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Pathology 1st aid > Hem/Onc Path > Flashcards

Hem/Onc Path Flashcards

1
Q

Heinz bodies and bite cells

A

G6PD deficiency

oxidation of Fe from ferrous (2+) to ferric (Fe3+)

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2
Q

Howel Jolly Bodies

A 
Hyposplenia
Mothball ingestion (napthalene)

basophilic nuclear remnants found in RBC

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3
Q

Fe defficiency anemia findings

A

low Fe
high TIBC
low ferratin

Microcytosis and hypochromatic

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4
Q

Alpha Thallessemia

A

Defect in production of alpha Hb

high ß

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5
Q

Hb Barts

A

4 gene deletion of alpha thalassemia
4 gamma Hb
No Alpha
Hydrops fetalis

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6
Q

HbH

A

3 gene deletion alpha thalassemia

very little alpha

ß4 Hb

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7
Q

Alpha thallessemia carrier mutation

A

1 gene mutation of alpha

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8
Q

2 gene mutation alpha thalassemia

A

low Sx

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9
Q

ß thalassemia

A

point mutatio in splce sites and promoter

decreased ß synthesis

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10
Q

ß thalassemia Major

A

Homozygote

ß chain absent –> severe anemia

Marrow expansion–crew cut skull

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11
Q

ß thalassemia minor

A

heterozygote

ß chain underproduced

Dx–HbA2 > 3.5% on electrophoresis

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12
Q

HbF

A

ß thalassemia major

alpha2 gamma 2 Hb

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13
Q 
Burton's lines on gingiva
encephalopathy
erythrocyte basophilic stippling
abd. colic
sideroblastic anemia
wrist drop
A

Lead poisoning

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14
Q

Tx Pb poisoning

A

Dimercaprol and EDTA–adults

Succimer–kids

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15
Q

Hereditary Sideroblastic anemia

A

X linke defect of ALA synthase gene

ringed sideroblasts with iron laden mitochondria

Defect in heme synthesis

labs–increased Fe and Ferratin, normal TIBC

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16
Q

Sideroblastic Anemia Tx

A

Pyridoxine (B6)

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17
Q

Increased homocystein
Normal MMA
Megaloblastic Anemia

A

Folate Deficiency

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18
Q

Increased Homocystein
Increased MMA
megaloblastic anemia

A

Vit B12 def.

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19
Q

Orotic Aciduria

A

Genetic mutation of enzyme that synth uridine from orotic acid

kid with megaloblastic anemia that can’t be cured with B12

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20
Q

Orotic Aciduria Tx

A

uridine monophosphate

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21
Q

non-megaloblastic macrocytic anemia

A

Liver disease
ETOH
reticulocytosis –> inc. MCV
Rx

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22
Q

Anemia of chronic disease

A

incresed hepcidin
dec. Fe and TIBC
Increased Ferratin

normocytic anemia

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23
Q

Fanconi’s Anemia

A

DNA repair defect

Aplastic anemia

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24
Q

Viruses that cause aplastic anemia

A

Parvovirus B19
EBV
HIV
HCV

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25
Q

Positive osmotic fragility test

Defect in membrane proteins

A

Hereditary spherocytosis

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26
Q

Back pain
Hemoglobinuria a few days later
bite cells
heinz bodies

A

G6PD Def.
Decreased glutathione
RBCs more susceptible to oxitative stress

X linked

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27
Q

Pyruvate Kinase Deficiency

A

Hemolytic Anemia in Newborn

defect in pyruvate kinase –> decreased ATP –> rigid RBCs

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28
Q

HbC defect

A

Glutamic acid to LYSINE mutation at residue 6 in ß-globin

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29
Q

Hemolytic Anemia
pancytipenia
venous thrombosis

increased compliment mediated RBC lysis

A

Paroxysmal Nocturnal Hemoglobinuria

acquired mutation of hematopoeitic stem cells

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30
Q

Paroxysmal Nocturnal Hemoglobinuria Labs

A

CD55/59 negative RBCs

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31
Q

Paroxysmal Nocturnal Hemoglobinuria Tx

A

Eculizumab

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32
Q

Sickle Cell Anemia mutation

A

substitution of Glutaminc Acid with Valine at position 6 on ß chain

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33
Q

Sickle Cell trait

A

Heterozygote

Malaria resistance

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34
Q

Virus that cuases aplastic crisis in sickle cell

A

Parvovirus B19

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35
Q

Osteomyelitis in sickle cell

A

Salmonella

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36
Q

Sickle Cell Tx

A

Hydroxyurea (increased HbF)

Bone marrow transplant

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37
Q

Warm and Cold autoimmune hemolytic anemia Ig’s

A

Warm–IgG

Cold–IgM (pentamer)

Coomb’s Positive for both

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38
Q

Warm hemolytic anemia associated with

A

SLE
CLL
Rx (alpha methyldopa)

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39
Q

Cold hemolytic anemia associated with

A

CLL
Mycoplasma pneumoniae
EBV (mono)

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40
Q

Direct Coomb’s test

A

Anti-Ig added to pts serum

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41
Q

Indirect coomb’s test

A

normal RBC’s added to pt’s serum

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42
Q

Porphyria cutanea tarda

A

Defect in uroporphyrinogen decarboxylase

Uroporphyrin accumulates–tea colored urine

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43
Q

Acute intermitent porphyria

A

Porphobilinogen deaminase defect

Porphobilinogen, ALA, uroprphyrine accumulate

44
Q

Lead poisoning effect on heme synthesis

A

Ferrochelatase and ALA dehydratase affected

protoporphyrin and ALA accumulate

45
Q

Hemophilia A and B defect

A

A VIII
B IX

X linked

46
Q

Hemophilia Sx

A

bleeding
Hemarthroses
easy bruising
Inc. PTT

47
Q

Defect in plug formation
Blood clots but won’t stick to wall
Decreased Plt count
Increased bleeding Time

A

Bernard-Soulier Syndrome

Decreased GpIb –> blood will clot but not adhere to vWF on endothelium

48
Q

Defect in platelet plug formation
no plt clumps on blood smear
incresed bleeding time
normal plts

A

Glanzmann’s Thrombocytopenia

Dec. GpIIb/IIIa–defect in plt-plt aggregation

49
Q

Defect in anti-GpIIb/IIIa Ab’s
decreased Plts
increased bleeding time
increased megakaryocytes

A

Idiopathic Thrombocytopenic purpura

increased megakaryocytes

50
Q 
Pentad of renal and neurologic Sx
fever
thromobocytopenia
microangiopathic hemolytic anemia
Increased BT
Increased LDH
Schistocytes
A

Thrombotic Thrombocytopenia purpura

51
Q

Thrombotic Thrombocytopenia purpura Deficiency

A

ADAMTS 13 –> vWF not degraded

52
Q

DDx HUS with Thrombotic Thrombocytopenia purpura

A

No neuro Sx with HUS

53
Q

von Willibrand’s Disease

A

Plts can’t adhere to endothelium due to decreased vWF (like Bernard Soulier)

Intrinsic Pathway defect (normal or inc PTT)

Autosomal Dominant

54
Q

von Willibrand’s Disease Dx

A

Ristocetin cofactor assay

55
Q

von Willibrand’s Disease Tx

A

Desmopressin (DDAVP)

releases vWF from endothelium

56
Q

DIC

A

Widespread activation of clotting cascade –> depletes factors

increased bleeding time, PT, PTT

Decreased plts

57
Q

DIC Causes

A 
Sepsis
Trauma
OB complications
acute pancreatitis
malignancy
nephrotic syndromes
Transfusion

(STOP Making New Thrombi)

58
Q

DIC Labs

A

Schistocytes
incresed DDQ
decreased fibrinogen, factors V and VIII

59
Q

Most common inherited hypercoag in whites

A

Factor V Leiden

Factor V resistant to protein C degradation

60
Q

PT gene mutation

A

increased PT production –> increased venous clots

61
Q

PTT stops increasing after heparin administration

A

Inherited antithrombin Deficiency

62
Q

Inability to inactivate factor V and VIII

A

Protein C and S deficiency

63
Q

Warfarin skin necrosis risk

A

Protein C and S deficiency

64
Q

Clincal use of FFP

A

DIC
Cirrhosis
Warfarin overdose (immediate)

65
Q

Clinical use of Packed RBCs

A

Acute blood loss

severe anemia

66
Q

Clinical use of Plts.

A

Stop sig. bleeding

67
Q

Cryoprecipitate use

A

Coagulation factor deficiencies involving fibrinogen and factor VIII

68
Q

Lymphoid and myeloid neoplasms with wide spread involvement of bone marrow

A

Leukemia

69
Q

Discrete tumor masses arising from lymph nodes

A

Lymphoma

70
Q

DDx leukemoid reaction with CML

A

Both have increased WBC, neutrophils, band cells (left shift)

Leukemoid rxn–increased Leukocyte Alkaline Phosphatase
CML–decreased LAP

71
Q

Hodgkin’s lymphoma associated with what virus

A

EBV

72
Q

Reed Sternberg cell

A

CD30 and CD15

binucleate cell associated with hodgkin’s lymphoma (owl’s eye)

73
Q

Burkitt’s Lymphoma

A 
NHL
young pts
Starry Sky pattern
t(8:14), c-myc and heavy chain
EBV associated
74
Q

Diffuse B cell lymphoma

A 
#1 adult NHL
Old
75
Q

Mantle Cell Lymphoma

A

old men NHL
t(11:14)
translocation of cyclin D1 and heavy chain Ig
poor prognosis CD5+

76
Q

Follicular Lymphoma

A

Adults NHL
t(14:18)
bcl-2 and heavy chain Ig

bcl-2 inh. apoptosis

77
Q

HTLV-1 (virus) cancer

A

Adult T cell lymphoma

78
Q 
monoclonal plasma cell with fried egg appearance
Clock Face chromatin
high IgG 
Elderly pt
Rouleaux formation
A

1 elderly cancer of bone

Multiple Myeloma

79
Q

Multiple Myeloma associated with

A 
Infection increase
1º amyloidosis (AL)
Punched out lytic bone lesions
M spike on protein electrophoresis
Ig light chain in urine (Bence Jone's Protien)
Rouleaux formation
80
Q

Bence Jone’s Protein in urine

A

Multiple Myeloma

Ig light chain

81
Q

DDx multiple myeloma from Waldenstrom’s Macroglobulinemia

A

No lytic bone lesions–WM

Both have M spike (IgM) causing hyperviscosity Sx

82
Q

Asymptomatic precurser to multiple myeloma

M spike

monoclonal expansion of plasma cells

A

MGUS (monoclonal gammoglobinopathy of undetermined significance)

83
Q 
< 15 years old
Mediatinal Mass
Increasd lymphoblasts in blood and bone marrow
TdT+
CALLA+
Responds well to Tx
A

ALL

84
Q

ALL associated with

A

Down’s Syndrome

85
Q

ALL transposition

A

t(12:21)–better prognosis

t(9:22)–philly chromosome bcr-abl

86
Q

> 60 yrs.
Smudge cel in peripheral blood smear
increased peripheral lymphocytosis
bone marrow involvement

A

CLL

87
Q

TRAP+
Mature B cell tumor
Elderly pt
Cells with projections

A

Hairy Cell Leukemia

88
Q

Hairy Cell leukemia Tx

A

Cladribine (Adenosine Analogue)

89
Q

Auer Rods
> 65 years old
increased circulating myeloblasts
DIC

A

AML

DIC in M3

90
Q

AML transposition (M3) and Tx

A

t(15:17)

Vitamin A (retinoic acid)

91
Q

Auer rods

A

Peroxidase positive cytoplasmic inclusion in granulocytes and myeloblasts

AML

92
Q

Philidelphia Chromosome

A

t(9:22)

bcr-abl

93
Q

t(8:14)

A

Burkitt’s Lymphoma

c-myc

94
Q

t(11:14)

A

Mantle Cell Lymphoma

cyclin D1 activation

95
Q

t(14:18)

A

Follicular Lymphoma

bcl-2 activation

96
Q

t(15:17)

A

M3 AML

Responsice to All trans retinoic acid (Vit. A)

97
Q

Child with lytic bone lesions and skin rash
Birbeck Granules
proliferative disorder of dendritic cells
T cells not stimulated
S-100 (neural crest) and CD1a + cells

A

Langerhans Cell Histiocytosis

98
Q

Birbeck Granules

A

Tennis racket looking cells in Langerhans Cell Histiocytosis

99
Q

Polycythemia Vera

A

JAK2 mutation
proliferation of stem cells without EPO stimulation
Increase of all blood cell types

Pt. often itches with hot shower

100
Q

Increased Megakaryocytes and plts.

JAK2 mutation

A

Essential Thrombocytosis

101
Q

Fibrotic obliteration of bone marrow
Tear drop cells (Dacrocytes)
Decreased RBCs
JAK2 mutation

A

Myelofibrosis

102
Q

bcr-abl transposition
increased cell division without apoptosis
Decreased RBC
Increased WBC and Plts

A

CML

Philly +

103
Q

Relative Polycythemia

A

decrease in plasma volume only

concentrates cells–increased Hct

104
Q

Appropriate Absolute polycythemia

A

Increased RBC mass, decreased O2 sat

lung disease, heart disease, high altitude

105
Q

Inappropriate Absolute polycythemia

A

Increase RBC mass

No ∆ plasma V or O2 sat

106
Q

Causes of Inappropriate absolute polycythemia

A 
RCC
Wilm's Tumor
Cyst
HCC
Hydronephrosis
Ectopic EPO
107
Q

Polycythemia Vera findings

A

increased plasma V

greatly increased RBC mass

Pathology 1st aid (11 decks)

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