Hematology part 2 DIT - part 1 in endo Flashcards
1
Q
protein responsible for the flexibility of RBCs
A
Spectrin
2
Q
Anisocytosis
A
RBCs of varying size
3
Q
Poikilocytosis
A
RBCs of varying shape
4
Q
erythrocytosis
A
same thing as polycythemia
5
Q
Life span of a RBC
A
120 days
6
Q
Incompatible blood transfusion is due to what type of process
symptoms?
A
Type II HTN
hemolysis
renal failure
shock
death
7
Q
erythroblastosis fettles is due to
prevented by? timeline of treatment (3)
A
mothers(Rho-) IgG antibodies crossing the placental and attacking the fetus’s Rho + RBCs
->anemia, jaundice-> kernicterus, hydrops fetalis, intrauterine death
Give RhoGram (IgGs that mask presence of Rho + RBCs) Give at: -28 wks - any traumatic event - w/in 3 days of delivery
8
Q
acanthocytes are?
Seen when? (2)
A
irregularly spiked RBCs (vs regularly spiked RBCs in echinocytes)
liver disease
abetalipoproetinemia
9
Q
Liver disease see what 2 changes to the RBCs
A
acanthocytes
target cells
10
Q
Basophilic stippling of the RBCs indicates what (3)
A
Lead poisoning*
Thalassemia
anemia of chronic disease
11
Q
Bite cells are seen in
A
G6PD deficiency - Macrophages removing oxydized hemoglobin (heinz bodies)
12
Q
Elliptocytes are?
seen in
A
pencil shaped cells
hereditary elliptocytosis
13
Q
schistocytes are?
seen in (3)
A
cut up RBCs
Due to DIC, TTP/HUS, trauma (metal heart valve)
14
Q
sideroblasts are?
differ from ringed siderblasts how?
A
nucleated RBC precursor w/ granules of iron surrounding the mitochondria in the bone marrow - NORMAL
ringed sideroblasts is due to a disorder in heme synthesis -> excess iron granules that circle the nucleus (can lead to anemia
15
Q
Causes of ringed sideroblasts (4)
A
lead poisoning
drugs
genetic conditions
myelodysplastic syndromes
16
Q
Sherocytes commonly seen in
A
hereditary spherocytosis
17
Q
teardrop cells seen in
A
bone marrow infiltration; like myelofibrosis (squeezed out)
18
Q
Target cells are seen in ((4)
A
THAL
Thalessemia
Hemaglobin C disease
Asplenia (sickle cell or post surgery)
Liver disease
19
Q
Heinz bodies are?
A
oxidation of heme in the RBCs in G6PD deficiency -> benatured hemoglobin precipitates
Can be “bite out”
20
Q
Howell jolly bodies are seen?
A
nucleated RBCs that are normally removed by the spleen
Seen in apslenic patients
21
Q
Asplenic patients you may see(2)
A
howell jolly bodies
Target cells
22
Q
HbA composition
A
normal adult hemoglobin - 97%
2 alpha
2beta
23
Q
HbA2 composition
A
2 % of normal Hgb
2 alpha
2 delta
24
Q
HbF composition
A
2 alpha
2 gamma chains
higher affinity for oxygen, less affinity for 2,3 DPG (allows placental transfer)
25
HbS composition
sickle cells
2 alpha
2 beta (glu -> valine)
26
HbC composition
hemoglobin C
2 alpha
2 beta (glu -> lys)
27
Hb barts composition
Severe thalassemia
no alpha
4 gamma
28
HbH compostion
Severe thalassemia
4 beta
no alpha
29
Fetal erythropeies occurs where (4)
timeline
young liver synthesizes blood
Yolk sac - 3-8 weeks
Liver* 6-birth
Spleen 15-30 wks
Bone marrow 22 wks - adult
30
Late adolesent/ adult erythropeisis occurs where specifically (3)
axilla skeleton
- vertebra
- pelvis
- ribs
31
Early childhood erthyropoeisis occurs where specifically?
flat and long bones
| -sternum, pelvis, ribs, cranial bones, vertebra, long bones
32
Lead poisoning interferes w/ these 2 enzymes
see what build up?
ferrochelatase
-protoporphyrin in the urine
```
aminolevulinic dehydratase (ALA dehydratase)
- aminolevulinic acid
```
33
can be given to inhibit porphyries and stop heme synthesis(2)
glucose and heme
34
induces heme synthesis
rate limiting step
alchohol, barbituates, seizure drugs, rifampin, metoclopramide
aminolevulinic acid synthase
35
acute intermittent porphyria is due to a defect in what enzyme
presents as (5)
pophobilinogen deamninase
Acute abdomen; acute neuropathy (heme substrates are neurotoxic)
```
Painful abdomen
port wine -colored urine
polyneuropathy
psychological disturbances
precipitated by drugs
```
36
patient's started on metclopramide for GERD and begins to have a painful abdomen, and dark urine, also polyneuropathy and agitation
acute intermittent porphyria due to issues w. porphobilinogen deaminase
```
build up of porphoblinogen
Painful abdomen
port wine -colored urine
polyneuropathy
psychological disturbances
precipitated by drugs
-alchohol, barbituates, seizure drugs, rifampin, metoclopramide
```
37
Porphyria cutanea tarda presents as ?
Due to defect where?
Most common - think of a homeless man
- blistering cutaneous photosensitivity
- hypertrichosis
- increase in ALT/AST
- tea color during
- facial hyperpigmentation
Associated w/ Hep C and alcoholism
defect in uroporphyrinogen decarboxylase
38
Presentation of lead poisoning?
```
global ecephalopathy
memory loss
delirium
mental deterioration
colicky pain
lead lines in gingiva and bone
foor/wrist drop
microcytic anemia
basophilic stippling
sideroblastic anemia
```
39
Causes of polycythemia (3)
Polycythemia vera
- monoclonal expansion due to abnormal stem cells
Chronic hypoxia
- Lung disease, congenital heart disease, high altitude
Secondary to ectopic production
- pheo
- renal cell
- hepatocellular carcinoma
- hemangioblastoma
60% of trisomy 21 babies are polycythemic
40
4 tumors that can lead to ectopic EPO -> polycythemia
pheo
renal cell
hepatocellular carcinoma
hemangioblastoma
41
what test can be used to diagnose beta thalassemia minor
hemoglobin electrophoresis
42
Lab values of thalassemia minor
Ferritin
TIBC
Serum Fe
All are normal
may see target cells in peripheral smear
43
microcytic anemia, swallowing difficulties and glossitis
Plummer Vinson Syndrome
44
Microcytic anemia and >3.5% HbA2 level
Thalassemia minor
45
Megaloblastic anemia not correctible with B12 or folate
orotic aciduria (w/o the hyper ammonia)
46
Microcytic anemia reversible by B6
sideroblastic anemia
47
HIV positive patient w/ macrocytic anemia
AZT; zidovudine
48
normocytic anemia and elevated creatine
chronic renal disease
49
Hypersegmented nuetraphils be suspicious of
Folate or B12 deficiency
50
Causes of hyposchromic, microcytic anemia(4)
Fe deficiency
Thalassemia
lead poisoning
Sideroblastic anemia
51
Iron deficiency can be due to? (3)
chronic bleeding - CA-
malnutrition/absorbtion
increased demand -pregnant
52
Lab findings w/ Fe deficiency
Serum Fe
Ferritin
Transferrin
TIBC
low Fe in serum
low Ferritin
Increased transferrin ( trying to mobilize limited stock)
Decreased TIBC (not saturated at all)
53
Alpha thalassemia presentation (4) due to?
4 copies of the gene inherited on chromosome 16
1 mutation - no anemia
2 mutations- alpha thalassemia trait, no anemia
3 mutations - anemia; HbH forms (4 globins beta)
4 mutations- hydrops fatilis w/ formation of Hb Barts (4 globins of gamma )
54
Populations at increased risk for alpha thalassemia
Africans - trans mutation
| Asians - cyst mutation
55
beta thalasemia presentation (2)
2 genes at work
Minor - one gene mutated (heterozygote) -> asymptomatic usually
see increased HbA2 on Hb electrophoreses (with less beta, more delta glob ins produced)
Major - genes mutated -> severe anemia
- Needs blood transfusions -> hemochromatosis
- Marrow expansion, chipmunk facies
- increased HbF (2 alpha 2 gamma); can't make any beta globin
- target cell
56
2 diseases w/ marrow expansion
crew cut appearance on X ray
Sickle cell and Beta thalassemia
57
Population at risk for beta thalassemia
Mediterranean
58
Sideroblastic anemia due to ?
defect in heme synthesis -> ringed sideroblasts ( iron laden macrophages)
- hereditary - X linked
- reversible: alcohol, lead, INH, Rifampin
Rx w/ pyridoxine
59
Anemia due to chronic disease lab values
Serum Fe
Ferritin
Transferrin
TIBC
Serum - low
Ferritin - high (acute phase reactant)
transferrin- decreased
TIBC - normal
60
Hemachromatosis lab values
Serum Fe
Ferritin
Transferrin
TIBC
Serum - High
Ferritin - High
Transferrin - Low (body senses a lot and doesn't want to mob)
TIBC - High ( a lot of saturation)
61
Pregnancy and OCP - Fe lab values
Serum Fe
Ferritin
Transferrin
TIBC
Serum - normal
Ferritin - normal
Transferrin - increased w/ estrogen
TIBC - Lower w/ excess binding sites
Transferrin production ramped up
62
Sideroblastic anemia Fe lab values
Serum Fe
Ferritin
Transferrin
TIBC
Serum - Increased (not using the Fe w/ defect in heme synth)
Ferritin - normal or increased
Trasnferrin -decreased, senses high serum
TIBC - is thus increased
63
Meagloblastic macrocytic anemia causes ( 3)
Impaired DNa synth -> increasing cytoplasm w/ ineffective nucleus maturation
B12 deficiency
Folate deficiency
Orotic aciduria
64
Folate deficiency due to (4)
Presentation (4)
malnutrition
-alcoholic
malabsorbtion
antifolates
- methotexate
- trimethoprin
increased requirement
-pregnanat
Presents w/
- megaloblastic/macrocytic anemia
- hyper seg PMNs
- glossitis
- homocystinuria w/o MMA increase
65
B12 deficiency due to (5)
Presentation
```
inefficent uptake ( vegans)
malabsorbtion - crohns
pernicious anemia
Diphyllobothrium datum
PPIs
```
Hyperseg PMNs
glossitis
homocystinuria w/ increased MMA
Neuro symptoms (B12 used in myelin synth)
66
Which vitamin Def presents w/ neuro symptoms and megaloblastic anemia
B12 deficiency
- Neuro: Peripheral neuropathy, posterior columns, lateral corticospinal, dementia
need to be aware and not just give folic acid w/ megaloblastic anemia
67
Orotic acid in the urine and fatigued
Hyperammonia?
Megaloblastic macrocytic anemia due to deficiency in UMP syntase in the production of Pyrimidines
No hyper ammonia ( Ornithine transcarbonylase in urea cycle)
Cannot be cured w/ B12 or folate
68
Non megaloblastic - Macrocytic anemia causes ((3)
DNA synthesis is unimpaired
```
Liver disease
Alcoholism - direct effect
Drugs
- 5 FU
- AZT
-hydroxyurea
```
69
Nonhemolytic normocytic anemia due to (3)
anemia of chronic disease
Aplastic anemia
Chronic kidney disease
70
Anemia due to chronic disease is due to what acute phase reactant pathology
an increase in hepcidin binds to ferroportin preventing the release of Fe out of macrophages
71
Aplastic anemia causes (4)
Radiation *
Viral: B19, EBV, HIV, HCV - especially if sickle cell
Fanconi's Anemia - DNA repair defect
Idiopathic
-> pancytopenia
72
Pancytopenia bone histology(2)
hypocellular
| bone marrow w/ fatty infiltration
73
Symptoms of aplastic anemia (3)
anemia
- fatigue
- malaise
- pallor
Leukoplakia
- Infection
thrombocytopenia
- purpura
-mucosal bleeding
petechiae
Stop the offending agent, transfuse, give G-CSF or GM -CSF
74
Rx for anemia due to chronic kidney disease
EPO
75
Basophilic nuclear remnants in RBCs
Howell Jolly body
Post splenctomy
76
Increased LDH indicates
either extravascular or intravascular hemolysis
Decreased haptoglobin also -> intravascular
Increased uncongugated bilirubin -> extravascular
77
Haptoglobin is?
molecule that binds to free hemoglobin found in the vasculature
decreased in intravascular hemolysis due to complex formation
78
Intravascular hemolysis examples and lab findings
See decreased haptoglobin and increased LDH
Due to mechanical destruction, autoimmune, paroxysmal nocturnal hemoglobinuria
79
Extravascular hemolysis examples and lab findings
RBCs removed from circulation and lysed
Examples: G6PD, hereditary spherocytosis, pyruvate kinase deficiency, sickle cell,
Increased LDH and uncongugated billirubin
80
Hereditary Spherocytosis presentation (5)
Rx?
Extravascular hemolytic disease _
Auto Dom mutation in spectrin, ankrin or band 3, protein 4.2
```
Jaundice,
Spherocytes,
increased MCHC and RDW,
+ osmotic fragility, neg Coombs,
Splenomegaly
```
Rx - splenectomy
81
aplastic crisis due to parvo B19 for example
Hereditary spherocytosis
| Sickle cell
82
G6PD deficiency is?
intravascular/Extravascular** normocytic anemia
X linked defect w/ less glutathione regeneration -> susceptibility to oxidative stress -> hemolytic anemia
83
Pyruvate kinase deficiency pathophys and presentation
normocytic hemolytic anemia
Auto recessive defect leads to decreased ATP production and inability to maintain Na/K pump -> rigid cells that are lysed
84
Hemolytic anemia in a newborn think of
pyruvate kinase deficiency
also could be IgG crossing over and attacking Rho +
85
HbC mutation vs HbS mutation
glutamic acid -> valine on chrom 6 = sickle cell
glutamic acid -> lysine = HbC
86
Paroxysmal nocturnal hemoglobinuria pathophys
Rx?
intravascular hemolytic anemia due to increased lysis by complement - lack CD 55/59 which is protective
eculizumab
87
hemolytic anemia, pancytopenia and venous thrombosis
hink red urine in the morning
Paroxysmal nocturnal hemoglobinuria
88
HAMs test is useful for
test for paroxysmal nocturnal hemoglobinuria
Low pH activates compliment
89
Lack of CD55/59 leads to
paroxysmal nocturnal hemoglobinuria and red urine in the morning
90
Why are newborns asymptomatic w/ sickle cell?
they are primarily HbF at first and then become HbS as they start producing beta chains
defect in glutamic acid -> valine
8% of AA carry the trait
91
splenic sequestration crisis in HbS is
sickle cells in the spleen slowly occlude and list the spleen -> edge shaped infarct
eventual autosplenectomy by age 4,increased risk against encapsulated organisms
low Hct and Hb
92
Presentation of sickle cell/ complications(6)
```
aplastic crisis - parvo B19
autosplenectomy - encapsulated organisms risk (SHiN)
splenic sequestration crisis
salmonella osteomyolitis
painful crisis
- dactylitis
- acute chess syndrome
Renal papillary necrosis -> hematurua
```
93
Rx for suckle cell (2)
bone marrow transplant
| hydroxyureas=
94
Extrinsic causes of normocytic hemolytic anemia(4)
Cause is outside of the RBC (Intrinsic means somethings wrong w/ the RBC)
Autoimmune
Microangiopathic anemia
Macroangiopathic anemia
infections
95
Infections that may cause hemolytic anemia(2)
malaria
| Babesia
96
Macroangiopathic anemia causes (2)
extrinsic anemia -> schistocytes
prosthetic heart valves or aortic stenosis; big vessels cause
97
Microangiopathic anemia causes
extrinsic anemia
Due to DIC, TTP-HUS, SLE and maligant HTN
(shear stress) or fibrin leads to schistocyte formation w/ obstructed narrowed lumens
98
Cold agglutins vs Warm agglutins - autoimmune anemia
Cold
- IgM
Due to: mycoplasma or EBV or CLL
Warm
-IgG
Due to SLE, malignancies (CLL, non Hodgkins), congenital immune compl, drugs
99
Cold agluttin anemia due to ? (3)
- IgM
Due to: mycoplasma or EBV or CLL
iGm bonds to RBCs -> complement fixation -> MAC lysis and opsinization
100
Warm agglutin anemia due to? (4)
Warm
-IgG
Due to SLE, malignancies (CLL, non Hodgkins), congenital immune compl, drugs
101
overlap in warm and cold anemia agglutinin causes?(2)
CLL
| EBV
102
Direct coombs test is?
Useful for(3)
Testing for the presence of antibodies already present on the RBC -give prepared antibodies to detect and agglutinate (Coombs)
Hemolytic anemia of newborn
drug induced autoimmune
hemolytic transfusion reaction
103
Indirect Coombs test is?
Useful for (2)
Testing the patient's serum which is incubated w/ normal RBCs to detect presence of Ab
Antibodies present to foreign blood (prior to transfusion) and screening for maternal antibodies to fetus blood
104
platelets derived from ?
last?
megalokaryocytes
no nucleus
come preformed w/ ganules containing: Ca, vWF, COX, TXA2, thrombin, fibrinogen, PDGF, lysosome enzymes
Lasts 8-10 days
105
vWF receptor
fibrinogen receptor
GpIb
GpIIb/IIIa
106
Von willibrand factor is found where?(2)
2 jobs?
in endothelial cells and in platelets
1. stabilizes factor 8 ( deficiency leads to problems similar to factor 8 deficiency, increases PTT (hemophilia A)
2. Bridges exposed collagen and to platelets w/ GpIb
107
3 steps in platelet plug formation
injury -> exposure of sub endothelial collagen and release of vWF
1. Adhesion
= platelet uses vWF to bridge collagen to platelet via GpIb
2. Activation
- > release of ADP, stuff for complement cascade later (Ca, Thrombin, fibrinogen), TXA2
- > ADP recruits Gp IIb/IIIa receptors to the cell surface
3. Aggregation
- Fibrinogen links the newly recruited GpIIb/IIIa receptors together of different platelets
- TXA2 encourages vasoconstriction and platelet aggregation
108
Endothelial cells contain(4)
vWF*
thromboplastin
tPA
PGI2
109
ASA roll on PT and PTT
none just irreversible blocks COX1 promotion of TXA2 formation decreasing platelet plug formation and increasing bleeding time
110
ADP receptor inhibitors (4)
Clopidogrel
Ticlopidine
prasugrel
ticagrelor
111
Uses for ADP receptor inhibitors (4)
acute coronary syndrome
post stent
reduce incidence or recurrence of stroke
ASA allergy?
112
GP IIb /IIIa inhibitors (3)
abcliximab
eptiFIBate
tiroFIBan
think about use in non ST elevated MI
113
Uses of G IIb/IIIa use
Non ST elevated MI
percutaneous transluminal coronary angioplasy
114
Platelet disorders are characterized by what symptoms(4)
microhemorrage
mucous membrane bleeding,
epistaxis
petechia - >purpura
increased bleeding time
115
Immune Thrombocytopenic purpura pathophys?
presentation?
defect in anti GbIIb/IIIa antibodies -> splenic macrophage consumption of platelets
Megalokaryocytes are enlarged due to trying to keep up
```
Low Platelet count and increased bleeding time
see microhemorrage (petechia/purpura, epistaxis, membrane bleeding)
```
NORMAL RBCs
116
Rx for ITP?
immune suppression
splenectomy - more platelets in circulation
IVIG
steriods
117
Thrombotic thrombolytic purpura (TTP) 5 clinical manifestations
Nasty Fever Torched His Kidneys
```
Neuro changes
Fever
Thrombocytopenia*
Hemolysis* -microangiopathic due to fibrin/Complement act
Kidney failure*
```
* seen in hemolytic uremia syndrome
118
How is HUS related to TTP
Occurs when?
Similar presentation of thrombocytopenia, Uremia/kidney failure, hemolysis
NO neurological changes or fever as in TTP
associated w/ post O127 H7 in kids
119
Pathogenesis of TTP
Deficiency in ADAMTS 13 -> decreased degradation of vWF multimers -> hyper activation and consumption of platelet factors and microvascular hemolytic anemia w/ partial activation of the complement system
```
Nasty Fever Torched His Kidneys
Neuro changes
Fever
Thrombocytopenia*
Hemolysis* -microangiopathic due to fibrin/Complement act
Kidney failure*
```
120
Schistocytes, fever, petchial rash and increased bleeding time be concerned w/
Thrombotic Thrombocytopenic Purpura
121
Bernard Soulier syndrome pathogenesis
labs?
defect in GpIb -> prevention of initial platelet adhesion
decreased platelet count due to slightly enlarged platelets being removed and increased bleeding time
122
Glanzman thrombasthenia
labs?
defect in GpIIb/IIIa -> prevention of aggregation of platelet cells (no clumping)
no thrombocytopenia, just can't form platelet plug
- PC
- Increased bleeding time
123
von willebrand disease pathogenesis
Labs?
PC
BT
PT
PTT
Autosomal dominant deficiency in vWF ->
1. decreased stabilization of factor 8
2. decreased platelet adhesion to sub endothelial collagen exposure
PC- normal
BT - increased
PT - normal
PTT - increased
124
Rx for vWF deficiency
Desmopressin - (ADH analog) increase the release vWF from the enthelium
125
Pathogenesis of DIC
```
Labs
PC
BT
PT
PTT
```
widespread activation of the clotting mechanism and complement system uses up platelets and complement factors; fibrin -> lysis of RBC and microvascular anemia
```
Sepsis
Trauma
Obstetrical problems - placenta abruptous/amniotic emboli
Pancreatitis (acute)
Malignancy
Transfusions
```
low PC
High BT
High PT
High PTT
also increased D dimer and low fibrinogen
126
schistocytes and increased bleeding time disorders (2)
TTP
DIC
127
causes of DIC (6)
STOP Making Thrombin
```
Sepsis
Trauma
Obstetrical problems - placenta abruptous/amniotic emboli
Pancreatitis (acute)
Malignancy
Transfusions
```
128
most common lymphoma in the US?
Diffuse B cell lymphoma
129
lymphoma associated w/ EBV(2)
burkitts lymphoma (NHL)
50% w/ hodgkins
130
Lymphoma associated w/ long term celiac disease
intestinal T cell lymphoma
131
Lymphoma equivalent to CLL
Small lymphocytic lymphoma
132
Most common non hodgkins lymphoma in kids?
lymphoblastic lymphoma
133
lymphoma associated w/ sjogrens, hashimotos and H pylori
marginal cell maltoma
134
cervcal painless LAD of the neck w/ fever, weightless and Nightsweats
hodgkins lymphoma
135
Leukemia vs Lymphoma
Leukemia originates/involves the bone marrow and tumor cells found in the peripheral blood
Lymphoma arises from lymph nodes
136
LAD presentation in hodkins lymphoma
painless non-tender LAD in a single group of nodes, can also be in the mediastinum; firm and rubbery
contiguous spread
137
Age distribution of hodgkins lymphoma vs nonhodgkins lymphoma
Hodgkins - bimodal 20s and 60s
Nonhodgkins is more variable 20-60
138
Constitutinal signs/ B symptoms are (3) associated w?
hodgkins lymphoma
Fever, weight loss light sweats
puritis also may be an issue
139
Spread and presentation of nonhodgkins lymphoma
multiple peripheral nodes w/ extranodal involvement being common (GI, thyroid, CNS)
noncontiguous spread (jumps)
Majority are B cell (some are T cell)
less constitutional symptoms
140
Reed stern berg cells are seen in?
Derived from?
Hodgkins disease
binucleate cell of B cell origin w/ CD 30 and CD 15
less of them the better - lymphocyte predominant and nodular sclerosing subtypes
141
Subtypes of hodgkin's lymphoma (4)
Lymphocytic predominant
- least common but best prognosis
nodular slcerosing
- most common and equal m vs f incidence
mixed cellularity
- 2nd most common
- poor prognosis
lymphocyte depleted
- poor prognosis
142
nodular sclerosing subtype of hodgkins lymphoma
histology
most common subtype of bodkins
Bands of collagen, sclerosis and fibrosis
143
Non hodgkin lymphoma and mutation
t8: 14
t11: 14
t14: 18
8: 14 - burkitts ( cmyc w/ Ig heavy chain)
11: 14 mantle cell (cyclin D w/ Ig heavy chain)
14: 18 follicular cell (bcl2 w/ Ig heavy chain)
144
Burkett lymphoma associated w?
3 types
EBV infections, found in younger kids; starry night; 8:14
Endemic - jaw in africa; nasopharyngeal carcinoma association
sporadic form in pelvis or abdomen
immune deficient - HIV
145
Diffuse large cell B lymphoma
most common in adults
| most common overall in the US
146
Mantle cell Lymphoma translocation
t11:14; cyclin D
poor prognosis, B cell
147
Follicular cell lymphoma translocation
t14:18; bcl 2
adults; indolent course, hard to Rx
148
T cell non- hodgkins lymphoma(3)
adult t cell lymphoma aw/ HTLV 1
Mycosis fungoides -> sezary
Intestinal T cell lymphoma
149
Cutaneous lesion that is aggressive, patient is from Japan and has a history of HTLV1 infection
Adult T cell lymphoma
can also be caribbean and west african in origin
150
cutaneous patch/nodule that resembles a fungal infection but does not heal w/ anti fungals/ Histology shows CD4 cells
when gets into the blood called?
Mycosis fungoides
sezary
151
Acute leukemia characterized by?
smear?
bone marrow?
rapid onset and rapidly progressive; pancytopenia (anemia, bleeding and infection)
> 50% in the bone marrow
- myeloblasts in AML
- lymphoblasts in ALL
in the periphery > 20% blasts
152
ALL presentation
PAS?
age?
Philadelphia chromosome (maybe)
usually in children; more so down syndrome
Bone pain is common
most have terminal deoxynucleotice transferase (TdT)
very good prognosis
PAS +
153
TdT positive in what leukemia?
ALL
PAS + also
154
AML presentation
PAS?
Age
Remember this line is granulocytes, RBCs, monocytes etc...
characteristically has Auer Rods (M3)
Most are CD 13/33
PAS - (usually nonspecific esterase + [peroxidase])
Age is usually 60 -65
155
Risk factors for AML
```
Radiation
benzene
alkylating agents
myeloproliferative disease (like polycythemia vera)
mlyelodysplastic syndrome
aplastic aneima
downs as well (ALL)
```
156
Chroic leukemia characterisics
```
insiderous onset
mature cells (<5%blasts)
```
associated w. hepatospelnomegaly and LAD
prominent infiltration of bone marrow and peripheral WBC counts may be high
157
CLL presentation
characteristic feature?
Age
most common adult leukemia
Adults over the age of 50 (usually 70s-80s)
95% have B cell markers
some progress to ALL
smudge cells
Autoimmune hemolytic aniema (cold/warm)
indolent
158
Smudge cells are seen in
CLL
159
CML
age and presentation
Charcteristic feature
may progress to AML in a blast crisis
see mature basophils and PMNs that are LAP (-) leukocyte alkaline phosphatase
-hyperplasia of all 3 cell ones but granulocyte precursors predominate
Age - 25-60
Always phildelphia chromosome 9;22 (bcr-abl)
Rx imatinib
fatigue, ab pain, spenomegaly
160
Leukocyte alkaline phosphatase (-) cells; numerous w/ mature basophils and PMNS
CML
161
t15:17 translocation
rx?
M3 AML
See more Auer rods
Rx al trans retinoic acid
162
Monoclonal antibody spike think of 3 things
Multiple myeinoma
Waldenstroms macroglobinemia
MGUS - monoclonal gammopathy of undetermined significance
163
leukemia commonly presents w/ bone pain
ALL
164
myleodysplastic syndrome
can see?
dysplasia of hematopoeitc cells in the myeloid tissue
Not CA yet
Pelger Huet anomaly - nuclei w/ 2 lobes
asymptomatic and can be incidental finding, watched
165
Tartate resistant acid phosphatase
stain that is positive in hairy cell leukemia,
a maute b cell tumor in the elderly
166
imatinib is used to treat?
CML
167
Myeloproliferative disorders(4)
neoplastic transformation of a single myeloid precursor into many
Polycythemia vera - RBCs
Essential thrombocytosis - megalokaryocytes
myelofibrosis - fibrosis
CML - granulocytes
associated w/ Jak 2 mutation (especially polycythemia)
168
Jak 2 mutation is associated w?
myeloproliferative disorders
especially polycythemia vera
169
Symptoms of polycythemia vera(5)
EPO?
```
plethora - red face
itching after a shower
splenomegaly
hypervisous blood
erythromyalgia
```
LOW epo - not stimulated by outside
170
Essential thrombocytosis is?
presentation
myeloproliferative disorder of megalokaryocytes
have LOW thrombopoeitin
bleeding w/ poor platelet function despite having so many or thrombosis
171
Myelofibrosis is
fibrotic obliteration of bone marrow due to myeloproliferative disorder
see tear drop cells
172
Leukemoid reaction
key lab dif from CML
Acute inflammatory response to infection or other stimuli
increase WBC w/ PMNs and neutrophil precursors
HIGH leukocyte alkaline phosphatase vs CML
173
Multiple myelinoma usually produces large amounts of what?
4 major findings
IgG usually, some IgA
Hypercalcemia
renal failure (casts of Ig block the tubule)
Anemia (plasma cells interfere w/ production)
bone lytic lesions
174
rbc's stacked like poker chips
rouleaux formation - in multiple myelinoma
175
patient presents w/ hypercalcemia, lower back pain and fatigue. What might you find in the urine
Bence jones proteins ( but only if you do a urine protein (UPEP)
Multiple myelinoma
176
what is found on protein electrophoresis when patient has increased susceptibility to infection and primary amyloidsos (AL)
M spike
177
Waldenstroms macroglobulinemia
M spike and hyperviscosity w/ no lytic lesions
178
MGUS - monoclonal gammopathy of undetermined significance
monoclonal expansion of plasma cells w/ M spike
symptomatic precursor to multiple myelinoma
watch
179
Plasmocytoma
solid tumor of plasma cells
```
2 types
-solitary plasmocytoma of bone
extramedulary plasmocytoma (head neck and nose)
```
NO lytic lesion
