Hematology-Oncology Flashcards
0
Q
Platelet
A
From megakaryocyte
Not enough or dysfunction-petechiae
1/3 stored in spleen
Contains dense granules (ADP, Ca), alpha granules (vWF, fibrinogen)
1
Q
WBCs, high to low in terms of percentage
A
Neutrophils Lymphocytes Monocytes Eosinophils Basophils
“Neutrophils like making everything better”
2
Q
Leukocyte
A
Granulocyte, mononuclear cells. 4000-10000/microliter.
3
Q
Neutrophil
A
Multilobed nucleus. Lysosomes contain hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin.
Multisegmented=folate, B12 def
4
Q
Monocyte
A
Kidney shaped nucleus. Become macrophages in tissues.
5
Q
Macrophage
A
Long tissue life. APC.
6
Q
Eosinophil
A
Bilobed nucleus. Helminthic infections. Down regulate allergic reactions through histaminase. Phagocytic.
7
Q
Basophils
A
Bilobate nucleus. Allergic reaction. Contain heparin, histamine, leukotrienes.
8
Q
Mast cell
A
Allergic reaction. Degranulate-histamine, heparin, eosinophil chemo tactic factors. Type 1 hypersensitivity.
9
Q
Dendritic cell
A
Main inducers of primary Ab response
10
Q
Lymphocytes
A
Round densely stained nucleus, small pale cytoplasm. B and T.
11
Q
B lymphocyte
A
Humoral immunity. Produce Abs.
12
Q
T lymphos
A
Cellular immune response. Regulate B lymphos and macrophages.
13
Q
Plasma cells
A
Off center nucleus, clock face chromatin. B cells differentiate into these.
Multiple myeloma is a plasma cell neoplasm.
14
Q
Vitamin K
A
Cofactor in synthesis of II, VII, IX, X, C, S
15
Q
Epoxide reductase
A
Activates vitamin K
16
Q
Warfarin
A
Inhibits epoxide reductase
17
Q
tPA
A
Degrades plasminogen into plasmin
18
Q
Antithrombin
A
Inactivates II, VII, IX, X, XI, XII
19
Q
vWF
A
Binds to expose collagen after endothelial damage. Platelets then bind to vWF. Eventually forms platelet plug.
20
Q
Abciximab
A
Inhibits GpIIb/IIIa
21
Q
Clopidogrel
A
Inhibit ADP-induced expression of GpIIb/IIIa
22
Q
Increased ESR
A
Infections, inflamm disease, malignancy, UC, pregnancy
23
Q
Decreased ESR
A
Polycythemia, sickle cell, CHF, microcytosis, hypofibrinogenemia
24
Bite cell
G6PD deficiency
25
Spur cell
Liver disease, abetalipoproteinemia
26
Elliptocyte
Hereditary elliptocytosis
27
Ringed sideroblasts
Sideroblastic anemia
28
Schistocyte, helmet cell
DIC, TTP/HUS, traumatic hemolysis
29
Spherocyte
Hereditary spherocytosis, autoimmune hemolysis
30
Teardrop cell
Bone marrow infiltration
31
Heinz bodies
Alpha thalassemia, G6PD def
32
Howell jolly bodies
Functional hyposplenia, asplenia
33
Microcytic anemias
```
Iron deficiency
ACD
Thalassemias
Pb poisoning
Sideroblastic anemia
```
34
Non-hemolytic normocytic anemias
ACD, aplastic anemia, kidney disease
35
Megaloblastic macrocytic anemias
Folate def
| B12 def
36
Non-megaloblastic, macrocytic anemias
```
Liver disease
alcoholism
reticulocytosis
metabolic disorders
drugs
```
37
Plummer-Vinson syndrome
Iron deficient anemia
Esophageal web
Atrophic glossitis
38
Hb Barts
4 gene deletion alpha globin. Incompatible with life.
39
HbH
3 gene deletion alpha globin.
40
Beta thalassemia minor
B chain underproduced. Usu asymptomatic. Increased HbA2.
41
B thal major
B chain absent. Severe anemia requiring transfusions. Marrow expansion ("crew cut") on skull XR. Increased HbF.
42
Pb poisoning
Lead lines on gingivae
Abdo colic
Wrist and food drop
Encephalopathy
Tx with EDTA, or succimer in kids
43
Sideroblastic anemia
X linked or alcohol or lead.
If creased iron, ferritin
Tx with pyridoxine
44
Folate def
Hypersegmented neutrophils, glossitis. Decreased homocysteine but normal methylmalonic acid.
45
B12 def
Hypersegmented neutrophils, glossitis, increased homocysteine AND methylmalonic acid
46
Intravascular hemolysis
Decreased haptiglobin
Increased LDH
Hb in urine
47
Extravascular hemolysis
Increased LDH, UBC (jaundice)
48
Causes aplastic anemia
Radiation or drugs (benzene, chloramphenicol, alkylation agents, antimetabolites)
Viral agents
Fanconi anemia
Idiopathic
49
G6PD def
X linked
Increased RBC susceptibility to oxidant stress...hemolysis
Back pain, hemoglobinuria few days later
Heinz bodies and bite cells
50
Paroxysmal nocturnal hemoglobinuria
Complement mediated RBC lysis. Increased urine hemosiderin.
51
Pyruvate kinase deficiency
Hemolytic anemia in newborn
52
Sickle cell
```
Crew cut on skull XR
Complications-
1. Aplastic crisis-parvovirus B19
2. Autosplenectomy-encapsulated bacteria
3. Splenic sequestration crisis
4. Salmonella osteomyelitis
5. Painful vaso-occlusive crisis...acute chest syndrome
6. Renal papillary necrosis
```
53
Porphyrias
Defective heme synth...leads to accum of heme precursors.
54
Acute intermittent porphyria
No porphobilinogen deaminase.
Buildup of porphobilinogen, delta-ALA, uroporphyrin
Red wine colored urine
Painful abdo
Neuro-psych sxs
55
Porphyria cutanea tarda
No uroporphyrinogen decarboxylase
Buildup of uroporphyrin
Tea colored urine
Photosensitivity
56
Pb poisoning-enzymes
No ferrocene lactase and ALA dehydratase
| Buildup of protoporphyrin
57
Major site hematopoiesis in early embryo life
Liver
58
Fetal Hb
Does not bind 2,3-BPG, so has higher oxygen affinity
59
Plasma
55% total blood volume
60
Serum
Plasma without clotting factors
61
Corticosteroids affect on blood
Inhibit PMN migration from circulation to periphery, thus benign leukocytosis
62
RBC energy source
90% glucose anaerobically degraded to lactate. Ernest through HMP shunt
63
Reticulocytes
Immature RBCs. Polyribosomes. Increased in blood loss, hemolytic anemia.
64
NK cell
Cytotoxic lymphocyte. Contains perforin, proteases. Target and kill cells lacking MHC1 (incl tumor derived cells, virus infected cells)
65
Causes eosinophilia
```
"NAACP"
Neoplasia
Asthma
Allergic processes
Collagen vascular disorders
Parasites
```
66
Dendritic cells
Main inducers of primary Ab response
67
B thalassemia minor
Reduced B chains
68
B thal major
Absent B chain.
Reduced RBC lifespan and apoptosis of precursors.
Anemia, splenomegaly, hemosiderosis from repeat transfusions, skeletal deformities ("crew cut" on skull XR)
Mediterranean
69
A thalassemia
Africa, Asian populations. Lack of alpha globin.
70
HbBarts
Gamma tetramers, four gene deletion alpha globin. Fatal.
71
Alpha thalassemia trait
2 gene deletion alpha globin. Minimal anemia
72
HbH
3 gene deletion. High oxygen affinity. Anemia disproportionate to amt Hb.
73
Schilling test
Tests if B12 def due to lack intrinsic factor
74
Neuro problems in B12 def
Demyelination of dorsal and lateral columns
| Ataxia, hyper reflexes, decreased positional and vibration sensation
75
G6PD
Protects RBCs from oxidant stress,
76
G6PD def
X linked. Black, middle eastern populations. Associated with resistance to malaria. Hemolysis from oxidant stress eg drugs, infections, favs beans.
History of neonatal jaundice and cholelithiasis
Episodic fatigue, pallor, symptomatic anemia
Heinz bodies, bite cells
77
Hereditary spherocytosis
AD def of spectrin, ankyrin. Increased RBC fragility. Spherocytes.
Anemia, splenomegaly, jaundice, maybe cholelithiasis.
78
Immunohemolytic anemias
Coombs positive
79
Erythroblastosis fetalis
Destruction of fetal Hb. Fetal hemolytic anemia.
80
Sickle cell
Sickling when deoxygenated..splenic destruction.
Blood hyperviscous.
Spleen particularly vulnerable since no collateral supply...auto splenectomy by 6 years.
HOWELL JOLLY BODIES.
81
"Crew cut" on skull XR
Extra medullary hematopoiesis.
| Sickle cell, B thal.
82
Paroxysmal nocturnal hemoglobinuria
Acquired. Lysis by endogenous complement.
83
HUS (Hemolytic Uremic Syndrome)
Hemolytic anemia, AKF, thrombocytopenia. Mostly kids. Most cases preceded by infectious diarrhea. Commonest cause AKF in kids.
84
LDH
Extensive in body tissues, e.g. RBCs, myocardium. Released during tissue damage, thus marker of common injuries and disease
85
Micropathic hemolytic anemia
Mechanical trauma by narrowed vessels. DIC, TTP, HUS, SLE, malignant HTN
86
Aplastic anemia
Severe anemia, neutropenia, thrombocytopenia. Failure/destruction of multipotent myeloid stem cells.
Idiopathic, radiation, benzene, drugs (chloramphenicol, sulfonamides, Alkylating agents, antimalarials, antimetabolites), viruses (parvovirus B19, EBV, HIV, HCV), Fanconi anemia
87
Thrombocytopenia
Purpura,mucosal bleeding, petechiae
88
Porphyrias
Defects in heme synthesis,resulting in accumulation of heme intermediates
89
Acute intermittent porphyria
Increased porphobilinogen and ALA
Causes myelin degeneration
Dark foul smelling urine, hallucinations, blurred vision, gross neuro manifestations eg foot drop
90
Porphyria cutanea tarda
Increased uroporphyrin, Fe, transferrin
Accumulation of Fe leads to siderosis which affects liver
Photosensitivity and cutaneous bullae.
Tx with low dose antimalarials
91
Hemophilia
Intrinsic pathway defect. Increased PTT.
A-VIII
B-IX.
92
Ehlers danlos
Inherited collagen and/or elastin abnormality. Decreased vessel wall strength, hyper elasticity of skin, joint abnormality, tissue fragility
93
PT
Extrinsic and common pathway. Factors V, VII, X, prothrombin, fibrinogen
94
PTT
Intrinsic. Increased with heparin
95
Waldenstrom macroglobulinemia
Hyperviscous blood damages vessels
96
Idiopathic thrombocytopenic purpura
Thrombocytopenia with normal or increased megakaryocytes. Antiplatelet Abs attach to platelets...removed by spleen.
97
TTP
ADAMTS13 def....which normally degrades vWF. Thus, platelet aggregation. Adult females.
98
Von Willebrand disease
Most common inherited bleeding disorder. AD. vWF normally carries factor VIII and mediates platelet adhesion.
99
Bernard Soulier
AR. Lack platelet surface GpIb (needed for platelet adhesion).
100
ALL
<15 years. Most responsive to tx. Bone marrow replaced by loads of lymphoblasts
Association with Downs.
101
CLL
>60 years. Oft a symptomatic. Smudge cells.
102
AML
Auer rods. Loads of myeloblasts. T(15;17) subtype= M3 or APL subtype
103
CML
Philedelphia chromosome t(9;22).
| Bcr-abl
104
Langerhans cell histiocytosis
Proliferation of langerhans cells. Birbeck granules "tennis rackets" on electron microscopy.
Immature cells that do not efficiently stimulate T lymphos.
105
JAK2 mutations
Polycythemia Vera, essential thrombocytosis, myelofibrosis
106
JAK2
Involved in Hematopoietic growth factor signaling
107
Polycythemia vera
Increased RBCs, WBCs, platelets
108
Myelofibrosis
Decreased RBCs
109
Essential thrombocytosis
Increased platelets
110
Bcr-abl
Increases cell division and decreases apoptosis
111
LMWH, eg enoxaparin
Act more on Xa. Better bioavailability, 2-4x longer half life than regular heparin.
112
Intravascular hemolysis
Hemoglobinemia, hemoglobinuria, decreased serum haptoglobin
Result of complement fixation, mechanical injury, or toxins
113
Extravascular hemolysis
Splenomegaly
Result of RBC injury, Ab attachment, or decrease in deformability.
114
Mixing study
Pt's blood mixed with all factors nec for clotting. If bleeding time corrects, then it is a clotting factor deficiency. If it does not correct, it is an Ab to one of the clotting factors.
115
Hereditary hemorrhagic telangiectasia
AD mutation leading to dilated tortuous vessels with thin walls in skin and mucous membranes
116
Bleeding time
Prolonged if platelet abnormality
117
Liver disease
Prevents production all coag factors (except vWF). Thus, hypersplenism and thrombocytopenia.
Can actuaries bleeding into joints and easy bruising.
