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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
Positive osmotic fragility test | Defect in membrane proteins
Hereditary spherocytosis
26
Back pain Hemoglobinuria a few days later bite cells heinz bodies
G6PD Def. Decreased glutathione RBCs more susceptible to oxitative stress X linked
27
Pyruvate Kinase Deficiency
Hemolytic Anemia in Newborn | defect in pyruvate kinase --> decreased ATP --> rigid RBCs
28
HbC defect
Glutamic acid to LYSINE mutation at residue 6 in ß-globin
29
Hemolytic Anemia pancytipenia venous thrombosis increased compliment mediated RBC lysis
Paroxysmal Nocturnal Hemoglobinuria acquired mutation of hematopoeitic stem cells
30
Paroxysmal Nocturnal Hemoglobinuria Labs
CD55/59 negative RBCs
31
Paroxysmal Nocturnal Hemoglobinuria Tx
Eculizumab
32
Sickle Cell Anemia mutation
substitution of Glutaminc Acid with Valine at position 6 on ß chain
33
Sickle Cell trait
Heterozygote Malaria resistance
34
Virus that cuases aplastic crisis in sickle cell
Parvovirus B19
35
Osteomyelitis in sickle cell
Salmonella
36
Sickle Cell Tx
Hydroxyurea (increased HbF) | Bone marrow transplant
37
Warm and Cold autoimmune hemolytic anemia Ig's
Warm--IgG Cold--IgM (pentamer) Coomb's Positive for both
38
Warm hemolytic anemia associated with
SLE CLL Rx (alpha methyldopa)
39
Cold hemolytic anemia associated with
CLL Mycoplasma pneumoniae EBV (mono)
40
Direct Coomb's test
Anti-Ig added to pts serum
41
Indirect coomb's test
normal RBC's added to pt's serum
42
Porphyria cutanea tarda
Defect in uroporphyrinogen decarboxylase Uroporphyrin accumulates--tea colored urine
43
Acute intermitent porphyria
Porphobilinogen deaminase defect Porphobilinogen, ALA, uroprphyrine accumulate
44
Lead poisoning effect on heme synthesis
Ferrochelatase and ALA dehydratase affected protoporphyrin and ALA accumulate
45
Hemophilia A and B defect
A VIII B IX X linked
46
Hemophilia Sx
bleeding Hemarthroses easy bruising Inc. PTT
47
Defect in plug formation Blood clots but won't stick to wall Decreased Plt count Increased bleeding Time
Bernard-Soulier Syndrome Decreased GpIb --> blood will clot but not adhere to vWF on endothelium
48
Defect in platelet plug formation no plt clumps on blood smear incresed bleeding time normal plts
Glanzmann's Thrombocytopenia Dec. GpIIb/IIIa--defect in plt-plt aggregation
49
Defect in anti-GpIIb/IIIa Ab's decreased Plts increased bleeding time increased megakaryocytes
Idiopathic Thrombocytopenic purpura increased megakaryocytes
50
``` Pentad of renal and neurologic Sx fever thromobocytopenia microangiopathic hemolytic anemia Increased BT Increased LDH Schistocytes ```
Thrombotic Thrombocytopenia purpura
51
Thrombotic Thrombocytopenia purpura Deficiency
ADAMTS 13 --> vWF not degraded
52
DDx HUS with Thrombotic Thrombocytopenia purpura
No neuro Sx with HUS
53
von Willibrand's Disease
Plts can't adhere to endothelium due to decreased vWF (like Bernard Soulier) Intrinsic Pathway defect (normal or inc PTT) Autosomal Dominant
54
von Willibrand's Disease Dx
Ristocetin cofactor assay
55
von Willibrand's Disease Tx
Desmopressin (DDAVP) releases vWF from endothelium
56
DIC
Widespread activation of clotting cascade --> depletes factors increased bleeding time, PT, PTT Decreased plts
57
DIC Causes
``` Sepsis Trauma OB complications acute pancreatitis malignancy nephrotic syndromes Transfusion ``` (STOP Making New Thrombi)
58
DIC Labs
Schistocytes incresed DDQ decreased fibrinogen, factors V and VIII
59
Most common inherited hypercoag in whites
Factor V Leiden Factor V resistant to protein C degradation
60
PT gene mutation
increased PT production --> increased venous clots
61
PTT stops increasing after heparin administration
Inherited antithrombin Deficiency
62
Inability to inactivate factor V and VIII
Protein C and S deficiency
63
Warfarin skin necrosis risk
Protein C and S deficiency
64
Clincal use of FFP
DIC Cirrhosis Warfarin overdose (immediate)
65
Clinical use of Packed RBCs
Acute blood loss | severe anemia
66
Clinical use of Plts.
Stop sig. bleeding
67
Cryoprecipitate use
Coagulation factor deficiencies involving fibrinogen and factor VIII
68
Lymphoid and myeloid neoplasms with wide spread involvement of bone marrow
Leukemia
69
Discrete tumor masses arising from lymph nodes
Lymphoma
70
DDx leukemoid reaction with CML
Both have increased WBC, neutrophils, band cells (left shift) Leukemoid rxn--increased Leukocyte Alkaline Phosphatase CML--decreased LAP
71
Hodgkin's lymphoma associated with what virus
EBV
72
Reed Sternberg cell
CD30 and CD15 binucleate cell associated with hodgkin's lymphoma (owl's eye)
73
Burkitt's Lymphoma
``` NHL young pts Starry Sky pattern t(8:14), c-myc and heavy chain EBV associated ```
74
Diffuse B cell lymphoma
``` #1 adult NHL Old ```
75
Mantle Cell Lymphoma
old men NHL t(11:14) translocation of cyclin D1 and heavy chain Ig poor prognosis CD5+
76
Follicular Lymphoma
Adults NHL t(14:18) bcl-2 and heavy chain Ig bcl-2 inh. apoptosis
77
HTLV-1 (virus) cancer
Adult T cell lymphoma
78
``` monoclonal plasma cell with fried egg appearance Clock Face chromatin high IgG Elderly pt Rouleaux formation ```
Multiple Myeloma #1 elderly cancer of bone
79
Multiple Myeloma associated with
``` 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
Bence Jone's Protein in urine
Multiple Myeloma Ig light chain
81
DDx multiple myeloma from Waldenstrom's Macroglobulinemia
No lytic bone lesions--WM Both have M spike (IgM) causing hyperviscosity Sx
82
Asymptomatic precurser to multiple myeloma M spike monoclonal expansion of plasma cells
MGUS (monoclonal gammoglobinopathy of undetermined significance)
83
``` < 15 years old Mediatinal Mass Increasd lymphoblasts in blood and bone marrow TdT+ CALLA+ Responds well to Tx ```
ALL
84
ALL associated with
Down's Syndrome
85
ALL transposition
t(12:21)--better prognosis t(9:22)--philly chromosome bcr-abl
86
> 60 yrs. Smudge cel in peripheral blood smear increased peripheral lymphocytosis bone marrow involvement
CLL
87
TRAP+ Mature B cell tumor Elderly pt Cells with projections
Hairy Cell Leukemia
88
Hairy Cell leukemia Tx
Cladribine (Adenosine Analogue)
89
Auer Rods > 65 years old increased circulating myeloblasts DIC
AML DIC in M3
90
AML transposition (M3) and Tx
t(15:17) Vitamin A (retinoic acid)
91
Auer rods
Peroxidase positive cytoplasmic inclusion in granulocytes and myeloblasts AML
92
Philidelphia Chromosome
t(9:22) bcr-abl
93
t(8:14)
Burkitt's Lymphoma c-myc
94
t(11:14)
Mantle Cell Lymphoma cyclin D1 activation
95
t(14:18)
Follicular Lymphoma bcl-2 activation
96
t(15:17)
M3 AML Responsice to All trans retinoic acid (Vit. A)
97
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
Langerhans Cell Histiocytosis
98
Birbeck Granules
Tennis racket looking cells in Langerhans Cell Histiocytosis
99
Polycythemia Vera
JAK2 mutation proliferation of stem cells without EPO stimulation Increase of all blood cell types Pt. often itches with hot shower
100
Increased Megakaryocytes and plts. | JAK2 mutation
Essential Thrombocytosis
101
Fibrotic obliteration of bone marrow Tear drop cells (Dacrocytes) Decreased RBCs JAK2 mutation
Myelofibrosis
102
bcr-abl transposition increased cell division without apoptosis Decreased RBC Increased WBC and Plts
CML Philly +
103
Relative Polycythemia
decrease in plasma volume only concentrates cells--increased Hct
104
Appropriate Absolute polycythemia
Increased RBC mass, decreased O2 sat lung disease, heart disease, high altitude
105
Inappropriate Absolute polycythemia
Increase RBC mass | No ∆ plasma V or O2 sat
106
Causes of Inappropriate absolute polycythemia
``` RCC Wilm's Tumor Cyst HCC Hydronephrosis Ectopic EPO ```
107
Polycythemia Vera findings
increased plasma V | greatly increased RBC mass

Pathology 1st aid (11 decks)

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