Hemeotology Flashcards
1
Q
What kicks off extrinsic pathway of coagulation cascade?
A
Tissue Factor (from damage) activates Factor 7
2
Q
What kicks off the intrinsic pathway of coag cascade?
A
Factor 12
3
Q
Hemophilia A is deficient in…
A
Factor 8
4
Q
Hemophilia B is deficient in…
A
Factor 9
5
Q
Accelerating factors?
A
Factors 5 and 8
6
Q
What cation is essentialnfor the coagulation cascade?
A
Ca+
7
Q
What molecule breaks down fibrin mesh?
A
Plasmin
8
Q
Functions of kallikrein
A
Converts plasminogen to plasmin
9
Q
PT
A
Prothrombin Time
**test how effectively tissue factor activates
Extrinsic and common pathway
Always reported as an INR
10
Q
PTT
A
Partial thromboplastin time (PTT)
Intrinsic and common pathway
11
Q
Bleeding time
A
Measuring platelet function
12
Q
Lab results of hemophilia
A
Increase PTT
**NO EFFECT on PT/INR
13
Q
Normal bleeding time but increased PT and PTT
A
Vitamin K deficiency
Co factor for carboxylation of factor 10, 9, 7, 2, C and S
Inadequate Vit. K intake
Newborns
Warfarin
End-stage liver disease
14
Q
Most common cause of hypercoaguability in whites?
A
Factor V Leiden = Mutant factor 5 that is resistant to degradation by activated protein C
15
Q
Heparin reversal
A
Protamine sulfate
16
Q
Warfarin reversal
A
Oral or IV Vitamin K (takes days)
Fresh frozen plasma, if unstable
17
Q
Preferred anticoagulant for immediate anticoagulation?
A
Heparin
18
Q
Preferred anticoagulant for long term?
A
Warfarin
19
Q
Preferred anticoagulant during pregnancy?
A
HEPARIN!!
20
Q
Anisocytosis
A
RBCs of varying sizes
21
Q
Poikilocytosis
A
RBCs of varying shapes
22
Q
Too many RBCs
A
Polycythemia/ erythrocytosis
23
Q
Immature erythrocytes in circulation?
A
Reticulocytes
24
Q
Basophilic stippling
A
clumps of denatured RNA
Lead Poisoning
25
Acanthocyte "spur cell"
Irregular spikes
Abetalipoproteinemia
Liver Disease
26
Schistocytes
Chopped up parts of RBC, pathologic intravascular clotting
27
Target cells
"HALT" said the hunter to its target
Hemoglobin C disease
Asplenia
Liver Disease
Thalassemia
28
Howell-Jolly body
Basophilic remnant of nucleus in red cell
Asplenic
29
Heinz Body/ Bite Cells
Oxidized hemoglobin = G6PD deficiency
Normally removed from RBCs by splenic macrophages resulting in bite cells
30
Drugs that cause oxidative damage with G6PD deficiency
"Spleen purges nasty inclusions from damaged cells"
```
Sulfonamides
Primaquine
Nitrofurantoin
Isoniazide
Fava beans*****
Dapsone
Cloroquine
```
31
How to differentiate a heinz body from a howell-jolly body?
RBC can only have 1 howell-jolly body (nuclear remnant)
Can have many heinz bodies (oxidized hemoglobin)
32
What allows RBCs to change shape as they pass through vessels?
Spectrin creates biconcave & flexible
33
Immature erythrocytes in circulation
Reticulocytes
34
Where does hematopoesis take place in the embryo?
"Young Liver Synthesizes Blood"
Yolk Sac (3-8 weeks)
Liver
Spleen
Bone Marrow (28 weeks)
35
Defect in sickle cell hemoglobin
2 alpha and 2 beta chains but one amino acid substitution in B chain (glutamic acid is replaced by valine)
Hemoglobin polymerize with in red cell and creates the sickle shape
36
Rate limiting step in heme synthesis
ALA-synthase, requires B6
37
Enzyme defective in lead poisoning
ALA dehydratase and ferrochelatase
38
```
Painful abdomen
Port-wine urine
Polyneuropathy
Psychological disturbances
Precipitated by Drugs
```
Acute intermittent porpyria
Defect in prophobilinogen deaminase
39
```
Blistering cutaneous photosensitivity
Tea colored urine
Hyertrichosis
Associated with hep C/ alcoholism
High LFTs
```
Porphyria cutanea tarda
Defect in uroporphyrinogen decarboxylase
*Most common porphyria
40
Malignancies with high epo leading to polycythemia
"Potentially Really High Hematocrit"
Pheochromocytoma
Renal Cell Carcinoma
Hepatocellular Carcinoma
Hemangioblastoma
41
alpha globin gene deletions
alpha thalassmia
African and asian
1 abnormal allele = no anemia
2 abnormal alleles = alpha thalassemia minor, no anemia
3 abnormal alleles = HgH disease, little alpha globin, and the excess B globin forms B4
4 abnormal alleles = incompatible with life, hydrops fetalis
42
Defect in beta globin gene most common in what populations?
Mediterannean
43
B chain is underproduced
B-thalassemia minor
Usually asymptomatic
Increased HbA2
44
Absent beta globin
B-thalassemia major
Target cell
SEVERE anemia
Requires blood transfustions
45
What would use a to treat a B-thalassemia major patient with hemochromatosis
Not phelbotomy = counter productive!!
Instead chelate the iron with deferoxamine!
46
Severe anemia
Target cells
Hair on end appearance on skull extra
Chipmunk faces
B thalassemia major
47
hypocellular bone marrow with fatty infiltration
Aplastic anemia
48
Microcytic anemia + swallowing difficulty + glossitis
Plummer Vinson
49
Microcytic anemia
| > 3.5% HbA2
B-Thal minor
50
Macrocytic anemia
| Hypersegmented neutrophils
B12 or Folate
51
Megaloblastic anemia
| Peripheral Neuropathy
B12 deficiency
52
Megaloblastic anemia
| Basophilic stippling
Lead poisoning
53
Microcytic anemia reversible with B6 supplements
Sideroblastic Anemia
54
HIV-positive patient with macrocytic anemia
ZIdovudine
55
Normocytic anemia with elevated creatinine
Anemia of chronic disease
56
+ osmotic fragility test
Hereditary spherocytosis
High % of lysis of RBCs at any given concentration of NaCl
57
Treatment for hereditary spherocytosis?
Splenectomy - will then see howell jolly bodies!
58
Common causes of sickle crisis?
Hypoxemia
Dehydration
Acidosis
**deoxygenated HbS polymerizes!!!
59
What resistance do heterozygotes for sickle cell trait have?
Resistance to malaria
60
Treatment for sickle cell
Hydroxyurea - increases production of HbF
Bone Marrow transplantation
61
Direct coombs test
Coombs reagent = anti-human antibodies (bind constant region)
Add coombs reagent to pts serum, if Abs present on RBCs they will agglutinate when coombs reagent is added
62
Indirect coombs test
Looking for antibodies in serum....
Normal RBCs added to pts serum, then add coombs reagent and watch for agglutination
63
Difference between hemoglobin S defect and hemoglobin C defect?
```
S = glutamic acid to valine
C = glutamic acid to lysine
```
64
What molecules are involved in platelet adhesion?
Endothelial damage exposes collagen
VWF binds collagen and then binds Gp1B receptor on platelets
65
Describe platelet activation
ADP is released from platelets bound to the vWF
ADP stimulates platelets to expose Gp2b3a receptors
Fibrin connects Gp2b3a receptors and allows for aggregation to occur
66
Clopidogrel
ADP receptor blockers
67
Abciximab
Tirofibal
Eptifibatide
Glycoprotein 2b/3a inhibitors
68
```
Mucous membrane bleeding
Epistaxis
Petechiae
Increased bleeding time
Normal PT/PPT
Anti Gp2b3a antibodies
```
Immune thromobcytopenia
Abs bind platelets and complex gets eaten by splenic macrophages
LOW platelet count
Increased megakaryocytes on bone marrow biopsy
69
Deficiency in metalloprotease ADAMTS`13
```
Fever
Anemia
Thrombocytopenia
Renal failure
Neuro symptoms
```
Thrombotic Thromocytopenic Purpura
ADAMTS13 is deficient
Less degradation of vWF multimers
More large vWF multimers
Increased platelet adhesion
Increased platelet adhesion and thrombosis
70
Bernard-Soulier Syndrome
Defect in Gp1b (defect in platelet to vWF adhesion)
71
Glanzmass thrombasthenia
Defect in Gp2b3a (defect in platelet to platelet aggregation)
72
Most common inherited bleeding disorder
Von Willebrand disease
Decreased vWF
Mixed! Affects both coag casacade and platelet plug formation
73
2 functions of vWF
Stabilize and protects factor 8
Binds to subendothelial collagen and Gp1b on platelets
74
Ristocetin cofactor assay
Ristocetin = makes vWF aggregate
SO if no vWF then no agglutination!
75
Treatment for vWF Disease
Desmopressin - releases vWF from stored in endothelium
76
```
Low fibrinogen
High D-dimer
Low platelet, high bleeding time
Schistiocytes
Bleeding
Multiorgan Failure
```
DIC
77
Causes of DIC
"STOP making thrombi"
```
Sepsis (gram neg)
Trauma
Obstetric complications
Pancreatitis (acute)
Malignancy
Transfusion
```
78
What defines Hodgkin lymphoma?
Presence of Reed-Sternberg cells
"Owl's Eyes"
79
Constitutional B symptoms of hodgkin lymphoma
Fever
Night Sweats
Wt loss
80
What CDs are found on Reed Sternberg cells
CD15 and CD30
2 owls eyes x 15 =30
**the more reed sternberg cells the worse the prognosis
81
"Starry Sky" = lymphocytes with interspersed macrophages
Associated with EBV
t(8;14)
Burkitt Lymphoma
82
Reed Sternberg
Owls eyes
| Hodgkin Lymphoma
83
Down syndrome is associated with which hematological malignancy?
Acute lymphoblastic Leukemia (ALL)
"We ALL fall DOWN"
84
Auer rods
Acute Myloid Leukemia
85
Defined by philadelphia chromosome
Chronic Myloid Leukemia
t(9:22) = BCR-ABL
Encodes constitutively activated tyrosine kinase
"Philadelphia CreaML Cheese"
86
Treatment for philadelphia chromosome
Imantinib = inhibits bcr-abl tyrosine kinase
87
Tdt+ Leukemia
ALL
88
Leukemia with smugde cells
CLL
89
Anemia
Renal insufficiency
Back Pain
Hypercalcemia
MULTIPLE MYELOMA!
90
Rouleaux formation
| Punched out lytic bone lesions on x-ray
Multiple Myeloma
91
What type of antibody is secreted in multiple myeloma?
IgG
92
Increased red cell mass (hematocrit) without elevated erythropoietin
Plethroa
Pruritis after hot shower
Splenomegaly
Hyperviscosity of blood
Polycthemia vera
93
Mutation associated with polycthemia vera
JAK2 mutation
94
Teardrop RBCs
Myleofibrosis = obliteration of bone marrow due to increased fibroblast activity in response to proliferation of monoclonal cell lines
95
Treatment for hemophilia A and Von Willibrand Disease?
Desmopressin... releases VWF and Factor 8 stored in endothelium
