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Flashcards in hematology oncology part 2 Deck (153):
1

infection in hereditary spherocytosis?

Parvovirus B19 leading to aplastic crisis

2

MCV in spherocytosis?

Normal to decreased

3

spherocytosis diagnosis?

Positive osmotic fragility test.

4

hemolysis type in G6PD?

extravascular and intravascular

5

G6PD presentation

Back pain + hemoglobinuria a few days following oxidant stress.

6

pyruvate kinase deficiency from codebook

• 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

7

pyruvate kinase pathophys

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

8

classic pyruvate kinase presentation

hemolytic anemia in a newborn

9

Don't confuse HbS with HbC

ok

10

hemolysis type in HbC

extravascular

11

HbC on labs (homozygote)

hemoglobin crystals inside RBCs and target cells

12

One disease smoking is protective for?

ulcerative colitis

13

etiology of PNH

Acquired mutation in a hematopoietic stem cell.

14

cancer and PNH?

PNH patients are at increased risk of acute leukemias

15

PNH triad

Coombs negative hemolytic anemia + pancytopenia + venous thrombosis.

16

treatment for PNH?

eculizumab

17

eculizumab MOA

terminal complement inhibitor

18

sickle cell etiology

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

19

hemolysis type in sickle cell anemia

Extravascular + intravascular hemolysis.

20

what can precipitate sickling?

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

21

newborns and sickle cell anemia

usually asymptomatic because of increased HbF

22

epidemiology of sickle cell in AA's

8% of African Americans carry an HbS allele

23

other thing sickle cell pts are at increased risk for?

stroke

24

Immunoglobulin in warm AIHA?

IgG (warm weather is Great)

25

when is warm AIHA seen?

1) Chronic anemia such as SLE and CLL
2) alpha-methyldopa

26

Immunoglobulin in cold AIHA?

IgM and complement (cold weather is Miserable)

27

when is cold AIHA seen?

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

28

pathophys of cold AIHA and cold

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

29

Duodenum supply

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.

30

Where is collateral circulation in duodenum?

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

31

Anatomic location distinguishing foregut from midgut?

2nd part of the duodenum

32

Coombs positive?

AIHA

33

Direct coombs test

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

34

Indirect Coombs test

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

35

When is microangiopathic anemia seen?

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

36

When is macroangiopathic anemia seen?

1) prosthetic heart valves
2) aortic stenosis

37

macroangiopathic anemia on smear...

schistocytes, just like microangiopathic

38

Intrinsic vs. extrinsic distinction

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

39

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

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

40

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

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

41

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

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

42

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

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

43

When do you get severe infections with neutropenia?

less than 500 cells

44

neutropenia definition

less than 1500 cells

45

Causes of neutropenia

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

46

lymphopenia definition

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

47

Causes of lymphopenia

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

48

eosinopenia definition

eosinophils less than 30 cells

49

Causes of eosinopenia

1) Cushing syndrome
2) corticosteroids

50

Corticosteroids and neutrophils/eosinophils mechanism

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.

51

What is a left shift?

A shift to more immature cell in the maturation process

52

When is a left shift usually seen?

With neutrophilic in the acute response to infection or inflammation.

53

What is a leukoerythroblastic reaction? When is it seen?

Left shift with immature RBCs.
1) severe anemia
2) marrow response (fibrosis, tumor taking up space in marrow).

54

accumulated substrate in lead poisoning

1) protoporphyrin
2) delta-ALA

55

How are adults exposed to lead?

batteries, ammunition

56

lead poisoning presentation in adults

Headache + memory loss + demyelination

57

defective enzyme in AIP?

porphobilinogen deaminase

58

accumulated substrate in AIP?

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

59

what can precipitate AIP?

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

60

Treatment for AIP?

Glucose and heme, which inhibit ALA synthase

61

Defective enzyme in porphyria cutanea tarda

Uroporphyrinogen decarboxylase

62

accumulated substrate in porphyria cutanea area?

Uroporphyrin (tea-colored urine)

63

Most common porphyria?

porphyria cutanea tarda

64

ferrochelatase action + site of action

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

65

What step does uroporphyrinogen decarboxylase catalyze?

uroporphyrinogen III --> coproporphyrinogen III

66

difference between left and right GI

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

67

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

aspirin.

68

Heme synthesis pathway

FA 395

69

What step does porphobilinogen catalyze?

Porphobilinogen --> hydroxymethylbilane

70

What step does delta-aminolevulinic acid dehydratase catalyze?

delta-aminolevulinic acid --> porphobilinogen

71

Rate limiting step of heme synthesis?

delta-aminolevulinic acid synthase

72

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

Glycine + succinyl-CoA --> delta-aminolevulinic acid

73

What regulates ALA synthase?

Decreased heme increases ALA synthase activity; increased heme decreases.

74

Why is iron toxic?

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

75

iron poisoning symptoms

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

76

Extrinsic pathway components

I,II,V,VII, and X

77

PT related to

extrinsic pathway

78

INR

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

79

What does PTT test?

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

80

hemophilia inheritance

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

81

hemophilia A deficiency of...

VIII

82

hemophilia B deficiency of...

IX

83

hemophilia C deficiency of...

XI

84

treatment for hemophilia

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

85

Vitamin K deficiency labs

Increased PT + increased PTT

86

Bernard-Soulier: PC, BT

PC normal or decreased
BT increased

87

Bernard-Soulier mechanism

Defect in platelet plug formation. Large platlets.
Decreased GpIb receptor leads to defect in platelet to vWF adhesion.

88

Glanzmann thrombasthenia: PC, BT

PC normal
BT increased

89

Glanzmann thrombasthenia mechanism

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

90

Glanzmann thrombasthenia labs

no platelet clumping

91

HUS: PC, BT

PC decreased
BT increased

92

Difference in presentation between HUS in kids and adults

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

93

HUS treatment

plasmapheresis

94

HUS very similar to

on the same spectrum as TTP; similar presentation

95

Immune thrombocytopenia: PC, BT

Decreased PC, Increased BT

96

Immune thrombocytopenia pathophys

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

97

Immune thrombocytopenia treatment

IVIG + splenectomy for refractory ITP

98

TTP: PC, BT

Decreased PC, increased BT

99

what is ADAMTS 13?

vWF metalloprotease

100

TTP pathophys

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

TTP labs

1) shistocytes
2) increased LDH

102

TTP treatment

Plasmapheresis + steroids

103

TTP symptoms

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

104

vW labs

Increased BT
Increased PTT

105

underlying problem in vW

defect in platelet to vWF adhesion

106

vW inheritance

Autosomal dominant

107

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

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

108

DIC labs

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

109

causes of DIC

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

110

Antithrombin deficiency

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

Mutation in factor V leiden

Point mutation --> Arg506Gln mutation near cleavage site.

112

protein C or S etiology

Decreased ability to inactivate factors Va and VIIIa

113

Common protein C deficiency presentation

thrombotic skin necrosis with hemorrhage after administration of warfarin.

114

Prothrombin etiology

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

115

Transfusion therapy for severe anemia?

packed RBCs (to increase hb and O2 carrying capacity)

116

Transfusion therapy for acute blood loss?

packed RBCs

117

transfusion therapy for DIC?

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

118

transfusion therapy for cirrhosis?

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

119

What is cryoprecipitate?

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

120

When is cryoprecipitate used?

Coagulation factor deficiencies involving fibrinogen and factor VIII.

121

Electrolyte complications of blood transfusion

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

122

Leukemia?

Lymphoid or myeloid neoplasm with widespread involvement of bone marrow.

123

Where are tumor cells usually found in leukemias?

In peripheral blood.

124

Lymphoma?

Discrete tumor mass arising from lymph nodes.

125

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

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

126

Prognosis in Hodgkin's?

Many have a relatively good prognosis.

127

non-Hodgkins' epidemiology

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

128

Note

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

129

RS cell markers

CD15, CD30

130

Plasma cell description in MM

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

131

Immunoglobulin types in MM

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

132

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

multiple myeloma

133

Waldenstrom macroglobulinemia

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

134

Rate of multiple myeloma development with MGUS

1-2% per year.

135

myelodysplastic syndromes etiology

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

136

myelodysplastic syndromes sequela..

Can transform to AML

137

Cause of myelodysplastic syndromes

1) de novo mutations
2) environmental exposure (eg radiation, benzene, chemo)

138

Common things in leukemias

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

Leukemia cutis

leukemic cell infiltration of skin

140

Prognosis of ALL in adults

BAD, worse than in kids

141

mediastinal mass presenting as SVC syndrome?

ALL

142

translocation associated with better prognosis in ALL?

T(12:21)

143

Leukemia most responsive to therapy?

ALL

144

ALL mets

CNS + testes

145

ALL markers

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

146

CLL/SLL markers

CD20+, CD5+ B-cell neoplasm

147

CLL/SLL presentation

often asymptomatic, slow progression.

148

Richter transformation

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

149

Dry tap on aspiration...

1) hairy cell leukemia
2) myelofibrosis

150

Hairy cell leukemia treatment

Cladribine + pentostatin

151

No increase in PTT following heparin administration think...

Antithrombin deficiency

152

What are the prothrombotic disorders?

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

153

transferrin saturation

serum iron/TIBC