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Year 5 Pathology > Hematology > Flashcards

Flashcards in Hematology Deck (192):
1

Factors in extrinsic pathway

TF, VII

2

Factors in intrinsic pathway

VIII, IX, XI, XII

3

Sxs of platelet disorders

Bleeding from skin, mucous membranes, petechiae, small superficial ecchymoses.
Immediate bleeding after sx

4

Sxs coagulation disorders

Bleeding into soft tissues, joints, and muscles. Large deep ecchymoses. Delayed bleeding after sx.

5

Platelet disorders resulting from increased consumption

Immune: ITP, drugs
Non-immune: TTp-HUS, DIC

6

Platelet disorders resulting from decreased platelet fn

Inherited: gp Ib mutation; gpIIb/IIIa mutation; storage pool disease
Acquired: Aspirin, uremia

7

Type I VWD

AD. Partial VWF deficiency

8

Type II VWD

AD. Loss multimers-altered quality

9

Type III VWD

AR. Complete deficiency VWF

10

Presentation VWD

Decreased platelet adhesion-->presents as platelet problem.
Increased bleeding time, normal PT, aPTT

11

Tx-VWD

Desmopressin, VIII, VWF concentrates

12

Clotting derangements in Vit K defic

Increased PT and aPTT

13

Clotting derangements in liver disease

Increased PT, aPTT

14

Protein C or S deficiency

AD. Early thrombosis (<40 years)

15

Prothrombin mutation

Increases transcription, therefore more likely to clot

16

Factor V Lieden

Cannot be degraded by protein C
-> Thrombophilia

17

Causes of acquired thrombophilia

Immobility/stasis, tissue trauma (sx), malig, anti-phospholipid syndrome, high estrogen states

18

Classic signs anemia

Sxs hypoxia
Conjunctival pallor, koilonychia, glossitis, angular stomatits, post-cricoid webs, high flow murmur

19

Causes of cellular bone marrow infiltration

Myeloproliferation, myelofibrosis, malignant spread

20

Causes of hypocellular bone marrow infiltration

Idiopathic, cytotoxic drugs +radiation, infxns, AI

21

Elliptocytosis

AD. Mild. No tx

22

ALL presentation

Bone marrow failure, LAD, HSM, testicular infiltration

23

AML presentation

Bone marrow failure, bleeding tendency, GUM/skin/CNS infxns

24

CLL

Mostly B cell
Elderly
Indolent course with good prognosis
Increased lymphocytes
Smudge cells

25

Polycythemia rubra vera-presentation and defect

Elderly
Non-specific sxs: tired, depressed, vertigo, visual disturbances, HTN, angina, intermittent claudication

JAK2 mutation

26

Polycythemia rubra vera-tx

Venesection, hydroxyurea, radioactive 32P

27

Essential thrombocythemia

Benign hx
Decreased quality platelets so actually bleed
Hypercellular marrow
JAK2 mutation
Tx w/ hydroxyurea

28

MGUS

Elderly
Paraprotein present

29

AIP

Psychosis, seizures, abdo pain, polyneuropathy, port wine urine.
AD. Precipitated by stress.
Tx-analgesia, CHO

30

Bacterial infxn transfusion rxn
-Sxs
-Tx

Sxs: Sudden onset fever, v unwell.
Tx: Stop transfusion, broad spectrum abx

31

Viral infxns possible from blood transfusions

Hep B/C, HIV, CMV, HTLV

32

Graft v host disease-sxs

Skin rashes, organ failure, marrow failure, diarrhea

33

Acanthocytosis

Abetalipoproteinemia, liver disease, hyposplenism

34

basophilic stippling

Lead poisoning
Megaloblastic anemia
Myelodysplasia
Liver disease
Hemoglobinopathy

35

Burr cells (echinocytes)

Uremia, GI bleeidng, stomach carcinoma

36

Heinz bodies

G6PD def, chronic liver disease

37

Leukoerythroblastic anemia

Marrow infiltration, i.e. myelofibrosis, malignancy

38

Pelger Huet cells

Hyposegmented neutrophils

39

Polychromasia

Sign of reticulocytes: premature release from BM

40

Right shift

Megaloblastic anemia, uremia, liver disease

41

Rouleaux formation

Chronic inflamm, paraproteinemia, MYELOMA

42

Schistocytes

MIHA e.g. DIC, HUS, TTP, pre-eclampsia

43

Stomatocytes

"Smiling faces" or "fish mouth" RBCs
Hereditary stomatocytosis, high alcohol intake, liver disease

44

Target cells

"HALT"
HbC disease
Asplenia
Liver disease
Thalassemia

45

Hb components

Heme + globin

46

Heme components

Fe + protoporphyrin

47

Microcytosis

MCV

48

Normocytic anemia

MCV 80-100
ACUTE blood loss--first stage IDA
Bone marrow failure
Renal failure
Hypothyroidism
Hemolysis
Pregnancy
F

49

Macrocytic anemia

MCV>100
Alcohol excess
Liver disease
B12 defic, folate defic
Hypothyroidism (can also be normocytic)

50

Anisocytosis

Blood cells of varying size

51

Plummer Vinson syndrome

Anemia
Dysphagia (esoph webs)
Beefy red tongue

52

Common causes IDA

Menorrhagia
Peptic ulcers/gastritis
Polyps/CRC
Meckel's
Hookworm infestation
Dietary deficiency
Malabsorption

53

ACD

Fe sequestered by hepcidin in macrophage
Increased ferritin, decreased TIBC
Decreased Fe, decreased % saturation

Causes:
-Chronic infection (TB, osteomyelitis)
-Vasculitis
-RA
-Malignancy

54

Sideroblastic anemia

Iron loading causing hemosiderosis
Same lab findings as hemochromatosis

Causes: Pb poisoning, B6 deficiency (ALAS cofactor), ALAS defect, myeloprolif or myelodysplastic syndromes

55

In which two causes of anemia is Fe low?

IDA, ACD

56

TIBC and ferritin relationship

Oppose each other, i.e. if TIBC high then ferritin low

57

Ferritin

Only low in IDA
Acute phase protein; increases with inflamm e.g. infection, malignancy

58

Megaloblastic blood film

Hypersegmented (>5) neutrophils, leukopenia, macrocytosis, anemia, thrombocytopenia

59

B12 deficiency

Takes years to develop (large liver stores)
Diet: Strict vegans, boozers
Malabsorption: pernicious anemia, gastrectomy, probs with termina ileum (Crohns, bacterial overgrowth, tropical sprue, diphyllobothrium latum)
Pancreatic insufficiency

60

Lab findings ih hemolytic anemias

Increased: unconj bili, urobilinogen, LDH, reticulocytosis/RDW

Intravascular types have decreased haptoglobin

61

RBC membrane defect disorders resulting in hemolysis

Hereditary spherocytosis
Hereditary elliptocytosis

62

RBC enzyme defect conditions resulting in hemolytic anemias

G6PD def
PK def

63

Non-immune causes hemolytic anemias

Mechanical heart valves
PNH, MAHA
Infections (malaria)
Drugs

64

Hereditary spherocytosis

AD
Spectrin, ankyrin, band 3 defect
Osmotic fragility test
Parvo B19 susceptibility
Pigmented gallstones
Splenomegaly
Tx: splenectomy. Howell-Jolly bodies post-splenectomy

65

Hereditary elliptocytosis

AD
Spectrin or band 4.1 mutations

66

G6PD deficiency

X linked
African, Mediterranean, Middle Eastern
No G6PD=no NADPH (HMP shunt)=reduced glutathione=oxidative injury by H2O2=intravasc hemolysis
Oxidants (drugs-malaria drugs, sulfonamides, aspirin; fava beans; stress) cause rapid anemia and jaundice
Bite cells and Heinz bodies
Enzyme assay 2-3 months after attack
Intarvasc hemolysis: dark urine

67

Pyruvate kinase deficiency

AR
Severe neonatal jaundice, splenomegaly, hemolytic anemia

68

Sickle cell

AR
Glu ->valine position 6 of beta chain
Trait usu asymptomatic (except kidneys)
Manifests around 6mo (HbF as newborn)
Decreased O2 tension causes polymerization and sickling
Get vaso-occlusion and infarction: dactylitis, stroke, acute chest syndrome, renal papillary necrosis
Tx: analgesia, hydration, SHiN vaccines, hydroxyurea

69

Beta thal minor

Asymptomatic carrier, mild anemia

70

Beta thal intermedia

Moderate anemia, splenomegaly, bony deformity, gallstones

71

Beta thal major

Severe anemia, FTT, HSM (extramedullary hematopoiesis), bony deformity.
May have heart failure

72

2 alpha gene deletion

Alpha thal trait
Asymptomatic, mild anemia

73

3 alpha gene deletion

HbH disease
Moderate anemia, splenomegaly

74

4 alpha gene deletion

Hb Barts
Incompatible with life

75

Warm Agglutinin Autoimmune Hemolytic Anemia

IgG
Coombs positive
Cause: mainly idiopathic, may be lymphoma, CLL, SLE, methydopa
Tx: steroids, splenectomy, immunosuppression

76

Cold Agglutinin-Autoimmune Hemolytic Anemia

IgM
Positive Coombs test
Oft has Raynauds
Causes: primarily idiopathic. may be lymphoma, infections (EBV, mycoplasma pneumoniae)
Tx: treat underlying condition, avoid cold, chlorambucil

77

Paroxysmal cold hemoglobinuria

Hb-uria after viral infection (e.g. measles)
Donath-Landsteiner abs attach to RBCs in cold; complement-mediated hemolysis on rewarming

78

Paroxysmal Nocturnal Hemoglobinuria

Acquired loss GPI on RBC
GPI anchors DAF which protects against complement in blood. If you lose GPI, you lose DAF, therefore complement-mediated lysis
Dx: Hamm's test or immunophenotype showing altered GPI

79

Where in gut is iron absorbed?

Duodenum

80

Transferrin

Transports iron in blood.

81

Ferritin

Binds iron in storage
Note that Fe on its own causes ROS damage via Fenton reaction

82

TIBC

Measure of transferrin molecules in blood

83

% saturation

Percentage of transferrin molecules that are bound by Fe (normal is 33%)

84

Most common type of anemia in hospitalized patients

ACD

85

Hepcidin

Sequesters Fe in storage sites by:
1. Limiting transfer of Fe from macros to erythroid precursors
2. Suppressing EPO

86

lab findings in ACD

Increased ferritin, decreased TIBC
Decreased serum Fe and % saturation
Increased FEP

87

Lab findings in IDA

Decreased ferritin, increased TIBC
Decreased serum iron, decreased % saturation
Increased FEP

88

HbF

a2g2

89

HbA

a2b2

90

HbA2

a2delta2

91

Causes macrocytic anemia without megaloblastic change

Booze
Liver disease
Drugs (e.g. 5-FU)

92

Where is folate absorbed?

Jejunum

93

Difference in labs between folate and B12 deficiency

Folate: normal methylmalonic acid
B12: increased methylmalonic acid

Both have high homocysteine (increases risk thrombosis)

94

Corrected retic count

(Retic count falsely elevated in anemia, as normal RBCs gone so retics are overall higher percentage)

Retic count * Hct/45

95

Extravascular hemolysis

Destruction by reticuloendothelial system (macros of spleen, liver, LNs)

Findings:
Anemia, splenomegaly, unconj jaundice, risk bili gallstones
Marrow hyperplasia if corrected retic count >3%

96

Intravascular hemolysis

Hb-emia
Hb-uria
Hemosiderinuria (renal tubular cells pick up Hb and break down to Fe, accumulates and shed with tubular cells)
Decreased haptoglobin

97

Microangiopathic hemolytic anemia (MIHA)

Intravasc hemolysis from VASCULAR cause...RBCs destroyed as pass through circulation.
Caused by microthrombi (TTP-HUS, HIC, HELLP), prosthetic heart valves, aortic stenosis
Schistocytes

98

Fever patterns:
-Falciparum
-Vivax
-Ovale
-malariae

Falcip: every day
Vivax, ovale: tertian (every other day)
Malariae: quartan (every third day)

99

Aplastic anemia

Damage to hematopoietic stem cells (CD34+) cauing pancytopenia with low retic count

Causes: drugs/chemicals, viral infections, AI damage

Biopsy: empty, fatty marrow

100

Primary hemostasis

Formation of platelet plug

101

Secondary hemostasis

Stabilization platelet plug
Coag cascade mediated

102

Mucosal bleeding

Epistaxis (most comm), hemoptysis, GI bleeding, hematuria, menorrhagia
Symptom of platelet abnormality

103

Petechiae vs purpura vs ecchymoses

Petechia: 1-2mm
Purpura: >3mm
Ecchymoses: >1cm

NB petechiae are a sign of thrombocytopenia, not usu seen with qualitative platelet defects (just quantitative)

104

ITP

AI production IgG against platelet antigens, e.g. GpIIb/IIIa
MCC thrombocytopenia in adults
Ab-bound platelets consumed by splenic macros causing thrombocytopenia

Acute: post viral infection or immunization. Kids. Self-limiting
Chronic: primary or secondary. Women of child bearing age

105

TTP-HUS

TTP: ADAMTS13 deficiency
HUS: E. coli O157:H7 verotoxin destroys endothelial cells resulting in platelet microthrombi

"FATRN"
Fever
Anemia (MIHA)
Thrombocytopenia
Renal failure
Neuro sxs

106

Bernard-Soulier

GpIb deficiency--impaired platelet adhesion
Mild thrombocytopenia with enlarged platelets

107

Glanzmann thrombasthenia

GpIIb/IIIa deficiency--impaired platelet aggregation

108

Uremia's effect on platelets

Disrupts adhesion and aggregation of platelets

109

Clinical difference between hemophilia A and B

There is none, you numpty

110

Vitamin K deficiency--causes

Newborn (lack GI colonization)
Long term antibiotic therapy
Malabsorption of fat-soluble vitamins

111

Heparin-Induced Thrombocytopenia

Platelet destruction.
Fragments destroyed platelets may activate others, causing thrombosis

112

DIC

Widespread microthrombi result in ischemia and infarction
Consumption factors and platelets result in bleeding, esp from IV sites and mucosal surfaces

Causes:
Obstetric complications--tissue thromboplastin in amniotic fluid activates coag
Sepsis, esp E. coli and N men
Adenocarcinoma (mucin activates coag)
APML
Rattlesnake bites

113

Lab findings in DIC

Decreased: platelets, fibrinogen, D-dimer
Increased: PT, PTT

114

tPA

Converts plasminogen to plasmin

115

Plasmin

Cleaves fibrin and serum fibrinogen, destroys coag factors, blocks platelet activation

116

How do you distinguish thrombosis from postmortem clot?

Lines of Zahn
Attachment to vessel wall
(Both these found in thrombosis)

117

Mechanisms by which intact endothelium prevents thrombosis

Block exposure subendothelial collagen and underlying tissue factor
Produce PGI2 and NO (vasodilation and inhibition platelet agg)
Secrete heparin-like molecules
Secrete tPA
Secrete thrombomodulin

118

Causes endothelial cell damage :(

Atherosclerosis
Vasculitis
High levels homocysteine

119

Cystathionine beta synthase deficiency

Results in high levels homocysteine and homocystinuria.
Findings: vessel thrombosis, mental retardation, lens dislocation, long slender fingers

120

Protein C or S deficiency

AD
Increased risk warfarin skin necrosis

121

Most common cause inherited hypercoagulable state

Factor V Lieden

122

Prothrombin mutation

Point mutation in thrombin that causes increased gene expresion, therefore more thrombi (pro-thrombin...)

123

ATIII deficiency

Decreases protective effect of heparin-like molecules produced by endothelium

124

Why does estrogen (e.g. OCP) cause increased thrombosis risk?

Increased production coag factors

125

Types of emboli

Thrombo-embolism (MCC)
Fat embolism
Gas embolism
Amniotic fluid embolism

126

Fat embolus

BONE FRACTURES, esp long bones, and soft tissue trauma
Dyspnea and petechiae on skin over chest

127

Gas embolus

Decompression sickness--rapid ascent while diving
Nitrogen gas precipitates out during rapid ascent
Joint and muscle pains (the bends) and resp sxs (the chokes)
Chronic form=Caisson disease. Multifocal ischemic necrosis of bone

May also occur during laparoscopic surgery

128

Amniotic fluid embolism

Amniotic fluid enters maternal circulation during labor/delivery
SOB, neuro sxs, DIC
Squamous cell and keratin debris from fetal skin found in thrombus

Extremely high mortality: only 10% survive

129

Pulmonary embolism

Most often clinically silent due to dual blood supply of lung
Usually small and self-resolves

10% cause infarction: SOB, hemoptysis, pleuritic CP, pleural effusion
Sudden death if saddle embolus
Pulm HTN if chronic emboli that are reorganized over time

130

Systemic embolism

Usu due to thromboembolus
Most commonly arise in L heart

131

factor II

Aka thrombin

132

JAK 2

Non-receptor tyrosine kinase

Mutations found in:
PV
ET
Myelofibrosis
Other Myeloproliferative disorders

133

Acquired causes sideroblastic anemia

Alcoholism, Pb poisoning, B6 deficiency

134

Disorders with increased MCHC

Hereditary spherocytosis
AI anemia

135

Metabisulfite test

Detects both sickle cell and sickle cell trait, i.e. any presence of HbS

136

HbC disse

LYSINE instead glutamic acid HbC crystals

137

PNH-cause

ACQUIRED deficiency GPI

138

GPI

Anchors DAF and MIRL which degrade complement getting too close to RBCs

139

Test for PNH

Look for presence CD55 (aka DAF) on cells. If no CD55, then can assume no GPI

140

MCC death in PNH

Thrombosis, e.g. portal vein
10% go on to develop AML

141

HIT

Heparin destroys platelets. Some of destroyed platelets go on to activate other platelets causing THROMBOSIS
Do not use warfarin--increased incidence skin necrosis

142

Secondary causes DIC

Obstetric complications (amniotic fluid activates tissue thromboplastin), sepsis, adenocarcinoma, APML (Auer rods), rattlesnake bite

143

GpIb-function

Binds vWF

144

GpIIb/IIIa

Platelet AGGREGATION. Uses fibrinogen as linker

145

Petechiae are a sign of what problem?

QUANTITATIVE platelet problem, i. thrombocytopenia. Not seen in qualitative disorders

146

ITP

AI: IgG against platelet antigens (eg. GpIIb/IIIa).
Most common cause thrombocytopenia in kids and adults
Ab-bound platelets consumed by splenic macrophages
Spleen also one making antibody
Acute-kids. Usu post-viral infxn or immunization
Chronic-adult women (may be secondary to SLE)
Tx: steroids (esp if acute), IVIG, splenectomy

147

MIHA

Pathological formation microthrombi in small vessels, which shears RBCs. Use up platelets making microthrombi
TTP-HUS is a major cause

148

TTP-mechanism

Deficiency ADAMTS13--can't degrade vWF multimers. Pile up vWF multimers causes abn platelet adhesion thus microthrombi

149

HUS-mechanism

E. coli O157:H7 vertotoxin damages endotherlial cells causing platelet microthrombi. Also decreases ADAMTS13
Kids + undercooked beef -->dysentery

150

Signs and sxs TTP-HUS

Fever
MIHA
Thrombocytopenia
Renal insufficiency (esp HUS)
CNS abnormalities (esp TTP)

Increased bleeding time, normal PT/PTTtX-ttp-hus

151

Tx-TTP-HUS

Plasmapheresis, 'roids

152

Bernard-Soulier

Genetic gpIb deficiency
Impaired platelet adhesion
Mild thrombocytopenia with enlarged platelets ("Big Suckers")

153

Glanzmann's thrombasthenia

Deficienct gpIIb/IIIa
Impaired platelet aggregation

154

Indications for transfusion

Acute blood loss >30% blood volume
Peri-op/critical care, when Hb

155

Intra operative cell salvage

Collect blood lost during surgery and reinfuse into pt.
NB-washed/filtered so lose coagulation factors and platelets

156

Type of blood used in transfusions for highly immunosuppressed individuals

Irradiated

157

Reaction when use the wrong blood in transfusion

Signs/sxs Intravascular hemolysis
Restless, chest/loin pain, fever, vomiting, flushing, collapse, later--hemoglobinuria

158

Febrile non-hemolytic transfusion reaction

Increase in temp by 1 degree C, chills, rigors
Common before blood leukodepleted, now rare
Stop/slow transfusion, give paracetamol. Can re-start

159

Allergic transfusion reaction

Usually with plasma
Common
Mild urticarial or itchy rash +/- wheeze
Tx with antihistamines

160

Delayed hemolytic transfusion reaction

1-3% pts who have received transfusion
Develop Sb to one of foreign RBC antigens
Complement-mediated phagocytosis
Extravascular hemolysis

161

Anaphylactic reaction to blood transfusion

Hypotensive shock, very breathless, wheeze, laryngeal and facial edema
E.g. Donor eaten peanuts (antigen) and pt has IgE Ab to this antigen already

162

TACO

Transfusion Associated Circulatory Overload
Within 6 hrs
Acute resp distress, tachycardia, increased BP, acute or worsening pulm edema, positive fluid balance

163

TRALI

Transfusion Related Acute Lung INjury
Dry cough, dyspnea, fever, not due to fluid overload or other causes
Donor anti-leukocytic Abs interact with pt's leukocyte antigens causing aggregation. These aggregations get stuck in pulm caps. Neutrophils in aggregates release proteolytic enzymes that cause lung damage

Can prevent by giving virally-activated plasma, and plasma from men

164

Cabot rings

Looped structures in RBCs. Found in megaloblastic anemia

165

Pappenheimer bodies

Iron granules. Foud in sideroblastic anemia, Pb poisoning, hemolytic anemias

166

Prothrombin 20210A mutation

Substitution G to A at position 20210 of prothrombin gene. Increases transcription thus more clotting after vessel damage
5% Caucasian population

167

Post-transfusion purpura

5-9 days after transfusion
Severe thrombocytopenia with bleeding
Tx with IVIG

168

Pencil bodies

Type of elliptocyte
Seen in IDA, thal, PK deficiency

169

Cooley's anemia

Old name for beta thal major

170

Cabot rings

Looped structures in RBCs
Caused by megaloblastic anemia

171

Pappenheimer bodies

Iron granules. Pb poisoning, sideroblastic anemia, hemolytic anemia

172

Buerger's disease

Aka thromboangitis obliterans
Strong relation to smoking
Recurrent arterial and venous thromboses
Corkscrew arteries in upper and lower limbs

173

Protein S

Degrades Factors Va, VIIIa

174

Antithrombin

Inhibits thrombin, factor Xa

175

C-myc

Burkitts

176

Lymphoma with centrocytes, centroblasts

Follicular

177

Bcl-2 associated lymphoma

Follicular

178

Donath-Landsteiner Abs

Paroxysmal cold Hb-uria

179

Non-megaloblastic causes of macrocytic anemia

"RALPH"
Reticulocytosis
Alcohol
Liver disease
Pregnancy?
Hypothyroidism

180

Hemochromatosis-sxs

Hepatomegaly/deranged LFTs
Arthralgia and chondrocalcinosis
Diabetes
DCM
Hypogonadism/impotence
Adrenal insufficiency
Skin color changes (slate grey or bronze)

181

MM-lab findings

Normal ALP
Increased ESP
Increased Ca

182

MGUS

183

Waldenstroms

High IgM
Hyperviscosity and vascular complications

184

Combined polycythemia

Aka "smoker's polycythemia"
Combination decreased plasma volume and decreased O2 (CO displaces) causing an increase in EPO

Be very suspicious if smoker + COPD picture

185

Secondary polycythemia

Increase in EPO or testosterone (anabolic roids)
Ectopic EPO from HCC, RCC, hemangioblastoma, pheo, uterine myomata

186

Primary polycythemia

LOW EPO
E.g. polycythemia vera

187

Non-classical subtype of Hodgkins

Nodular lymphocytic

188

Ann Arbor Staging for Hodgkins

I: Single LN region
II: 2+ LN on SAME SIDE of diaphragm
III: LN on both sides diaphragm
IV: extranodal spread

Add "a" if no B sxs, add "B" if there are B sxs

189

B sxs

Significant unexplained fever
Night sweats
Weight loss >10% in preceding 6/12

190

Imantinib

Tyrosine kinase inhibitor.
Used in CML

191

Absence LONG arm chromo 5

Subtype of myelodysplastic syndrome

192

Absence SHORT arm chromo 5

Cri-du-chat

(They will try to trick you with long v short arm)