Heme/onc: First Aid For Step 2 Ck

Front 1

Antidote for Heparin over-anticoagulation

Back 1

Protamine.

Front 2

Mechanism of Heparin

Back 2

Activates Antithrombin III

Front 3

Mechanism of Warfarin

Back 3

inhibits vitamin K

Front 4

Antidote for Warfarin over-anticoagulation

Back 4

Vitamin K

Front 5

Mechanism of Enoxaparin

Back 5

inhibits factor Xa

Front 6

Petechiae indicate dysfunction of what?

Back 6

platelets

Front 7

Hemarthroses indicate disorder of what?

Back 7

coag factors

Front 8

Heparain Bridge is required due to a temporary hypercoagulability before Warfarin is able to inhibit Vit K dependent factors to anti-coagulate. What is the mechanism?

Back 8

Protein C and S are inhibited first

Front 9

What are the Vit K dependent coag factors?

Back 9

II, VII, IX, X (remember there are four; II+VII=IX, and one more: one+IX=X)

Front 10

Most common Hemophilia (90%).

Back 10

Hemophilia A (Factor VIII defic)

Front 11

Hemophilia B makes up what percent of all hemophilias?

Back 11

9% (remember Factor IX deficiency = 9 percent)

Front 12

Hemophilia A is deficiency in what coag factor?

Back 12

VIII (remember EIGHT sounds like A)

Front 13

V-Lieden is clotting disorder because factor V is resistant to what?

Back 13

Activated Protein C (C+S=APC)

Front 14

Hemophilia B is deficiency in what coag factor?

Back 14

IX

Front 15

Intrinsic Coag Cascade pathway is measured by what blood test?

Back 15

PTT

Front 16

Heparin elevates what coag blood test?

Back 16

PTT

Front 17

Warfarin elevates what coag blood test?

Back 17

PT

Front 18

Extrinsic Coag Cascade pathway is measured by what coag blood test?

Back 18

PT

Front 19

Most common inherited Bleeding Disorder (1% of the population)?

Back 19

von Willebrand's Disease (vWD)

Front 20

vWF normally binds what coag factor?

Back 20

VIII

Front 21

Hemophilia A has what form of inheritance?

Back 21

X-linked Recessive

Front 22

Hemophilia B has what form of inheritance?

Back 22

X-linked Recessive

Front 23

Why does arthropathy and joint destruction occur in untreated Hemophiliacs?

Back 23

Hemosiderin deposits cause fibrosis

Front 24

How to differentiate between Clotting Factor Deficiency and Clotting Factor Inhibitor.

Back 24

Mixing study: mix pt blood with normal blood and test PTT: if it does not correct, likely Inhibitor present.

Front 25

Mild symtoms of Hemophilia develope around what level of clotting factor levels (compared to normal)?

Back 25

5% of normal

Front 26

Treatment for bleeding Hemophiliac?

Back 26

Cryoprecipitate

Front 27

What does the half life of Factor VIII mean for treatment in Hemophilia A who is bleeding?

Back 27

12 hours (if bleeding, will need cryoprecipitate BID)

Front 28

What does the half life of Factor IX mean for treatment in Hemophilia B who is bleeding?

Back 28

24 hours (if bleeding, will need cryoprecipitate qDay)

Front 29

Mild hemophilia A can be treated with this medication.

Back 29

Desmopressin (DDAVP)

Front 30

Patients on Desmopression should be fluid restricted to avoid this complication.

Back 30

Hyponatremia

Front 31

15% of Hemophilia A patients have to stop taking replacement factor VIII. Why?

Back 31

they develop neutralizing IgG antibodies to Factor VIII

Front 32

von Willebrand's Disease (vWD) has what form of inheritance pattern?

Back 32

Autosomal Dominant

Front 33

This over the counter medication is contraindicated in vWD.

Back 33

Aspirin (avoid platelet inhibitors)

Front 34

Usual location of bleeding in vWD.

Back 34

mucosal (e.g. nose bleeds)

Front 35

How do female patients with vWD usually present?

Back 35

menorrhagia

Front 36

Treatment for patient with vWD who is actively bleeding.

Back 36

Desmopressin (DDAVP)

Front 37

What is the Ristocetin Cofactor Asay (for vWD)?

Back 37

measures vWF ability to agglutinate platelets in vitro

Front 38

Most common inherited Thrombophilia.

Back 38

Factor V Leiden

Front 39

What is the function of Activated Protein C (APC)?

Back 39

inhibits Factor V (inhibits clotting cascade)

Front 40

Why will vWD patients sometimes have elevated PTT?

Back 40

relative VIII deficiency

Front 41

Why would women with Factor V Leiden often present first to their OB/GYN doctor?

Back 41

may have recurrent miscarriages

Front 42

CLOTTING-SX mnemonic for acquired hypercoagulable states.

Back 42

Cancer
Lupus Antigen/antiphospholipid syndrome
Obesity
Trauma
Tobacco
Immobility
Nephrotic syndrome
Gravid
Surgery
X-(tx)-Meds (e.g. OCPs)

Front 43

3 Vitamins to give in treatment of Hyprehomocysteinemia.

Back 43

B6 alone or with: B12 and Folate

Front 44

No identifiable cause for clotting. What hypercoagulability labs to order? (7) Px-in-CALVES mnemonic

Back 44

Prothrombin G0210A
C-protien
Antithrombin III
Lupus Antigen/Antiphospholipid Syndrome
V Leiden (APC resistance)
Extra Homocystein
S-protein

Front 45

3 main cuases of DIC.

Back 45

Delivery (obsetric stuff)
Infection (epecially septicemia)
Cancer (APML aka AML M3)

Front 46

In DIC, which coag factor is elevated?

Back 46

PT and PTT

Front 47

In TTP, which coag factor is elevated?

Back 47

neither PT or PTT is elevated

Front 48

In vWD, which coag factor is elevated?

Back 48

PTT

Front 49

In Hemophilia, which coag factor is elevated?

Back 49

PTT

Front 50

In liver disease which coag factor is elevated?

Back 50

PT and PTT

Front 51

How to differentiate chronic DIC from Liver disease on Step 2?

Back 51

D-Dimer markedly elevated in DIC

Front 52

Triad of Hemolytic Uremic Syndrome (HUS).

Back 52

Microangiopathic Hemolytic Anemia
Renal Failure
Low Platelets

Front 53

TTP pentad

Back 53

Low Platelets
Microangiopathic Hemolytic Anemia
Renal Failure
Neurologic Signs
Fever

Front 54

What is HELLP syndrome?

Back 54

Hemolytic anemia
Elevated Liver enzymes
Low Platelets

Front 55

Key determinant of calling it TTP vs HUS on lab findings.

Back 55

More Severe Renal Failure is more likely HUS

Front 56

1st DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?

Back 56

3-6 months

Front 57

2nd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?

Back 57

6-12 months

Front 58

3rd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?

Back 58

Lifetime

Front 59

Thrombosis and Hemorrage occur in DIC. What is the mechanism of the clotting?

Back 59

deposition of fribrin in small vessels

Front 60

Thrombosis and Hemorrage occur in DIC. What is the mechanism of the bleeding?

Back 60

depletion of clotting factors

Front 61

Hospital inpatient developes petechiae, then starts oozing blood from IV site; high D-dimer. Diagnosis?

Back 61

DIC

Front 62

Petichea suggest deficiency of what?

Back 62

platelets

Front 63

Bleeding into boy cavities or joints suggests deficiency of what?

Back 63

clotting factor/s

Front 64

When to give platelets to a patient with DIC.

Back 64

If 1) bleeding and 2) plts less than 20,000

Front 65

How to differentiate DIC from liver disease?

Back 65

VIII is not decreased in liver dx, but is depleted in DIC

Front 66

Key determinant of calling it TTP vs HUS in history.

Back 66

HUS associated with recent/current diarrhea; also it's most commonly called HUS in kids

Front 67

HSP differential from TTP in patient with purpura and renal failure.

Back 67

HSP has normal plts and NO hemolytic anemia

Front 68

1st Diagnostic Step in TTP

Back 68

peripheral smear

Front 69

Appearance Microangiopathic Hemolytic anemia on peripherial smear?

Back 69

schistocytes

Front 70

How to differentiate TTP from DIC (or Liver disease)?

Back 70

clotting factors are normal in TTP

Front 71

3 blood labs abnormalities that indicate hemolysis.

Back 71

high LDH, low Haptoglobin, high indirect bili

Front 72

When to give platelets in TTP.

Back 72

Never on step 2; can give if life-threatening bleeding

Front 73

1st line treatment in TTP or HUS.

Back 73

plasmapheresis

Front 74

If patient with TTP or HUS is not responding to daily plasmapheresis, what is tx?

Back 74

plasmapheresis BID and add corticosteroids

Front 75

Most common reason for low platelets in women of childbearing age?

Back 75

ITP

Front 76

Typical age and sex of Chronic ITP patient.

Back 76

20-40 and female

Front 77

Typical age and sex of Acute ITP patient.

Back 77

2-6 (male=female)

Front 78

Presentation of Chronic ITP

Back 78

Asymptomatic with incidental thrombocytopenia

Front 79

Pathophysiologic mechanism of platelet destruction in ITP.

Back 79

IgG antibodies formed against platelets

Front 80

Presentation of Acute ITP.

Back 80

abrupt hemorrhage complications following a viral illness

Front 81

Prognosis: acute vs chronic ITP

Back 81

acute onset ITP: spontaneous remission
insidious onset ITP: remains chronic

Front 82

What is the threshold for starting treatment (steroids +/- IVIG) in patient with ITP.

Back 82

if plts are less than 20,000

Front 83

1st line treatment of ITP.

Back 83

Steroids: Prednison or Decadron

Front 84

When to try anti-D (Rh) immunoglobin in patient with ITP.

Back 84

if Steroids fail to raise plts above 20-30

Front 85

Steroids, IVIG, anti-D all fail to raise plts above 20 in ITP patient. Next step in management?

Back 85

immunize, then splenectomy

Front 86

In pregnant patients who develop auto-immune thrombocytopenia, what happens in the fetus?

Back 86

also develops thrombocytopenia

Front 87

2 urine abnormalities in hemolytic anemia.

Back 87

Hemoglobin (once haptoglobin is all occupied)
Hemosiderin (in sloughed tubular cells)

Front 88

What cell can differentiate into a macrophage?

Back 88

Monocyte

Front 89

What cell produces platetets?

Back 89

Megakaryocyte

Front 90

What type of cell is also called a plasma cell?

Back 90

B-Cell

Front 91

What cell is a nucleated form of a red blood cell?

Back 91

reticulocyte

Front 92

PMNs are given their name by what cytologic feature?

Back 92

PolyMorphic nature of the Nucleus

Front 93

Lymphoblast gives rise to what 2 cell types.

Back 93

B and T cells

Front 94

Most common cause of anemia

Back 94

Iron Deficiency

Front 95

Most common cause of iron deficiency

Back 95

GIB

Front 96

Most common cause of iron deficiency in toddlers.

Back 96

insufficient dietary intake for growth

Front 97

Most common cause of iron deficiency in child-bearing aged women

Back 97

menses

Front 98

Mechanism of iron deficiency in pregnant pt.

Back 98

insufficient dietary intake for growth

Front 99

1st step in approach to anemia.

Back 99

MCV (micro, normo, or macrocytic)

Front 100

MCV in iron deficiency.

Back 100

low (microcytic)

Front 101

Tests to order if macrocytic anemia.

Back 101

B12 and Folate

Front 102

Anemia in elderly individual. Diagnosis?

Back 102

Colon Cancer until proven otherwise

Front 103

Anemic patient craving things like ice and dirt. What is this called?

Back 103

Pica

Front 104

fingernails in iron deficiency (especially kids).

Back 104

koilonychia (spoon nails)

Front 105

Causes of microcytic anemia (TICS mnemonic)

Back 105

Thalassemia
Iron Deficiency
Chronic Disease
Sideroblastic

Front 106

Fe defic. vs Anemia of Chronic Disease: most important component of iron studies.

Back 106

ACD: TIBC up
Fe defic: TIBC down

Front 107

Retic count in Fe defic.

Back 107

low

Front 108

Ferritin is high in patient with Fe defic. Why?

Back 108

acute phase reactant

Front 109

Common cause of Fe Defic especially in Africa.

Back 109

Hookworm causing occult bleeding

Front 110

Most common cause of B12 deficiency

Back 110

pernicious anemia

Front 111

What causes pernicious anemia?

Back 111

destruction of parietal cells reduces secrection of intrinsic factor which means reduced B12 absorption

Front 112

Besides Folate or B12 deficiency, these types of drugs which interfere with DNA synthesis also can cause macrocytic anemia.

Back 112

Chemotherapy meds

Front 113

Most common cause of Folate Deficiency.

Back 113

Poor dietary intake

Front 114

What infection can result in B12 deficiency?

Back 114

Tapeworm (Diphyllobothrium latum)

Front 115

B12 deficiency is a known complication of this surgery.

Back 115

Gastrectomy

Front 116

Demyelinating disorder (subacute combined degeneration of the cord) with neuropsychiatric dysfuntion from deficiency of this vitamin.

Back 116

B12

Front 117

What granulocytes changes in patients with macrocytic anemia?

Back 117

hypersegmented nuclei

Front 118

What is the schilling test? (classic but rarely performed)

Back 118

ingestion of radiolabeled cobalim both with and without intrinsic factor

Front 119

What 2 tests to order in conjunction with B12 and Folate?

Back 119

mehtylmalonic acid (MMA) and homocysteine levels

Front 120

On follow-up, patient with B12 and Folate deficiency now has increased reticulocytes. Why?

Back 120

production is no longer inhibited by B12 and Folate deficiency even though it takes longer for the anemia to correct

Front 121

MCV in hemolyitic anemias.

Back 121

usually normocytic

Front 122

anemia after Fava Beans ingestion. Diagnosis?

Back 122

G6PD deficicney

Front 123

Inheritance of G6PD deficiency.

Back 123

X-linked Recessive

Front 124

Most common cause of hemolytic events in patients with G6PD

Back 124

infection

Front 125

Black soldier in Vietnam developed hemolytic anemia after taking this medication?

Back 125

anti-malarial (quinine)

Front 126

Defect in a membrane cytoskeleton causes RBCs to be round. Diagnosis?

Back 126

Hereditary Spherocytosis

Front 127

What mutation causes Sickle Cell?

Back 127

point mutation in Beta Globin (glu to val)

Front 128

Dark urine secondary to hemolytic anemia. Diagnosis?

Back 128

paroxysmal nocturnal hemoglobinuria

Front 129

Mechanical heart valves can cause this RBC abnormality.

Back 129

hemolysis

Front 130

Autoimmune hemolytic anemia (AIHA) can be induced from these 2 infections.

Back 130

EBV or mycoplasma

Front 131

Reticulocyte count in hemolytic anemia.

Back 131

high

Front 132

Jaundiced patient found to have normocytic anemia. Cause?

Back 132

hemolysis

Front 133

How to test for autoimmune hemolysis?

Back 133

Coombs test

Front 134

Treatment for autoimmune hemolytic anemia.

Back 134

Steroids

Front 135

Supplement to give after hemolytic event?

Back 135

Iron

Front 136

Microangiopathic Hemolitic Anemia on peripheral smear. DDX (4)?

Back 136

HUS
TTP
DIC
HELLP (obstetric complication)

Front 137

Maternal serum tested on test blood at 28 weeks to see if her blood is Rh positive. What type of coombs test?

Back 137

Indirect

Front 138

Rh + baby's blood is tested to see if antibodies from Rh - Mom are bound to his blood. What type of coombs test?

Back 138

Direct

Front 139

Positive Direct vs. Indirect Coombs test. Where are the antibodies located?

Back 139

Direct: antibodies attached to RBCs (attacking)
Indirect: antibodies in Serum (not attacking yet)

Front 140

Positive Direct vs. Indirect Coombs test. Where are the antibodies from?

Back 140

Direct: from self (autoimmune); or from transfusion (from donor or from mother to baby)
Indirect: from self (potential transfusion rxn)

Front 141

Typical CBC in Aplastic Anemia

Back 141

pancytopenic

Front 142

Absent radii, short stature, café-aulait spots. Diagnosis?

Back 142

Fanconi's Anemia

Front 143

Hereditary disorder which causes Aplastic Anemia.

Back 143

Fanconi's Anemia

Front 144

Most common cause of Mortality in Aplastic Anemia.

Back 144

infection (immunocompromised)

Front 145

Sickle Cell Patient with pancytopenia. What caused the Aplastic Crisis?

Back 145

Parvovirus B19

Front 146

CBC shows pancytopenia. Next 2 steps in work-up?

Back 146

peripheral smear then BM bx

Front 147

Treatment of apalstic Anemia. (3 fold)

Back 147

Blood Transfusions
Stem Cell Transplantation
Treat infections aggressively

Front 148

What causes the "functional" asplenia in Sickle Cell disease (SCD)?

Back 148

vaso-occlusive splenic infarction

Front 149

What other common cell on peripheral smear in Sickle Cell patient (besides sickled cells)?

Back 149

Target cells

Front 150

Why do Sickle Cell Disease (SCD) patients have DNA remnants called Howel Jolly bodies found on Peripheral Smear

Back 150

Autosplenectomy

Front 151

What bacteria causes most of the osteomyelitis associated with Sickle Cell Disease (SCD)?

Back 151

Salmonella

Front 152

What is the risk of chronic transfusion therapy (besides transfusion rxn)?

Back 152

iron overload (secondary hemochromotosis)

Front 153

Infectious Disease prophylaxis in Sickle Cell Disease (2)?

Back 153

pneumococcal vaccine
ppx penicillin until age 10

Front 154

3 most common causes of Vaso-Occlusive Crisis in SCD?

Back 154

Dehydration
Infection
Cold temperatures

Front 155

Goal sickle cell variant percentage for treatment of Vaso-Occlusive Crisis in SCD (via blood transfusion)?

Back 155

keep sickle variant under 40%

Front 156

How to prevent acute chest syndrome (e.g. pneumonia) during vaso-occlusive crisis in SCD pt? (3)

Back 156

1) transfusion until sickle variant is less than 40%
2) incentive spirometry +/- O2 supplementation
3) aggressive hydration (IVF)

Front 157

What surgery is common is Sickel Cell Disease (SCD)?

Back 157

cholecystecomy

Front 158

SCD treatment to increase fetal hemoglobin.

Back 158

Hydroxyurea

Front 159

SCD treatment that is contraindicated in pregnancy (teratogenic)

Back 159

Hydroxyurea

Front 160

Hip problem associated with SCD?

Back 160

Avascular necrosis of the hip

Front 161

Patients say this is the most important management of vaso-occlusive crisis (in SCD).

Back 161

pain control

Front 162

Classic race associated with Thalassemia on Step 2 questions.

Back 162

Mediterrainian (Middle Eastern)

Front 163

How to diagnose Sickle Cell Disease (2)

Back 163

1) peripheral blood smear
followed by 2) hemoglobin electrophoresis

Front 164

How to diagnose Thalassemia?

Back 164

hemoglobin electrophoresis

Front 165

Prevention of iron overload in patients who require repetative transfusions.

Back 165

Deferoxamine

Front 166

Thalassemia is beta or alpha based on what?

Back 166

Where the defect is (i.e. beta thalassemia is a defect in beta hemaglobin)

Front 167

Completely absent Beta globin genes. What type of Thalassemia?

Back 167

Beta Thalassemia Major

Front 168

Missing 1 of 2 Beta globin genes. What type of Thalassemia?

Back 168

Beta Thalassemia Minor

Front 169

No Alpha globin genes. What type of Thalassemia?

Back 169

Hydrops Fetalis

Front 170

1 of 4 Alpha globin genes. What type of Thalassemia?

Back 170

Hemoglobin H disease

Front 171

2 of 4 Alpha globin genes. What type of Thalassemia?

Back 171

Alpha Thalassemia Trait

Front 172

3 of 4 Alpha globin genes. What type of Thalassemia?

Back 172

Silent Carrier

Front 173

Microcytic anemia develops at 6 months and requires chronic transfusions. What type of thalassemia?

Back 173

Beta Thalassemia Major (0 of 2 Beta genes)

Front 174

Why do symptoms not show before about 6 months in Beta Thalassemia?

Back 174

Hgb F still around before then

Front 175

Microcytic anemia, but asymptomatic. What type of thalassemia?

Back 175

Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes)

Front 176

Patients die in utero. What type of thalassemia?

Back 176

Hydrops Fetalis (0 of 4 Alpha genes

Front 177

Severe microcytic anemia from birth and requires chronic transfusions. What type of thalassemia?

Back 177

Hemoglobin H disease (1 of 4 Alpha genes)

Front 178

1/3 of patients develop skeletal changes due to increased erythropoiesis. What type of thalassemia?

Back 178

Hemoglobin H disease (1 of 4 Alpha genes)

Front 179

Microcytic anemia, but asymptomatic. What type of thalassemia?

Back 179

Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes)

Front 180

Person has thalassemia but does not know it because is asymptomatic and no anemia present. What type?

Back 180

Silent Carrier (3 of 4 Alpha genes)

Front 181

Why are dehydrated patients often pan-polycythemic?

Back 181

Cells are hemoconcentrated because of decreased plasma volume

Front 182

What is the level of erythropoetin in Polycythemia Vera?

Back 182

low

Front 183

Symptomatic treatment for polycythemia induced hypoxia from hyperviscosity syndrome.

Back 183

phlebotomy

Front 184

Cytoreductive Treatment of Polycythemia Vera (PCV)?

Back 184

Hydroxyurea or Interferon

Front 185

Polycythemia Vera patients are pro-thrombotic, and therefore should be treated with this med.

Back 185

Aspirin

Front 186

Prognosis of Polycythemia Vera (PCV)?

Back 186

7-10 years with treatment

Front 187

Clonal proliferation of a pluripotent marrow stem cell which causes erythrocytosis. Diagnosis?

Back 187

Polycythemia Vera (PCV)

Front 188

Polycythemia found in patient and Erythropoetin (EPO) level is high. Diagnosis?

Back 188

EPO producing tumor

Front 189

Why do Incan natives often have high RBC counts?

Back 189

adaptation to high altitude hypoxia

Front 190

Patient with polycythemia. What 2 tests to order.

Back 190

EPO level (low in PCV)
Imaging (check for neoplasms)

Front 191

2 most common types of blood transfusion reactions.

Back 191

Nonhemolytic febrile reaction
Minor allergic reaction

Front 192

1st step in treatment of a blood transfusion reaction.

Back 192

stop the transfusion!

Front 193

Dramatic hemoglobinuria after hemolytic transfusion reaction may lead to this.

Back 193

acute tubular necrosis and renal failure

Front 194

1-6 hours after transfusion, patient gets fever. Most likely cause?

Back 194

Nonhemolytic febrile reaction

Front 195

Most common cause of hemolytic transfusion reaction.

Back 195

ABO incompatibility from clerical error

Front 196

Anaphylactic reaction to blood transfusion. What immunodeficiency?

Back 196

IgA deficiency (anaphylaxis to donor IgA)

Front 197

Minor allergic reactions to blood transfusions have this symptom and often nothing more.

Back 197

Hives (urticaria)

Front 198

Transfusion reaction with immediate burning at IV site, tachycardia, fever, nausea, flushing, hypotension.

Back 198

Hemolytic reaction

Front 199

Treatment for hemolytic transfusion reaction.

Back 199

1) stop transfusion
2) IVF
3) increase renal blood flow (pressors and diuretics)

Front 200

Treatment of nonhemolytic febrile transfusion reaction?

Back 200

acetominophen

Front 201

Treatment of minor allergic reaction to transfusion

Back 201

antihistamines (diphenhydramine)

Front 202

Abnormality of heme productions that can cause photodermatitis, neuropsychiatric complaints, or colicky abdominal pain. Diagnosis?

Back 202

Porphyria

Front 203

Pink Urine. Diagnosis

Back 203

Porphyria

Front 204

Hemolytic Anemia precipitated by fasting. Diagnosis?

Back 204

Erythropoietic Porphyria

Front 205

Brown urine after a party where pt consumed alcohol and barbiturates. Diagnosis.

Back 205

Erythropoietic Porphyria

Front 206

Treatment for a mild attack of porphyria?

Back 206

high doses of glucose

Front 207

Treatment for severe attak of porphyria (and mechanism)?

Back 207

IV hematin (negative feedback to heme synthetic pathway)

Front 208

Most common porphyria. Presents with skin erythema and blisters (photodermatitis) and no abdominal pain.

Back 208

Porphyria cutanea tarda

Front 209

This blood disorder commonly presents with neuropsych complaints. Often mistaken for Guillain Barre.

Back 209

Porphyria

Front 210

Typical age in Acute Lymphocytic Leukemia (ALL).

Back 210

under 15; "L" for Little people

Front 211

Most common childhood malignancy.

Back 211

Acute Lymphocytic Leukemia (ALL); "L" for Little people

Front 212

Typical age in Acute Myelogenous Leukemia (AML).

Back 212

usually under 40; "M" for middle

Front 213

The 4 oncologic emergencies.

Back 213

Hypercalcemia
Spinal Cord Compresion
Superior Vena Cava Syndrome
Leukostasis

Front 214

ALL presentation.

Back 214

anemia (pallor, fatigue)
thrombocytopenia (petichiae, purpura, bleeding)
medullary expansion (bone pain)

Front 215

Look for this on abdominal exam in patients with Acute Leukemias.

Back 215

hepatosplenomegally

Front 216

Diagnostic test for Acute Leukemias

Back 216

BM aspiration or peripheral blood if blassts are presnt

Front 217

Marrow aspirate criteria which is diagnostic for Leukemia.

Back 217

more than 20% blast cells

Front 218

Auer rod. What specific type of AML?

Back 218

APML (Type M3 AML)

Front 219

Which has scant cytoplasm: Lymphoblast or Myeloblast?

Back 219

Lymphoblast has less cytoplasm

Front 220

Describe CBC in acute leukemias (AML and ALL)

Back 220

anemia
thrombocytopenia
leukocytosis (non-functional); with neutropenia

Front 221

FAB sytem subtypes of ALL?

Back 221

L1, L2, L3

Front 222

FAB sytem subtypes of AML?

Back 222

M0 to M7

Front 223

Which FAB system subtype of AML has the best prognosis? Why?

Back 223

M3, because it responds to all-trans-retinoic acid (ATRA) therapy

Front 224

Mainstay treatment for acute leukemias (AML, ALL).

Back 224

chemotherapy

Front 225

symptomatic treatment for acute leukemias (AML, ALL)

Back 225

transfusions, antibiotics

Front 226

Treatment for AML or ALL refractory to chemo.

Back 226

BM transplant

Front 227

Why give allopurinol to ALL and AML patients?

Back 227

during chemo to prevent tumor lysis syndrome from hyperuricemia and renal insufficiency

Front 228

Risk of this complication in ALL or AML when WBC count is higher than 100,000.

Back 228

Leukostasis

Front 229

Treatment for Leukostasis (blasts occluding the microciruclation causing lung, brain, and bleeding problems).

Back 229

Hydroxyuria +/- leukapharesis to rapidly decrease WBC count

Front 230

Which FAB system subtype of ALL has a poor prognosis due to the association with Burkitt's Lymphoma?

Back 230

L3

Front 231

What percent of ALL patients undergo complete remission with Chemo?

Back 231

85%

Front 232

The most common type of leukemia.

Back 232

Chronic Lymphocytic Leukemia (CLL)

Front 233

Almost all cases of Chronic Lymphocytic Leukemia (CLL) are clonal expansions of this type of cell.

Back 233

Well differentiated B-Lymphocytes

Front 234

Typical age and sex of CLL patients.

Back 234

over 60 y/o and Male (2:1); "CL" for SEEniLe (old people)

Front 235

Typical age of CML patients

Back 235

30-60; "M" for middle

Front 236

Flow cytometry scatter plot shows what antigens in CLL cells?

Back 236

CD5 (T-cell antigen) and CD20 (B-Cell antigen)

Front 237

List the 4 poor prognostic symptoms/signs in the Rai Staging of CLL from bad to worst (LSAT mnemonic)

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0: Lymphocytosis (no treatment at this stage)
I: Lymphadenopathy (ok prognosis; needs chemo)
II: Splenomegaly
III: Anemia
IV: Thrombocytopenia (worst prognosis)

Front 238

When to start Chemo treatment in CLL

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When symptomatic (recurrent infections or lymphadenopathy

Front 239

CLL patient now with high LDH. Dx?

Back 239

Autoimmune Hemolytic Anemia

Front 240

Why do CLL patients sometimes get BM bx even though staging is based on sx?

Back 240

BM bx can help assess fi responding to therapy

Front 241

What other immunodeficiency do CLL patients often develop (besides the Leukocytosis with functional Leukopenia)?

Back 241

Hypogammaglobulinemia

Front 242

Why do lymph nodes and spleen get enlarged with CLL?

Back 242

lymphocyte accumulation

Front 243

Why do CLL patients often get anemia and thrombocytopenia (poorer prognosis)?

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lymphocyte accumulation in the Bone Marrow

Front 244

Is CLL curable?

Back 244

no, but disease-free intervals can be attained

Front 245

What is the name of the BCR-ABL translocation in CML?

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Philadelphia Chromosome t(9,22)

Front 246

Most common presentation of CML.

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asymptomatic: usually found in work-up of
incidental anemia +/- leukocytosis
on routine CBC check

Front 247

Symptoms of progressing CML.

Back 247

typical constitutional cancer sx: weight loss, anorexia

Front 248

3 phases of CML and time in them

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1) Chronic: usually asymptomatic; 5 years, longer if on treatment
2) Accelerated: the transition (days); abrupt increase in basophils and decrease in plts
3) Blast: looks like acute leukemia; survival is 3-6 months

Front 249

Selective inhibitor of BCR-ABL tyrosine kinase.

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Imatinib (Gleevec)

Front 250

Treatment for CML in blast stage (last stage).

Back 250

can try stem cell transplant or chemo; can try a clinical trial or whatever

Front 251

What shows on Peripheral Smear with CML?

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Leukocytosis, granulocytes in all stages of maturation

Front 252

Typical WBC count in patient with CML.

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Very high (100,000-500,000)

Front 253

Typical age and sex in Hairy Cell Leukemia a rare malignancy (2% of adult leukemias).

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HCL is similar to CLL in symptoms and presentation: Older than 55 and Male (5:1); "CL" for SEEniLe

Front 254

Cell type in Hair Cell Leukemia (HCL).

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Well differentiated B-Lymphocytes (again just like CLL)

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CBC in CLL.

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diff shows Lymphocytosis (Lymphs over 5000)

Front 256

What Leukemia is a misonomer?

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HCL (usually leukopenic)

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1st diagnostic step in HCL (sensitivity lacks).

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peripheral blood smear: see Hairy Cells on smear in 85% of HCL patients

Front 258

Confirmatory test in HCL.

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BM bx with TRAP staining

Front 259

What percent of HCL patients have a benign course and never require therapy?

Back 259

10%

Front 260

When to initiate treatment in HCL.

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symptomatic due to cytopenias

Front 261

Treatment in HCL.

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nucleoside anologs (e.g. cladribine)

Front 262

Diagnostic test necessary for Lymphomas.

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Excisional lymph node biopsy

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90% of lymphomas are clonal expansions of this type of cell.

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B-Cell

Front 264

Another name for Chronic Lymphocytic Leukemia.

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Small Lymphocytic Leukemia (SLL)

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Lymphoma grades I-IV (Ann Arbor classification)

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I - one lymph node
II - lymph nodes on one side of the diaphragm
III - lymph nodes on both sides of the diaphragm
IV - Marrow involved

Front 266

4 parts of tumor lysis syndrome (PUCK)

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HyperPhosphatemia
HyperUricemia
HypoCalcemia
HyperKalemia

Front 267

Most common (40%) Lymphoma in childhood (adolescent males)

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T-Cell Precursor Leukemia/Lymphoma

Front 268

Follicular Lymphoma translocation

Back 268

t(14,18)

Front 269

Diffuse Large B-Cell Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma

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high grade, aggressive Non-Hodgkins type; so it gets aggressive therapy

Front 270

MALT gastric lymphoma (5% of lymphomas) is caused by what?

Back 270

H. Pylori

Front 271

Follicular Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma

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Low grade, indolent Non-Hodgkins type; so it gets palliative treatment

Front 272

Treatment for Lymphomas (when symptomatic).

Back 272

Radiation and Chemo

Front 273

Lymphoma Chemo regimine (CHOP).

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Cyclophosphamide (Cytoxan)
Adriamycin (hydroxydaunorubicin)
Oncovin (vincristine)
Prednisone

Front 274

Pancytopenia and Lymphadenopathy. Next steps in work-up?

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Lymph-node excisional biopsy

Front 275

Indolent Lymphomas present how?

Back 275

painless lymphadenopathy
cytopenia
hepatosplenomegaly

Front 276

Aggressive Lymphomas present how?

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fever, night sweats, weight loss
bulky adenopathy
extranodal masses (GI, GU, skin, thryoid, CNS)

Front 277

EBV, HTLV-1, HCV have been known to introduce exogenous genes. They are dubbed "oncogenic viruses" for causing this type of cancers.

Back 277

Lymphoma

Front 278

Hodgkin's Disease (HD) is a monoclonal expansion of this type of cell.

Back 278

B-Cell

Front 279

Usual stage of presentation in Hodgkin's Disease.

Back 279

Stage 2 (usually above the diaphragm)

Front 280

ABVD chemo regimen option for Hodgkin's Disease (HD).

Back 280

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

Front 281

MOPP chemo regimen option for Hodgkin's Disease (HD).

Back 281

Mechlorethamine
Oncovin (vincristine)
Procarbazein
Prednisone

Front 282

Owls-eye cells: Reed-Sternberg Cells (giant abnormal B cells with bilobar nuclei and huge eosinophilic nucleoli). Diagnosis?

Back 282

Hodgkin's Disease

Front 283

Treatment for Hodgkin's Disease.

Back 283

Chemo and Radiation

Front 284

As survival increases for Hodgkin's Lymphoma, this complication of radiation has become a popular concern.

Back 284

radiation induced secondary neoplasms

Front 285

A clonal proliferation of malignant plasma cells; diagnosis?

Back 285

Multiple Myeloma

Front 286

Bone pain at rest. Suspect what?

Back 286

Malignancy

Front 287

Why isn't presence of monoclonal M proteins sufficient to diagnose Multiple Myeloma.

Back 287

MGUS is common; also other lymphoproliferative disorders can express M protein

Front 288

Why are Multiple Myeloma patients prone to infection?

Back 288

overproduction of M protein causes failure to produce other antibodies in sufficient quantity

Front 289

Anemia and bone pain. Suspect what?

Back 289

Multiple Myeloma

Front 290

Triad of diagnosis for Multiple Myeloma

Back 290

over 10% plasma cells in BM (BM bx)
M-protein spike (SPEP or UPEP)
evidence of lytic bone lesions (XRs)

Front 291

Why hypercalcemia in Multiple Myeloma

Back 291

lytic bone processes (osteoclastic activity)

Front 292

Multiple Myeloma treatment

Back 292

prednisone and melphalan etc. (chemo)

Front 293

Multiple Myeloma treatment if refractory to initial chemo

Back 293

Support a more intensive chemo dose with autologous stem cell transplantation

Front 294

Why monitor Multiple Myeloma and MGUS patients with CBC and BMP? (3)

Back 294

checks for specific end-organ damage (anemia, hypercalcemia, renal insufficiency)

Front 295

A LymphoPlasmocytic Lymphoma

Back 295

Waldenstrom's Macroglobulinemia (a NHL)

Front 296

Presentation of Waldenstrom's Macroglobulinemia: insidious or aggressive lymphoma?

Back 296

insidious sx of non-hodgkin's lymphoma

Front 297

Considered a precursor to Multiple Myeloma

Back 297

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Front 298

DDX of Multiple Myeloma: elevated M protein with Raynaud's phenomenon and neuro sx.

Back 298

Waldenstrom's Macroglobulinemia (a NHL)

Front 299

Work-up for Multiple Myeloma with bone marrow biopsy showed Dutcher bodies (PAS+IgM deposits around the nucleus). Diagnosis?

Back 299

Waldenstrom's Macroglobulinemia (a NHL)

Front 300

Tissue is infiltrated by IgM and neoplastic plasma cells. Diagnosis?

Back 300

Waldenstrom's Macroglobulinemia (a NHL)

Front 301

Treatment of Waldenstrom's Macroglobulinemia? (2)

Back 301

plasmapheresis (for high IgM)
chemo (for underlying lymphoma)

Front 302

Extracellular deposits of protein fibrils. Diagnosis?

Back 302

Amyloidosis

Front 303

3 major organs effected by Systemic Amyloidosis.

Back 303

Kidney
Heart
Liver

Front 304

Amyloid deposits limited to the brain. Diagnosis?

Back 304

Alzheimer's disease

Front 305

Congo red staining. Diagnosis?

Back 305

Amyloidosis

Front 306

Amyloid L (AL), overproduction of Monoclonal Light Chain fragments, is associated with what 2 diseases?

Back 306

Multiple Myeloma and Waldenstrom's Macroglobulinemia (a NHL)

Front 307

Multiple Myeloma and Waldenstrom's Macroglobulinemia: which is the most common cause of M-protein monoclonal overproduction?

Back 307

Multiple Myeloma is 30x as common (WM is rare)

Front 308

Treatment for AL, a primary Amyloidosis.

Back 308

Chemotherapy (experimental) to reduce protein burden

Front 309

Treatment for Dialysis related deposition of Beta microglobulin (Amyloidosis).

Back 309

Can try kidney transplant

Front 310

Treatment for Heritable Amyloidosis (e.g. prealbumin deposits).

Back 310

Can try liver transplant

Front 311

Chronic inflammation (e.g. RA) stimulates repetitive production of an acute phase reactant AA. What is AA?

Back 311

a type of Amyloidosis (amyloid A)

Front 312

Neutropenia and hypothermia. Suspect what?

Back 312

Fungemia

Front 313

Neutropenia and fever. Treatment?

Back 313

Broad-spectrum antibiotics immediately

Front 314

Neutropenia with anemia or thrombocytopenia; next step in diagnosis

Back 314

BM bx

Front 315

Neutropenia with oculocutaneous albinism. Diagnosis?

Back 315

Chediak-Higashi syndrome

Front 316

Patient with isolated neutropenia has no known drug exposures, no family history; next step in diagnosis?

Back 316

BM bx

Front 317

Following neutropenia in Chemo patient. What treatment can shorten duration of neutropenia?

Back 317

G-CSF (granulocyte colony stimulating factor)

Front 318

Acute neutropenia. Usual complication.

Back 318

Sepsis

Front 319

Chronic neutropenia (e.g. collagen vascular disease). Usual complications.

Back 319

Mouthish: stomatitis, gingivitis, sinusitis
Anusish: peri-rectal infection

Front 320

Causes of Secondary Eosinophilia (NAACP)

Back 320

Neoplasm
Allergies
Asthma
Collagen vascular disease
Parasites

Front 321

What 2 parts of the WBC diff adds up to Neutrophil Count?

Back 321

Segs and Bands

Front 322

What is the definition of Eosinophilia?

Back 322

over 350 (Eos% x WBC count)

Front 323

Definition of Neutropenia

Back 323

under 1500 (Segs% + Bands% x WBC count)

Front 324

Hematuria and Eosinophilia found after returning from Afria. Diagnosis?

Back 324

likely schistosomyasis

Front 325

Patient with atopic dermatitis found with incidental eosinophilia. Should be questioned about what sx?

Back 325

Asthma sx (sob, etc.)

Front 326

Why take a diet history in patient with Eosinophilia?

Back 326

find possible allergens

Front 327

Coccidioidomycosis or helminth infection will cause eosinophilia in 2 locations. Where?

Back 327

Blood and CSF

Front 328

Treatment of Hypereosinophilic Syndrome (HES), a primary eosinophilia.

Back 328

Corticosteroid and cytotoxic agents

Front 329

What is an autologous transplant?

Back 329

transplant from donor to self (e.g. stem cell)

Front 330

In Allogenic (non-twin) transplants, what needs to be matched?

Back 330

ABO and HLA

Front 331

Timing of the 3 types of transplant rejection.

Back 331

Hyperacute: minutes
Actue: days to months
Chronic: months to years

Front 332

Pathomechanism of the 3 types of transplant rejection.

Back 332

Hyperacute: preformed antibodies
Actue: T-cell mediated
Chronic: chronic immune rxn causing fibrosis

Front 333

Treatment for hyperacute transplant rejection (within minutes).

Back 333

Cytotoxic agents

Front 334

1st line treatment to prevent/treat acute (T-cell mediated) transplant rejection (within months).

Back 334

Short course of high-dose corticosteroids

Front 335

What is the mechanism of Graft Versus Host Disease (GVHD)?

Back 335

donated T-cells attack host tissues

Front 336

Treatment for GVHD

Back 336

high dose corticosteroids

Front 337

Why is it better for Leukemic patients getting bone marrow transplants to get it allogenically instead of autologously?

Back 337

Graft versus host-Leukemia effect where donated T-cells react against patient's leukemic cells

Front 338

5 weeks after transplant, patient develops skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems. Suspect what?

Back 338

Graft Versus Host Disease (GVHD)

Front 339

Triple therapy if short steroid course fails to manage Acute (T-cell mediated) transplant rejection.

Back 339

Corticosteroid
Tacrolimus
Azothioprine

Front 340

Associated Neoplasm: Down Syndrome

Back 340

ALL

Front 341

Associated Neoplasm: Xeroderma Pigmentosum

Back 341

Squamous cell and basal cell carcinomas of the skin

Front 342

Associated Neoplasm: Chronic Atrophic Gastritis

Back 342

Gastric adenocarcinoma

Front 343

Associated Neoplasm: Postsurgical gastric remnants

Back 343

Gastric adenocarcinoma

Front 344

Associated Neoplasm: Pernicous Anemia

Back 344

Gastric adenocarcinoma

Front 345

Associated Neoplasm: Tuberous Sclerosis

Back 345

Astrocytoma and cardiac rhabdomyoma

Front 346

Associated Neoplasm: Actinic Keratosis

Back 346

Squamous Cell carcinoma of the skin

Front 347

Associated Neoplasm: Barrett's Esophagus (GERD)

Back 347

Adenocarcinoma of the Esophagus

Front 348

Associated Neoplasm: Plummer Vinson Syndrome

Back 348

Squamous Cell Carcinoma of the Esophagus

Front 349

Associated Neoplasm: Cirrhosis

Back 349

Hepatocellular Carcinoma

Front 350

Associated Neoplasm: HBV

Back 350

Hepatocellular Carcinoma

Front 351

Associated Neoplasm: HCV

Back 351

Hepatocellular Carcinoma

Front 352

Associated Neoplasm: Ulcerative Colitis

Back 352

Colonic Adenocarcinomar

Front 353

Associated Neoplasm: Paget's Disease of the Bone

Back 353

Secondary osteosarcom and fibrosarcoma

Front 354

Associated Neoplasm: Immunodeficiency

Back 354

Lymphoma

Front 355

Associated Neoplasm: AIDS

Back 355

Aggressive Lymphoma (NHL) and Kaposi's Sarcoma

Front 356

Associated Neoplasm: Myasthenia Gravis

Back 356

Thymomas

Front 357

Associated Neoplasm: Acanthosis Nigricans

Back 357

visceral (stomach, lung, breast, uterus)

Front 358

Associated Neoplasm: Nevus

Back 358

Melanoma