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Flashcards in Hem Onc Deck (306)
1

What are the platelet receptors for vWF and fibrinogen respectively?

vWF - GPIb. Fibrinogen - GPIIb/IIIa

2

How do reticulocytes show up under Wright-Giemsa stain and why?

Blue due to ribosomal RNA presence

3

What causes the green color in most pus and sputum?

Myeloperoxidase from neutrophils (contains heme)

4

What is the most common cause of hypersegmented PMNs?

Folate deficiency

5

Causes of eosinophilia

NAACP. Neoplastic, Asthma, Allergic process, Collagen vascular disease, Parasites

6

How does induction therapy work?

Prevents allergies by inducing blocking antibody (IgG against allergen) by introducing small amounts of allergen over time. Prevents IgE-mast cell interaction

7

What type of antibodies are anti-AB and anti-Rh respectively?

Anti-AB are IgM, anti-rh are IgG

8

What factor is specifically measured by the PT?

VII

9

What factors does antithrombin inhibit?

7, 9, 10, 11, 12

10

What factors do protein C and S inhibit?

5 and 8 (but not 5 leiden)

11

Between the PT and aPTT which measures the intrinsic pathway and which the extrinsic?

PT measures extrinsic, aPTT measures intrinsic

12

Pathology associated with spur cells (acanthocytes)

Liver disease, abetalipoproteinemia

13

Pathology associated with basophilic stippling

Thalassemias, Anemia of chronic disease, Iron deficiency, Lead poisoning (w/ hypochromic microcytic anemia)

14

Pathology associated with bite cells

G6PD deficiency

15

Pathology associated with schistocytes, helmet cells

DIC, TTP/HUS, Traumatic hemolysis

16

3 causes of DIC

Gram negative sepsis, Acute pancreatitis, Burn injury

17

Common cause of TTP/HUS

E Coli 0157 (ask about a recent diarrheal illness)

18

Pathology associated with teardrop cells

Bone marrow infiltration (eg. myelofibrosis)

19

Pathology associated with target cells

HbC, Asplenia, Liver disease, Thalassemia

20

Pathology associated with Heinz bodies

Alpha-thal, G6PD deficiency. Due to oxidation of iron from ferrous to ferric denaturing hemoglobin

21

Pathology associated with Howell-Jolly bodies

Function hyposplenia or asplenia. Due to basophilic nuclear remnants in RBCs

22

Types of alpha thalassemias

4 gene deletion - Hb Barts (g4) is incompatible with life. 3 gene deletion - HbH disease (b4). 1-2 gene deletion - no significant anemia

23

How do you confirm the diagnosis of b-thal minor?

Increased HbA2 (>3.5 percent) on electrophoresis

24

What is increased in major and minor b-thal?

HbF

25

X-ray finding in b-thal major

Crew cut skull x-ray (marrow expansion). Also Chipmunk facies

26

What heme synthesis enzymes are inhibited by lead?

ALA dehydratase and ferrochelatase

27

Lines on gingivae and epiphyses on x-ray, encephalopathy and erythrocyte basophilic stippling, abdominal colic, sideroblastic anemia, wrist and food drop

Lead poisoning

28

Treatment for lead poisoning

EDTA and Dimercaprol. Succimer for kids

29

Defect and genetics in sideroblastic anemia

X-linked defect in gamma-ALA synthase. Can also be caused by alcohol and lead. Will find increased iron, normal TIBC, increased ferritin

30

Treatment for sideroblastic anemia

Pyridoxine (B6)

31

What leads to thalassemias?

Mutations that cause defective mRNA processing (a mutation 3 bases upstream of start codon interferes w mRNA binding to ribosome)

32

How do you distinguish folate deficiency from B12 deficiency.

B12 will come with neuro signs. Also, normal methylmalonic acid in folate def, but increased in B12 def

33

What compound is found in increased levels in both folate and B12 deficiency?

Homocysteine

34

What are the main spinal cord areas targeted by B12 deficiency?

Posterior columns (vibration/proprio) and lateral corticospinal tract (spasticity)

35

How do you distinguish B12 deficiency from tabes dorsalis?

Lateral corticospinal tract affected in B12 deficiency but not in tabes dorsalis

36

A few drugs that cause nonmegaloblastic macrocytic anemia (3)

5-FU, AZT, hydroxyurea

37

Findings in intravascular hemolysis

Decreased haptoglobin, increased LDH, hemoglobin in urine

38

Findings in extravascular hemolysis

Increased LDH and increased unconjugated bilirubin

39

How do you calculate corrected reticulocyte count?

(Hct / 45) x [Retic Count]. If polychromasia present, divide result by 2

40

What is the main regulator of iron in the body?

Hepcidin

41

Major causes of aplastic anemia

Benzene, chloramphenicol, alkylating agents, antimetabolites, parvo B19, EBV, HIV, HCV, Fanconis anemia, Immune mediated (often follows acute hepatitis)

42

Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

Aplastic anemia

43

What GI related effect does hemolytic anemia predispose to?

Pigmented gallstones

44

What test would you run (besides a CBC) if you suspect spherocytosis and what would be the result?

Osmotic fragility. RBCs will lyse in hypotonic saline

45

Treatment for hereditary spherocytosis

Splenectomy

46

Genetics of G6PD deficiency

X-linked

47

Back pain with hemoglobinuria a few days later. RBCs with heinz bodies and bite cells

G6PD deficiency

48

Cause of intrinsic hemolytic normocytic anemia in newborn (and most common associated finding)

Pyruvate kinase deficiency. Will find splenic hypertrophy from increased work

49

What substitution gives HbC?

Glutamic acid to lysine at position 6

50

Labs and complications of PNH

Increased urine hemosiderin. Complications - thrombosis

51

What substitution causes HbS?

Glutamic acid for valine at position 6

52

X-ray findings of sickle cell anemia

Crew cut

53

What is the immediate effect of an HbS mutation?

Hydrophobic interactions among hemoglobin molecules with HbS are altered

54

What causes autosplenectomy in sickle cell patients?

Vascular occlusion in the spleen

55

Most common complications of sickle cell related to microorganisms

Aplastic crisis (parvo B19) and osteomyelitis (salmonella, followed by s aureus and e coli)

56

How do children with sickle cell often present?

Dactylitis (painful swelling of the hand)

57

How would kidney problems in a sickle cell patient present

Either rapid onset hematuria (renal papillary necrosis) or microhematuria (medullary infarcts)

58

Treatment for sickle cell and how it helps

Hydroxyurea increases HbF. Give oxygen in crises. Last resort is bone marrow transplantation

59

Put the following in order of how far they travel on electrophoresis: HbA, HbC, HbS

(Least far to farthest) HbC, HbS, HbA

60

What composes warm agglutinins and what conditions are they seen in?

IgG. SLE, CLL, alpha-methyldopa and other drugs

61

What composes cold agglutinins and in what conditions are they seen

IgM. CLL, Mycoplasma pneumonia, infectious mono

62

Conditions in which microangiopathic anemia is seen

DIC, TTP-HUS, SLE, malignant hypertension. Will see schistocytes

63

Iron studies in pregnancy/OCP use

Serum iron nl, Transferrin up, Ferritin nl, Transferrin sat down

64

Iron findings in lead poisoning

Serum iron up, transferrin down, ferritin nl, transferrin sat up

65

What gene is mutation in PNH?

PIG-A (a GPI anchor)

66

In what condition will a CD55/CD59 deficiency be seen?

PNH (GPI anchor is necessary for CD55/59 attachment)

67

Hemolytic anemia, hypercoagulability, decreased blood counts

Suspect PNH

68

Headache, memory loss, demyelination

Lead poisoning (adult)

69

Defect in AIP

Porphobilinogen deaminase (aka uroporphyrinogen-I-synthase)

70

What is the rate limiting step in heme synthesis?

ALA synthase (first step). Defective in x-linked sideroblastic anemia

71

Painful abdomen, red-wine colored urine, polyneuropathy, psychologic disturbances, recent medication change.

AIP

72

What is the treatment for AIP?

Glucose and heme (they inhibit ALA synthase)

73

Defect in porphyria cutanea tarda

Uroporphyrinogen decarboxylase

74

Blistering cutaneous photosensitivity

Porphyria cutanea tarda (the most common porphyria)

75

List the substance that accumulates in each of the following - lead poisoning, AIP, porhyria cutanea tarda

Lead poisoning - protoporhyrin (in blood), AIP - porphobilinogen, ALA uroporphyrin (in urine), PCT - uroporphyrin (tea-colored urine)

76

Defect and genetics in Hemophilia A and B

X-recessive. A - 8, B - 9

77

Bleeding studies in hemophilia A and B

Increased PTT, normal PT, normal bleeding time

78

Defect and labs in Bernard-Soulier disease

Defect in platelet plug formation due to decreased GP1b (platelets cant bind vWF). Decreased platelet count, increased bleeding time

79

Defect and labs in Glanzmanns thrombasthenia

Decreased GpIIb/IIIa leads to defect in platelet-to-platelet aggregation. No change in platelet count, increased BT

80

Defect and labs in ITP

Anti-GPIIb/IIIa antibodies (platelet-Ab complex consumed by splenic macrophages). Decreased platelet count, increased bleeding time, increased megakaryocytes

81

Defect and labs in TTP

Deficiency of ADMTS13 leads to decreased degradation of vWF multimers (increased platelet aggregation and thrombosis). Low platelet count, high BT, schistocytes, high LDH

82

Neurologic and renal symptoms, fever, thrombocytopenia, microangiopathic hemolytic anemia

TTP

83

What bleeding abnormalities would be found in patients who have missed their dialysis a while and why?

Very long BT with normal platelet count, PT, and PTT. Uremia causes qualitative platelet d/o

84

vWF disease primarily affects binding of what to what?

Platelets to collagen (due to decreased levels of vWF)

85

Treatment for vWF disease and why

DDAVP. Releases vWF stored in endothelium

86

Labs in vWF disease

PC normal, BT high, PT normal, PTT normal or high

87

Labs in DIC

PC low, BT high, PT high, PTT high

88

Causes of DIC (7)

Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion

89

What is the most common cause of inherited hypercoagulability and how does the defect cause a problem?

Factor V Leiden. It cannot be degraded by protien C

90

What deficiency increases the chance of thrombotic skin necrosis after warfarin administration?

Protein C or S deficiency

91

How do you distinguish leukemia from leukemoid reaction?

High leukocyte alkaline phosphatase in leukemoid reaction

92

Which lymphoma is a localized single group of nodes with contiguous spread?

Hodgkins

93

Which lymphoma includes B symptoms most often

Hodgkins

94

Which lymphoma involves noncontiguous spread?

NHL

95

What is the relationship of Reed-Sternberg cell presence to diagnosis of Hodgkins?

Necessary but not sufficient

96

List the types of Hodgkins lymphoma from best prognosis (also most common) to worst prognosis (also least common)

Nodular sclerosing (most common, best prognosis), Mixed cellularity, Lymphocyte predominant, Lymphocyte depleted (least common, worst prognosis)

97

Starry sky appearance

Burkitts lymphoma. Due to lymphocytes interspersed with macrophages

98

What is the most common NHL and in whom does it usually occur?

Diffuse large B-cell. Usually older adults, but 20 percent in children

99

Mantle cell lymphoma marker and epidemiology

Older males, CD5 positive

100

What parts of the world are most affected by adult t-cell lymphoma and what is the causal agent?

HTLV-1. Japan, West Africa, Caribbean

101

What is the marker with mycosis fungoides/Sezary and how does it present?

CD4+. May present with cutaneous patches/nodules

102

Hypercalcemia, constipation, renal insufficiency, anemia, fatigue, back pain

Multiple myeloma (will also see lytic bone lesions)

103

What will you find in the kidney with multiple myeloma?

Atrophic tubules and eosinophilic casts

104

Bence Jones protein

Ig light chains in the urine. Usually have a Kappa-Kappa configuration

105

Markers in ALL

CALLA and TdT+

106

Mediastinal mass causing respiratory symptoms and SVC syndrome in a child

Consider ALL

107

What yields a better prognosis in ALL?

t(12,21)

108

What lymphoma and leukemia are the same disease and what is the difference between them?

Small Lymphocytic Lymphoma and CLL. CLL has increased peripheral blood lymphocytosis

109

Marker for hairy cell leukemia

TRAP

110

Auer rods

AML (common APL which is M3). Release leads to DIC

111

Treatment for APL (AML M3)

ATRA (vitamin A) induces differentiation of myeloblasts

112

CML translocation (give chrosomes and genes)

t(9,22). BCR-ABL

113

Burkitts translocation (give chrosomes and genes)

t(8,14). C-Myc activation

114

Follicular lymphoma translocation (give chrosomes and genes)

t(14,18). BCL-2 Activation

115

APL (AML M3) translocation (give chrosomes and genes)

t(15,17)

116

Ewings sarcoma translocation (give chrosomes and genes)

t(11,22)

117

Mantle cell lymphoma translocation (give chrosomes and genes)

t(11,14)

118

Markers in Langerhans cell histiocytosis (LCH)

S-100 and CD1a

119

What are JAK2 mutations associated with?

Chronic myeloproliferative disorders

120

What types of cells are increased in polycythemia vera?

RBCs, WBCs and platelets

121

Causes of ectopic erythropoietin (5)

RCC, Wilms tumor, cyst, HCC, hydronephrosis

122

Enoxaparin

Low molecular weight heparin. Act more on Xa, better bioavability, 2-4x longer half life. No monitoring necessary but not easily reversible

123

How does HIT work?

Heparin binds platelet factor IV causing antibody production which binds and activates platelets

124

Lepirudin and Bivalirudin

Direct thrombin inhibitors

125

Which one crosses the placenta, heparin or warfarin?

Warfarin. (Just remember that heparin is natural to the body, so it cant cause problems in pregnancy)

126

Fondaparineux

Pentasaccharide Xa inhibitor

127

Streptokinase

Thrombolytic

128

APSAC (anistreplase)

Thrombolytic

129

What change in the platelet count do thrombolytics cause?

None

130

What do you treat thrombolytic toxicity with?

Aminocaproic acid (an inhibitor of fibrinolysis)

131

What effect does aspirin have on blood studies?

Increased BT, no change in PT or PTT

132

Clopidogrel and Ticlopidine

Irreversibly block ADP receptors and inhibit platelet aggregation. Prevent IIb/IIIa expression.

133

What hematologic drug has a side effect that may present with fever and mouth ulcers?

Ticlopidine (neutropenia)

134

Cilostazol and dipyridamole

PDE III inhibitor (increased cAMP in platelets inhibits aggregation). Also vasodilate

135

Abciximab

Monoclonal antibody to IIb/IIIa receptor

136

Anticancer drugs acting on the S phase

Antimetabolites and etoposide

137

Anticancer drugs acting on the G2 phase

Etoposide and Bleomycin

138

Anticancer drugs acting on the M phase

Vinca alkaloids and taxols

139

What do you give to reverse methotrexate effects?

Leucovorin

140

What do you give to reverse 5-FU effects?

Thymidine (not leucovorin)

141

Cytarabine (ara-C)

Pyrimidine analog (inhibits DNA pol)

142

Dactinomycin

Intercalates DNA. Use in childhood tumors

143

Doxorubicin and daunorubicin

Generates free radicals, intercalates DNA. Use in Hodgkins. Cardiotoxicity (prevent with dexrazoxane), alopecia

144

Bleomycin

Free radical formation, DNA breaks. Pulmonary fibrosis, skin changes

145

Etoposide, teniposide

Inhibits topoisomerase II (causes DNA degradation). Myelosuppression, GI irritation, alopecia

146

Cyclophosphamide and ifosfamide

X-link DNA. Bioactivation by liver. Prevent hemorrhagic cystitis with mesna (binds metabolite)

147

Carmustine, lomustine, semustine, streptozocin

Alkylating agents. GBM. Dizziness, ataxia

148

Busulfan

Alkylating agent. CML. Pulmonary fibrosis, hyperpigmentation

149

Vincristine and Vinblastine

Block MT polymerization. Neurotoxic and paralytic ileus (vincristine), myelosuppression (vinblastine)

150

Paclitaxel (and other taxols)

Hyperstabilize MTs. Ovarian and breast carcinomas. Myelosuppression and hypersensitivity

151

Cisplastin, carboplatin

Alkylating-like agents. Cross link DNA. Nephrotoxic (prevent with amifostine), acoustic nerve damage, chloride diuresis

152

What is the most commonly used glucocorticoid in cancer chemotherapy

Prednisone (may induce apoptosis)

153

Tamoxifen and raloxifene

SERMs. Antagonists in breast and agonists in bone. May increase risk of endometrial ca (tamoxifen)

154

Trastuzumab

Antibody to Her-2. Cardiotoxicity

155

Imatinib

BCR-ABL inhibitor. Fluid retention

156

Most common chemotherapy causing cardiotoxicity

Doxorubicin

157

Most common chemotherapy causing myelosuppression

5-FU, 6-MP and Methotrexate

158

Aput tumors

S-100 positive. Melanoma, small cell of lung, bronchial carcinoid, carcinoid tumor, neuroblastoma

159

Young girl with necrotic mass coming out of her vagina. Vimentin and keratin negative, desmin positive.

Embryonal rhabdomyosarcoma (most common sarcoma of children)

160

What type of tumor tends to be found in the midline?

Teratomas

161

Most common site for lymphomas not in a lymph node

Stomach (H pylori)

162

Most common lymphoma

Follicular B cell

163

What is the presentation of hydatidiform moles and when do they present?

First trimester, signs of preeclampsia (HTN, proteinuria, edema)

164

Which type of hydatidiform mole can progress to cancer and what type of cancer?

Complete (46XX), choriocarcinoma. Partial (69 chromosomes) does not progress to cancer

165

Which vessel in the fetus has the highest O2 concentration?

Umbilical vein

166

What is seen in a hydatidiform mole that is not seen in a choriocarcinoma?

Chorionic villus

167

What hormones does the synctiotrophoblast (or a hydatidiform mole or choriocarcinoma) secrete?

B-hCG and hPL

168

Choristoma

Heterotopic tissue (i.e. pancreatic tissue in the GI tract)

169

How many doubling times does it take to get a tumor that can be detected clinically?

About 30 doublings

170

What types of cancer tend to spread to lymph nodes before going hematogenous?

Carcinomas (sarcomas can go directly hematogenous)

171

What carcinoma breaks the rule and goes directly hematogenous (without stopping in a lymph node first)?

Follicular carcinoma of the thyroid

172

In what organ is primary cancer more common than metastasis?

Kidney (renal adenocarcinoma)

173

What lab abnormality will likely be seen in metastasized prostate cancer?

Increased alkaline phosphate (because prostate carcinoma is blastic to bone)

174

What is seen on EM of a vascular malignancy?

Wibble palad bodies (have vWF in them)

175

Steps in oncogenesis (and define each)

Initiation (mutation), Promotion (multiple copies of mutation are made), Progression (sub-specialization)

176

What type of mutation is responsible for loss of function in all TS genes?

Point mutation

177

Why is hepatocellular carcinoma common in Asia?

Because hepatitis B and aflatoxin (aspergillus) are common

178

What is the cause of the rapidly increasing incidence of primary CNS lymphoma?

HIV

179

Associations with UVA and UVB light

UVA - black light (contributes to skin cancer indirectly, less dangerous), UVB - skin cancers (basal cell, squamous cell, melanoma), UVB - thymidine dimers

180

What metal is associated with skin cancer?

Arsenic

181

Most common cause of white eye reflex

Congenital cataract (CMV, rubella, corticosteroids, Cushings). Rb is notable, but less common

182

What is the basic rule for BCC and SCC?

Upper lip and above is BCC, lower lip and down is SCC

183

What is the only bacterium associated with cancer?

H pylori

184

What is the general rule for colon cancer symptoms?

Left side obstructs, right side bleeds (watch for anemia with right side)

185

What can metastasize to virchows node besides gastric cancer?

Pancreatic

186

Trousseaus sign

Superficial migratory thrombophlebitis due to carcinoma of the head of the pancreas

187

Two common hematologic findings in cancer patients

Hypercoagulability and thrombocytosis

188

Most common cause of fever in cancer patients

Gram negative infection (e coli from indwelling catheter, pseudomonas from respirator, also s aureus from catheter even though its gram positive)

189

What is the most common paraneoplastic syndrome?

Hypercalcemia

190

Cancers that PTHrP

Squamous of the lung and RCC

191

What are two skin signs that if they show up quickly might signal cancer, and which cancer do they signal?

Acanthosis nigricans and seborrheic keratosis. Quick development may signal gastroadenocarcinoma

192

What collagen vascular disease is associated with cancer and which cancers?

Dermatomyositis - leukemia, lymphoma and lung cancer. Can get patches on knuckles (goltrins patches)

193

What endocarditis is associated with cancer and which cancers?

Marantic (nonbacterial thrombotic) endocarditis. Mucuous producing cancers (eg colon). Be careful, they can embolize

194

What tumor can convert calcitonin to amyloid?

Medullary carcinoma of the thyroid

195

What tumors is AFP associated with?

Yolk sac tumor and HCC

196

How can CEA (marker for colon cancer) cause problems outside the colon?

CEA-Ag complexes can deposit in the kidney and cause diffuse membranous glumerulonephritis (a nephrotic syndrome)

197

Most common benign and malignant primary CNS tumors in kids

Benign - cerebellar cystic astrocytoma (most common overall), malignant - medulloblastoma

198

Most common cancers in men and women respectively

Men - prostate, lung, colon. Women - breast, lung, colon

199

Most common infection transmitted by accidental needle stick in the hospital

Hepatitis B

200

How long does it take after anemia develops to get an elevated reticulocyte count?

5-7 days

201

What is the cutoff for an expected reticulocyte count in anemia?

Corrected retic count should be 3 percent or greater

202

What is the most common cause for failure of the Hb and Hct to go up sufficiently after transfusion?

GI bleed (which is most common cause of anemia worldwide)

203

Where are iron, folate and B12 absorbed respectively?

Iron - Duodenum, Folate - jejunum, B12 - terminal ileum

204

What is the most common cause of malabsorption and what areas does it affect?

Celiac sprue. Duodenum and jejunum

205

What finding on a blood smear is an important marker for alcoholism?

Target cells (due to altered cholesterol concentration)

206

What is the relationship between transferrin and TIBC?

They are the same

207

What determines the number of cell divisions an RBC precursor undergoes when developing in the marrow?

Hb concentration

208

4 globins and the 3 normal hemoglobins

Globins - A, B, D, G. HbA is 2A2B, HbA2 is 2A2D, HbF is 2A2G

209

Whats the best way to distinguish between IDA and anemia of chronic disease?

ACD - high ferritin levels, IDA - high TIBC (transferrin)

210

What is the inhibitory neurotransmitter in the spinal cord?

Glycine

211

3 causes of sideroblastic anemias

Alcohol, Vitamin B6 deficiency, Lead poisoning

212

EM findings in alcoholism

Megamitochondria (alcohol is an uncoupling agent and screws up mitochondria)

213

What causes a ringed sideroblast?

Iron that enters the mitochondria but cant get out (because its not properly assembled into heme)

214

Primary populations for alpha thalassemia

Far easterners and blacks

215

What will be the results of Hb electrophoresis in alpha thalassemia?

Normal. Alpha globin is required in all the normal hemoglobins (HbA, HbA2 and HbF) so they are all equally decreased

216

Why is the rate of choriocarcinoma high in the far east?

Because the higher incidence of alpha-thal leads to spontaneous abortion (Hb Barts) which predisposes to choriocarcinoma

217

Treatment for alpha thalassemia

None. Do not give iron

218

Populations in which beta thal is common

Blacks, greeks, italians

219

What will you see on Hb electrophoresis in beta thal?

Decrease in HbA, increase in HbA2 and HbF

220

Most common cause of iron deficiency anemia in a newborn

Bleeding meckels diverticulum

221

What causes cerebral edema in lead poisoning?

Buildup of delta-lemavinylinic acid

222

Common lead poisoning scenarios

Child - eating paint. Adults - automobile shop (batteries), moonshine (made in old radiator), pottery painter (lead based paint)

223

Iron studies in alpha and beta thal

Serum iron - normal, TIBC - high, Saturation - high, Ferritin - high

224

What are the two main jobs of B12?

Transfer methyl group from folate to homocysteine (to make methionine) and helping with odd chain FA metabolism

225

How does B12 deficiency lead to neurologic symptoms?

Succinyl CoA builds up (failure of odd chain FA metabolism) and screws up myelin

226

What drug inhibits intestinal conjugase and what is the effect of this?

Phenytoin. This significantly reduces folate absorption (conjugase has to turn polyglutamate to monoglutamate folate)

227

What two substances inhibit folate absorption?

OCPs and alcohol

228

How many lobes does a PMN have to have to be called hypersegmented?

5 or more

229

Drugs that cause aplastic anemia

Chloramphenical, indomethacin, phenylbutazone, thyroid drugs

230

Do you get hemoglobinuria in intravascular hemolysis, extravascular hemolysis, or both?

Intravascular only

231

Which type of hemolysis more typically produces jaundice, intravascular or extravascular?

Extravascular

232

Most common causes of intrinsic hemolysis

MAD. Membrane defect (spherocytosis, PNH), Abnormal Hb (SC), Deficiency of enzyme (G6PD)

233

In sickle cell disease, what percentage of a cells hemoglobin has to be sickled for the cell to sickle?

60 percent

234

What is the most common cause of death in a child with sickle cell disease

Strep pneumo sepsis

235

At what age does sickle cell disease typically present?

6-9 months

236

Two x linked recessive enzyme deficiences

G6PD and Lesch-Nyhan

237

What damages the RBCs in G6PD deficiency and what blood smear finding does it cause?

Peroxide damages hemoglobin, leading to Heinz bodies (Hb clumped together)

238

What populations is G6PD typically seen in?

Same populations as beta thal - blacks, Greeks, Italians

239

Two drugs most commonly used in G6PD questions

Primaquine and dapsone

240

Which is more common, warm or cold agglutinins and what is the most common cause of it?

Warm. Most common cause is lupus

241

Hypersensitivity reactions to penicillin

Rash - Type 1. Hemolytic anemia - Type 2

242

How does methyldopa lead to hemolytic anemia?

It alters Rh antigens on RBCs and we end up making antibodies against our own Rh antigens

243

How does quinidine lead to hemolytic anemia?

It forms immune complexes with IgM

244

Three main drug induced autoimmune hemolytic anemias and the hypersensitivity type for each

PCN - type 2 (but rash is type 1), Methyldopa - type 2, Quinidine - type 3

245

What is the most common cause of chronic intravascular hemolysis

Aortic stenosis

246

What infection in kids can lead to such high WBC counts that we get concerned it might be ALL?

Pertussis. Viral infections can also do it

247

What do we associated with atypical lymphocytes?

Mononucleosis - EBV

248

What drug is associated with atypical lymphocytes?

Phenytoin

249

What receptor does EBV use to infect B cells?

CD21

250

Generalized painful lymphadenopathy, exudative tonsilitis, very high transaminases, spleen enlargement

EBV mono. Also get jaundice sometimes and spleen can rupture (no contact sports)

251

What does the heterophile antibody test use and what does it test for?

Uses anti-horse or anti-sheep RBC antibodies to test for EBV mono

252

What helminth infection does not cause eosinophilia?

Adult ascariasis (because they stick to the intestine and do not invade)

253

Which leukemia is also a myeloproliferative disease?

CML

254

Most common complication of polycythemia rubivera

Thrombosis (most commonly Budd-Chiari)

255

4 Hs of polycythemia rubivera

Hyperviscosity, Hypovolemia, Histaminemia, Hyperuricemia

256

A patient takes a shower and gets itchy all over his or her body

Polycythemia rubivera (the increased numbers of mast cells and basophils hang out in skin and degranulate more easily)

257

What distinguishes between acute and chronic leukemia

Blasts less than 30 percent is chronic, greater than 30 percent is acute

258

Leukemia in ages 0-14

ALL

259

Leukemia in ages 15-39

AML

260

Leukemia in ages 40-59

AML or CML (separate with bone marrow looking for blasts or philadelphia chromosome)

261

Leukemia in 60 and over

CLL

262

What stain can tell the difference between mature neutrophils and neoplastic neutrophils

Leukocyte Alkaline Phosphatase (LAP). Mature will take it up, neoplastic will not

263

Kid with sternal tenderness, fever, generalized nontender lymphadenopathy, hepatosplenomegaly, normocytic anemia, WBC count 50,000

ALL. Most commonly CD10+, CALLA+. May be associated with Downs

264

Elderly patient, smudge cells, normocytic anemia, hypogammaglobulinemia

CLL. High risk of infection (most common cause of death in CLL)

265

What two tissues are resistant to invasion by cancer cells?

Cartilage and elastic tissue

266

Langerhans cell histiocytosis looks like a tennis raquet on EM. What else has this look on EM?

Clostridium tetani spores

267

Where in the lymph nodes are macrophages usually found?

In the sinuses

268

Reed sternberg cells have an Owls eye appearance. What else has this appearance?

Giardia, CMV, aschoff nodules in rheumatic fever

269

Woman with mass in anterior mediastinum and neck. Fatigue, night sweats, weight loss.

Nodular Sclerosing Hodgkins. Look for RS cells, be sure to rule out TB if she has a cough

270

What do pts with Pompe disease typically die of?

Cardiac failure due to excess deposition of normal glycogen in the heart

271

What do OCPs do that makes them thrombogenic

Increase synthesis of factors 5 and 8, increase synthesis of fibrinogen, inhibit antithrombin 3

272

How does smoking lead to thrombogenesis?

Damages endothelial cells (exposing collagen to the clotting system)

273

Where is vWF made?

Megakaryocytes and endothelial cells

274

What are the effects of PGI2 and TXA2 respectively on blood vessel diameter?

PGI2 is a vasodilator, TXA2 is a vasoconstrictor

275

What is the main vasoconstrictor in Prinzmetals angina?

TXA2

276

Both platelets and mast cells when activated release a preformed chemical then start making chemicals. What are these substances (give the preformed and manufactured for each platelets and mast cells)?

Platelets - preformed is ADP (and others), manufactured is TxA2. Mast cells - preformed is histamine (and 5HT and eosinophil chemotactic factor), manufactured is PGs and leukotrienes

277

Are petechia, echymoses, and epistaxis more characteristic of platelet or coagulation abnormalities?

Platelet

278

What procedure imposes the greatest hemostatic stress on the body?

Wisdom tooth extraction (good question to screen for coagulation disorders)

279

What is the test for vWF disease?

Ristocetin cofactor assay (ristocetin wont cause platelets to clot if missing vWF)

280

Factors of the common final pathway (in order)

10, 5, 2, 1

281

Factors of the intrinsic pathway (in order)

12, 11, 9, 8. Then common final pathway (10, 5, 2, 1)

282

Best test for DIC

D-dimer. Next best is fibrin split products

283

How can you tell the difference between petechia and spider angioma (telangiectasia) on physical exam?

Petechia do not blanch but spiger angiomas will (because its an AV fistula)

284

Kid with epistaxis and spots that do not blanch. Low platelet count

ITP

285

Treatment for ITP

Cotricosteroids if severe, if not severe just leave it alone

286

Two main causes of HUS

E coli O157H7 and Shigella toxin

287

Main differences between vWF disease and Hemophilia A

1) Genetics (hemophilia A is XR and vWF is AD), 2) Number of deficiencies (hemophilia A its only 8 anticoagulant, vWF it is 8 Ag, 8 anticoagulant, and vWF), 3) vWF includes symptoms of platelet dysfunction

288

Why is vWF disease often underdiagnosed in women?

Because OCPs cause increased synthesis of 8 Ag, 8 anticoagulant and vWF (the things deficient in vWF disease), which can mask the disease

289

What is the difference between anti-AB and anti-A/anti-B?

Anti-AB is IgG whereas anti-A and anti-B are IgM

290

Diseases associated with AB blood groups

A - gastric cancer, O - duodenal ulcer

291

What antigen is missing in the black population which makes them less likely to get a malaria and which malaria?

Duffy antigen, reduces chances of getting plasmodium vivax

292

G6PD, thalassemias, and SCD protect from which malaria?

Plasmodium falciparum

293

What is the most common antibody in the US?

Anti-CMV

294

What is the most common infection transmitted by blood transfusion?

CMV

295

What is the most common type of hepatitis picked up from transfusion?

Hep C

296

What causes febrile transfusion reactions?

HLA antibodies against leukocytes in donor blood

297

Who is most at risk for febrile reaction with transfusion?

1) Patients who have been transfused before many times. 2) Women (may have picked up HLA Abs from fetal-maternal bleed)

298

Which Ig is the most potent complement activator?

IgM. It will activate straight C1-C9 and you will get a MAC. Eg. hemolytic transfusion reactions (if wrong blood type given) happen very fast due to IgM

299

What cells are responsible for delayed hemolytic transfusion reactions?

Memory B cells. Antibodies arent detected on cross-match because the memory B cells are dormant. But once they see an Ag on the transfused blood they start making Ab again

300

Woman with a difficult delivery (abruptio placenta) one week ago returns with jaundice, anemia, and uncojugated hyperbilirubinemia

Delayed hemolytic transfusion reaction (assuming she was transfused)

301

If someone has serious surgery and returns with jaundice how can you separate delayed hemolytic transfusion reaction from halothane and from hepatitis?

Delayed hemolytic transfusion - about 1 week, halothane - over a week, hepatitis - 6 to 8 weeks

302

What type of hypersensitivity is involved in delayed hemolytic transfusion reactions?

Type 2

303

Most common cause of jaundice in first 24 hours for a newborn

ABO incompatibility (physiologic jaundice of newborn starts at day 3)

304

If a baby has ABO incompatability, what blood group must the mom be?

O (because anti-AB is the only one that is IgG and thus the only one that can cross the placenta)

305

Which leads to a more serious hemolytic anemia, ABO incompatibility or Rh incompatibility?

Rh

306

What other condition protects against Rh sensitization and how?

ABO incompatability. If fetal blood is both Rh and ABO incompatible and there is maternal fetal bleed at birth the anti-A and anti-B IgM will destroy cells so fast there wont be time for anti-Rh sensitization