Lymphoblasts

120
Q 
TTP
mnemonic for symptoms
lab findings
pathogenesis
treat
NEVER give \_\_, why
A

_ _ _ 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
Q

treat HIT

A

stop the heparin
start -troban… dabigatran, argatroban, etc
send hit panel

122
Q

most common cause of thrombophilia

what to think if young woman with DVT history of miscarriages and family history of lupus

A

Factor V Leiden is most common cause of thrombophilia

AntiPhospholipid Antibody Syndrome (Lupus) if young woman hinting at autoimmune

123
Q

most common complication of sickle cell trait

A

painless hematuria - sickling in renal medulla

124
Q

prolonged PT or PTT?

vit k deficiency
hemophilia A

A

vit k def - PT prolongued…. PTT too if severe

hemophilia A (factor 8) PTT prolongued

125
Q

TdT is expressed only in…

A

TdT only in Pre B and Pre T Lymphoblasts

126
Q

PAS vs Peroxidase positivity Lymphoblasts vs Myeloblasts

A

PAS lymphoblasts pre B and T

Peroxidase myeloblasts

127
Q

TF

lymphadenopathy and splenomegaly in ALL

A

T

… not just lymphomas…

128
Q 
neoplasm of mature B cells
EBV associated
mandibular or abdominal mass
high mitotic index
starry sky histology
agressive
responds well to chemo

diagnosis

A

Burkitt Lymphoma

129
Q

anemic baby with hypochromic, microcytic RBCs and target cells think

A

thalassemia, alpha or beta

130
Q

prolonged PT or PTT?

vit k deficiency
hemophilia A

A

vit k def - PT prolongued…. PTT too if severe

hemophilia A (factor 8) PTT prolongued

131
Q

why would 8yo Sickler have Howell-Jolly Bodies on peripheral smear?

A

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
Q

basophilic stippling on peripheral smear think

A

thalassemia
lead
heavy metal poisoning

with basophilic stippling on peripheral smear (ribosomal precipitates)

133
Q

TF

helmet cells / shistocytes on smear in sickle crisis

A

F
helmets / shistocytes = MAHA = traumatic extrinsic hemolysis = DIC TTP/HUS

sickle hemolysis is intrinsic, non-traumatic

134
Q

pathogensis of anemia of prematurity

treatment

A

impaired EPO production
short RBC lifespan
iatrognic blood draws

limit bood draws
iron supplementation
transfuse as necessary

135
Q

TF

Anisocytosis on peripheral smear in 8 month old suggests iron deficiency anemia

A

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
Q

kid post infection, antibiotics, radiation, insecticide, or toxin with anemia and profound hypocellularity on bone marrow biopsy

diagnosis

A

Acquired Aplastic Anemia

137
Q

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

A

Fanconi Anemia

138
Q

acute severe anemia in sickler kid

ddx? how does age and reticulocyte count inform? pathopys?

A

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
Q

severe anemia Hb v6 and low retics in sickle cell crisis kid ^5yo

diagnosis
most common cause

A

aplastic crisis
low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 infection suddenly halts erythropoiesis

140
Q

iron deficiency is rare in kids before but common after how old

A

iron deficiency rare before 6 mos (mom supplied stores)

common after 6 mos

141
Q

blood iron and ferritin levels in kid with thalassemia

A

iron and ferritin high in thalassemia from increased rbc turnover

142
Q

TIBC and ferritin in anemia of chronic disease

A 
TIBC decreased (hide iron)
Ferritin increased (inflammatory marker)

in anemia of chronic disease

143
Q

acute severe anemia in sickler kid

ddx? how does age and reticulocyte count inform? pathopys?

A

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
Q

severe anemia Hb v6 and low retics in sickle cell crisis kid ^5yo

diagnosis
most common cause

A

aplastic crisis
low retics and after age of autosplenectomy (^5yo)
-parvovirus B19 infection suddenly halts erythropoiesis

145
Q

what does plasma exchange accomplish

what 2 heme onc disorders is it used for

A

plasma exchange Removes Toxic Substances from plasma

used for TTP and HUS

146
Q

EPO level in polycythemia vera

why

A

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
Q

ESR level in polycythemia vera

A

ESR low in PCV

high rbc to firbin ratio, lower esr… esr elevates with inflammation typically

148
Q

what kind of transfusion reaction caused by recipient anti-IgA vs IgE and Mast cells

A

anti-IgA -Anaphylactic transfusion reaction (angioedema hypotension sob…shock)

IgE and Mast cells - Urticarial/Allergic transfusion reaction (rash)

149
Q

DVT wihth high plasma homocysteine, treat

A

heparin to warfarin

Pyridoxine (B6), Folate, and B12 (if low) supplementation (cofactors for homocysteine metabolism)

150
Q

what does plasma exchange accomplish

what 2 heme onc disorders is it used for

A

plasma exchange Removes Toxic Substances from plasma

used for TTP and HUS

151
Q

short and long term complications of ivc filter both involve thrombus formation… at different locations - which ones

A

short - thrombus at venipuncture site

long - thrombus accumulation around filter in ivc

152
Q

“amnestic antibody response” causes what kind of transfusion reaction

A

delayed hemolytic transfusion reaction

153
Q

what kind of transfusion reaction caused by recipient anti-IgA vs IgE and Mast cells

A

anti-IgA -Anaphylactic transfusion reaction (angioedema hypotension sob…shock)

IgE and Mast cells - Urticarial/Allergic transfusion reaction (rash)

154
Q

DVT wihth high plasma homocysteine, treat

A

heparin to warfarin

Pyridoxine (B6), Folate, and B12 (if low) supplementation (cofactors for homocysteine metabolism)

155
Q

ITP

pathogenesis

A

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
Q

short and long term complications of ivc filter both involve thrombus formation… at different locations - which ones

A

short - thrombus at venipuncture site

long - thrombus accumulation around filter in ivc

157
Q

old lady with recurrent PNA and URTIs and osteolytic rib fractures and mild anemia

diagnosis

A

multiple myeloma

158
Q

most common infections with multiple myeloma

why

A

PNAs
URTIs

ineffective antibodies - hypogammaglobulinemia… and if advanced maybe replacing other immune cells

159
Q

TF

hypogammaglobulinemia with multiple myeloma

A

T

antibodies produced are ineffective

160
Q

ITP

pathogenesis

A

IgG autoantibodies against platelet glycoproteins - destroy platelets and inhibit megakaryocyte platelet destruction

Medicine (13 decks)

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