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STEP > hematology oncology part 2 > Flashcards

hematology oncology part 2 Flashcards

1
Q

infection in hereditary spherocytosis?

A

Parvovirus B19 leading to aplastic crisis

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2
Q

MCV in spherocytosis?

A

Normal to decreased

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3
Q

spherocytosis diagnosis?

A

Positive osmotic fragility test.

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4
Q

hemolysis type in G6PD?

A

extravascular and intravascular

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5
Q

G6PD presentation

A

Back pain + hemoglobinuria a few days following oxidant stress.

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6
Q

pyruvate kinase deficiency from codebook

A
  • Coded character: Piratte swinging around bathroom + /pyruvate kinase deficiency. Tyrion pissing on the baby/autosomal recessive. Solid, rigid RBC on the floor/defect in pyruvate kinase causes decreased ATP production, leading to rigid RBCs. Baby bleeding out in the sink/presentation = hemolytic anemia in a newborn. /Pyruvate kinase requires all the same cofactors as alpha-ketoacid dehydrogenase (TLCFD).
  • Location: BG first floor bathroom
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7
Q

pyruvate kinase pathophys

A

defect in pyruvate kinase leads to decreased ATP leading to rigid RBCs, leading to extravascular hemolysis

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8
Q

classic pyruvate kinase presentation

A

hemolytic anemia in a newborn

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9
Q

Don’t confuse HbS with HbC

A

ok

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10
Q

hemolysis type in HbC

A

extravascular

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11
Q

HbC on labs (homozygote)

A

hemoglobin crystals inside RBCs and target cells

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12
Q

One disease smoking is protective for?

A

ulcerative colitis

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13
Q

etiology of PNH

A

Acquired mutation in a hematopoietic stem cell.

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14
Q

cancer and PNH?

A

PNH patients are at increased risk of acute leukemias

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15
Q

PNH triad

A

Coombs negative hemolytic anemia + pancytopenia + venous thrombosis.

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16
Q

treatment for PNH?

A

eculizumab

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17
Q

eculizumab MOA

A

terminal complement inhibitor

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18
Q

sickle cell etiology

A

point mutation causing replacement of glutamic acid with valine in beta chain

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19
Q

hemolysis type in sickle cell anemia

A

Extravascular + intravascular hemolysis.

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20
Q

what can precipitate sickling?

A

1) hypoxemia
2) high altitude
3) acidosis
4) dehydration

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21
Q

newborns and sickle cell anemia

A

usually asymptomatic because of increased HbF

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22
Q

epidemiology of sickle cell in AA’s

A

8% of African Americans carry an HbS allele

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23
Q

other thing sickle cell pts are at increased risk for?

A

stroke

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24
Q

Immunoglobulin in warm AIHA?

A

IgG (warm weather is Great)

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25
Q

when is warm AIHA seen?

A

1) Chronic anemia such as SLE and CLL

2) alpha-methyldopa

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26
Q

Immunoglobulin in cold AIHA?

A

IgM and complement (cold weather is Miserable)

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27
Q

when is cold AIHA seen?

A

1) acute anemia triggered by cold
2) CLL
3) mycoplasma infections
4) mononucleosis

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28
Q

pathophys of cold AIHA and cold

A

RBC agglutinates cause painful, blue fingers and toes with cold exposure.

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29
Q

Duodenum supply

A

Supplied proximally by a branch of the common hepatic (gastroduodenal). This is proximal to the 2nd part of the duodenum. Distal to 2nd part is SMA (inferior pancreaticoduodenal). So inferior pancreaticoduodenal supplies 3rd and 4th sections.

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30
Q

Where is collateral circulation in duodenum?

A

Superior and inferior pancreaticoduodenal arteries form an anastomotic loop between the celiac trunk and the SMA.

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31
Q

Anatomic location distinguishing foregut from midgut?

A

2nd part of the duodenum

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32
Q

Coombs positive?

A

AIHA

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33
Q

Direct coombs test

A

Anti-Ig antibody added to patients blood. RBCs agglutinate if RBCs are coated with Ig.

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34
Q

Indirect Coombs test

A

Normal RBCs added to patient’s serum. If serum has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent added.

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35
Q

When is microangiopathic anemia seen?

A

1) DIC
2) TTP/HUS
3) SLE
4) malignant HTN

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36
Q

When is macroangiopathic anemia seen?

A

1) prosthetic heart valves

2) aortic stenosis

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37
Q

macroangiopathic anemia on smear…

A

schistocytes, just like microangiopathic

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38
Q

Intrinsic vs. extrinsic distinction

A

Intrinsic to the RBC (structural defect or error in biochem process). Extrinsic to the RBC

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39
Q

Iron deficiency

1) serum iron
2) transferrin/TIBC
3) ferritin
4) % transferrin saturation (serum iron/TIBC)

A

1) down
2) increased
3) decreased
4) way down

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40
Q

Chronic disease

1) serum iron
2) transferrin/TIBC
3) ferritin
4) % transferrin saturation (serum iron/TIBC)

A

1) decreased
2) decreased
3) increased
4) no change

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41
Q

Pregnancy/OCP use

1) serum iron
2) transferrin/TIBC
3) ferritin
4) % transferrin saturation (serum iron/TIBC)

A

1) no change
2) increased
3) no change
4) decreased

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42
Q

Hemochromatosis:

1) serum iron
2) transferrin/TIBC
3) ferritin
4) % transferrin saturation (serum iron/TIBC)

A

1) increased
2) decreased
3) increased
4) way increased

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43
Q

When do you get severe infections with neutropenia?

A

less than 500 cells

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44
Q

neutropenia definition

A

less than 1500 cells

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45
Q

Causes of neutropenia

A

1) sepsis/postinfection
2) drugs (chemo)
3) aplastic anemia
4) SLE
5) radiation

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46
Q

lymphopenia definition

A

less than 1500 cells in adults; less than 3000 cells in children

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47
Q

Causes of lymphopenia

A

1) HIV
2) DiGeorge
3) SCID
4) SLE
5) corticosteroid
6) radiation
7) sepsis
8) postoperative

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48
Q

eosinopenia definition

A

eosinophils less than 30 cells

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49
Q

Causes of eosinopenia

A

1) Cushing syndrome

2) corticosteroids

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50
Q

Corticosteroids and neutrophils/eosinophils mechanism

A

1) decrease activation of neutrophil adhesion molecules, impairing migration out of the vasculature to sites of inflammation.
2) Sequester eosinophils in lymph nodes and cause apoptosis of lymphocytes.

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51
Q

What is a left shift?

A

A shift to more immature cell in the maturation process

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52
Q

When is a left shift usually seen?

A

With neutrophilic in the acute response to infection or inflammation.

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53
Q

What is a leukoerythroblastic reaction? When is it seen?

A

Left shift with immature RBCs.

1) severe anemia
2) marrow response (fibrosis, tumor taking up space in marrow).

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54
Q

accumulated substrate in lead poisoning

A

1) protoporphyrin

2) delta-ALA

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55
Q

How are adults exposed to lead?

A

batteries, ammunition

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56
Q

lead poisoning presentation in adults

A

Headache + memory loss + demyelination

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57
Q

defective enzyme in AIP?

A

porphobilinogen deaminase

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58
Q

accumulated substrate in AIP?

A

1) porphobilinogen
2) delta-ALA
3) coporphobilinogen (in urine)

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59
Q

what can precipitate AIP?

A

1) CYP-450 inducers
2) alcohol
3) starvation

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60
Q

Treatment for AIP?

A

Glucose and heme, which inhibit ALA synthase

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61
Q

Defective enzyme in porphyria cutanea tarda

A

Uroporphyrinogen decarboxylase

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62
Q

accumulated substrate in porphyria cutanea area?

A

Uroporphyrin (tea-colored urine)

63
Q

Most common porphyria?

A

porphyria cutanea tarda

64
Q

ferrochelatase action + site of action

A

Final step of heme synthesis: protoporphyrin –> heme (incorporation of iron into protoporphyrin)

65
Q

What step does uroporphyrinogen decarboxylase catalyze?

A

uroporphyrinogen III –> coproporphyrinogen III

66
Q

difference between left and right GI

A

Right side is microsatelite instability/mismatch repair pathway.
Left side is more APC pathway.

67
Q

What is protective against progression to GI cancer with APC pathway?

A

aspirin.

68
Q

Heme synthesis pathway

A

FA 395

69
Q

What step does porphobilinogen catalyze?

A

Porphobilinogen –> hydroxymethylbilane

70
Q

What step does delta-aminolevulinic acid dehydratase catalyze?

A

delta-aminolevulinic acid –> porphobilinogen

71
Q

Rate limiting step of heme synthesis?

A

delta-aminolevulinic acid synthase

72
Q

What step does delta-aminolevulinic acid (ALA) synthase catalyze?

A

Glycine + succinyl-CoA –> delta-aminolevulinic acid

73
Q

What regulates ALA synthase?

A

Decreased heme increases ALA synthase activity; increased heme decreases.

74
Q

Why is iron toxic?

A

Cell death due to peroxidation (oxidative degradation of membrane lipids).

75
Q

iron poisoning symptoms

A

Nausea + vomiting + gastric bleeding + lethargy + scarring leading to GI obstruction

76
Q

Extrinsic pathway components

A

I,II,V,VII, and X

77
Q

PT related to

A

extrinsic pathway

78
Q

INR

A

Calculated from PT. 1 = normal, greater than 1 = prolonged.

79
Q

What does PTT test?

A

Function of common and intrinsic pathway (all factors except VII and XIII).

80
Q

hemophilia inheritance

A

A and B are x-linked excessive. **hemophilia C is autosomal recessive.

81
Q

hemophilia A deficiency of…

A

VIII

82
Q

hemophilia B deficiency of…

A

IX

83
Q

hemophilia C deficiency of…

A

XI

84
Q

treatment for hemophilia

A

Desmopressin + factor VIII for A, IX for B, XI, for C

85
Q

Vitamin K deficiency labs

A

Increased PT + increased PTT

86
Q

Bernard-Soulier: PC, BT

A

PC normal or decreased

BT increased

87
Q

Bernard-Soulier mechanism

A

Defect in platelet plug formation. Large platlets.

Decreased GpIb receptor leads to defect in platelet to vWF adhesion.

88
Q

Glanzmann thrombasthenia: PC, BT

A

PC normal

BT increased

89
Q

Glanzmann thrombasthenia mechanism

A

Defect in platelet plug formation.

Decreased GpIIb/IIIa leads to defect in platelet-to-platelet aggregation.

90
Q

Glanzmann thrombasthenia labs

A

no platelet clumping

91
Q

HUS: PC, BT

A

PC decreased

BT increased

92
Q

Difference in presentation between HUS in kids and adults

A

In kids, usually caused by 0157:H7 and presents with diarrhea; in adults no diarrhea and STEC infection not required.

93
Q

HUS treatment

A

plasmapheresis

94
Q

HUS very similar to

A

on the same spectrum as TTP; similar presentation

95
Q

Immune thrombocytopenia: PC, BT

A

Decreased PC, Increased BT

96
Q

Immune thrombocytopenia pathophys

A

Anti-GpIIb/IIIa antibodies lead to splenic macrophage consumption of platelet-antibody complex. Often with viral illness.

97
Q

Immune thrombocytopenia treatment

A

IVIG + splenectomy for refractory ITP

98
Q

TTP: PC, BT

A

Decreased PC, increased BT

99
Q

what is ADAMTS 13?

A

vWF metalloprotease

100
Q

TTP pathophys

A

Inhibition or deficiency of ADAMTS 13 leads to impaired degradation of vWF multimers. Large vWF multimers leads to increased platelet adhesion and increased platelet aggregation and thrombosis.

101
Q

TTP labs

A

1) shistocytes

2) increased LDH

102
Q

TTP treatment

A

Plasmapheresis + steroids

103
Q

TTP symptoms

A

Pentad of neurologic and renal symptoms + fever + thrombocytopenia + microangiopathic hemolytic anemia.

104
Q

vW labs

A

Increased BT

Increased PTT

105
Q

underlying problem in vW

A

defect in platelet to vWF adhesion

106
Q

vW inheritance

A

Autosomal dominant

107
Q

DIC

1) PC
2) BT
3) PT
4) PTT

A

1) decreased
2) increased
3) increased
4) increased

108
Q

DIC labs

A

1) shistocytes
2) Increased fibrin degradation products (D-dimers)
3) decreased fibrinogen
4) decreased factors V and VIII

109
Q

causes of DIC

A 
STOP Making New Thrombi
Sepsis
Trauma
Obstetric complications
Pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
110
Q

Antithrombin deficiency

A

Inherited deficiency of antithrombin. No direct effect on PT, PTT, or thrombin time but diminishes increase in PTT following heparin administration.
Can also be acquired from nephrotic syndrome through loss in urine, which decreases inhibition of factors IIa and Xa.

111
Q

Mutation in factor V leiden

A

Point mutation –> Arg506Gln mutation near cleavage site.

112
Q

protein C or S etiology

A

Decreased ability to inactivate factors Va and VIIIa

113
Q

Common protein C deficiency presentation

A

thrombotic skin necrosis with hemorrhage after administration of warfarin.

114
Q

Prothrombin etiology

A

mutation in 3’ untranslated region leads to increased production of prothrombin and increased plasma levels and venous clots.

115
Q

Transfusion therapy for severe anemia?

A

packed RBCs (to increase hb and O2 carrying capacity)

116
Q

Transfusion therapy for acute blood loss?

A

packed RBCs

117
Q

transfusion therapy for DIC?

A

fresh frozen plasma (in order to increase coagulation factor levels)

118
Q

transfusion therapy for cirrhosis?

A

fresh frozen plasma (in order to increase coagulation factor levels)

119
Q

What is cryoprecipitate?

A

Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin.

120
Q

When is cryoprecipitate used?

A

Coagulation factor deficiencies involving fibrinogen and factor VIII.

121
Q

Electrolyte complications of blood transfusion

A

1) iron overload
2) hypocalcemia (citrate is a Ca2+ chelator)
3) hyperkalemia (RBCs may lyse in old blood units)

122
Q

Leukemia?

A

Lymphoid or myeloid neoplasm with widespread involvement of bone marrow.

123
Q

Where are tumor cells usually found in leukemias?

A

In peripheral blood.

124
Q

Lymphoma?

A

Discrete tumor mass arising from lymph nodes.

125
Q

Lymph node involvement in Hodgkin’s vs. non-Hodgkin’s lymphoma

A

Hodgkin’s –> localized, single group of nodes with contiguous spread.
non-Hodgkin –> multiple lymph nodes involved; extra nodal involvement common; noncontiguous spread.

126
Q

Prognosis in Hodgkin’s?

A

Many have a relatively good prognosis.

127
Q

non-Hodgkins’ epidemiology

A

young adulthood and over 55 years; more common in men except for nodular sclerosing type.

128
Q

Note

A

Don’t assume Hodgkin’s with constitutional B signs/symptoms

129
Q

RS cell markers

A

CD15, CD30

130
Q

Plasma cell description in MM

A

“fried egg” appearance. “clock-face” chromatin and intracytoplasmic inclusions containing immunoglobulin.

131
Q

Immunoglobulin types in MM

A

mostly IgG (55%) but can also be IgA (25%)

132
Q

Most common primary bone tumor in 40-50 age group?

A

multiple myeloma

133
Q

Waldenstrom macroglobulinemia

A

Hyper viscosity syndrome with M spike leading to blurred vision and Raynaud’s but no CRAB findings.

134
Q

Rate of multiple myeloma development with MGUS

A

1-2% per year.

135
Q

myelodysplastic syndromes etiology

A

stem-cell disorders involving ineffective hematopoiesis, leading to defects in cell maturation of all non lymphoid lineages.

136
Q

myelodysplastic syndromes sequela..

A

Can transform to AML

137
Q

Cause of myelodysplastic syndromes

A

1) de novo mutations

2) environmental exposure (eg radiation, benzene, chemo)

138
Q

Common things in leukemias

A

1) anemia (decreased RBCs)
2) infections (decreased mature WBCs)
3) hemorrhage (decreased platelets)
4) lymphocytosis (malignant leukocytes in blood)
So don’t attribute any one of these to one type.

139
Q

Leukemia cutis

A

leukemic cell infiltration of skin

140
Q

Prognosis of ALL in adults

A

BAD, worse than in kids

141
Q

mediastinal mass presenting as SVC syndrome?

A

ALL

142
Q

translocation associated with better prognosis in ALL?

A

T(12:21)

143
Q

Leukemia most responsive to therapy?

A

ALL

144
Q

ALL mets

A

CNS + testes

145
Q

ALL markers

A

TdT marker of pre-T and pre_B cells, CD10 marker of pre-B cells

146
Q

CLL/SLL markers

A

CD20+, CD5+ B-cell neoplasm

147
Q

CLL/SLL presentation

A

often asymptomatic, slow progression.

148
Q

Richter transformation

A

SLL/CLL transformation into an aggressive lymphoma, most commonly DLBCL

149
Q

Dry tap on aspiration…

A

1) hairy cell leukemia

2) myelofibrosis

150
Q

Hairy cell leukemia treatment

A

Cladribine + pentostatin

151
Q

No increase in PTT following heparin administration think…

A

Antithrombin deficiency

152
Q

What are the prothrombotic disorders?

A

1) antithrombin deficiency
2) Factor V Leiden
3) Protein C or S deficiency
4) Prothrombin gene mutation

153
Q

transferrin saturation

A

serum iron/TIBC

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