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USLME Kiss my ass and pray > Hematology part 2 DIT - part 1 in endo > Flashcards

Hematology part 2 DIT - part 1 in endo Flashcards

1
Q

protein responsible for the flexibility of RBCs

A

Spectrin

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2
Q

Anisocytosis

A

RBCs of varying size

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3
Q

Poikilocytosis

A

RBCs of varying shape

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4
Q

erythrocytosis

A

same thing as polycythemia

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5
Q

Life span of a RBC

A

120 days

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6
Q

Incompatible blood transfusion is due to what type of process

symptoms?

A

Type II HTN

hemolysis
renal failure
shock
death

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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
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8
Q

acanthocytes are?

Seen when? (2)

A

irregularly spiked RBCs (vs regularly spiked RBCs in echinocytes)

liver disease
abetalipoproetinemia

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9
Q

Liver disease see what 2 changes to the RBCs

A

acanthocytes

target cells

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10
Q

Basophilic stippling of the RBCs indicates what (3)

A

Lead poisoning*

Thalassemia
anemia of chronic disease

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11
Q

Bite cells are seen in

A

G6PD deficiency - Macrophages removing oxydized hemoglobin (heinz bodies)

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12
Q

Elliptocytes are?

seen in

A

pencil shaped cells

hereditary elliptocytosis

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13
Q

schistocytes are?

seen in (3)

A

cut up RBCs

Due to DIC, TTP/HUS, trauma (metal heart valve)

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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

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15
Q

Causes of ringed sideroblasts (4)

A

lead poisoning
drugs
genetic conditions
myelodysplastic syndromes

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16
Q

Sherocytes commonly seen in

A

hereditary spherocytosis

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17
Q

teardrop cells seen in

A

bone marrow infiltration; like myelofibrosis (squeezed out)

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18
Q

Target cells are seen in ((4)

A

THAL

Thalessemia
Hemaglobin C disease
Asplenia (sickle cell or post surgery)
Liver disease

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19
Q

Heinz bodies are?

A

oxidation of heme in the RBCs in G6PD deficiency -> benatured hemoglobin precipitates

Can be “bite out”

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20
Q

Howell jolly bodies are seen?

A

nucleated RBCs that are normally removed by the spleen

Seen in apslenic patients

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21
Q

Asplenic patients you may see(2)

A

howell jolly bodies

Target cells

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22
Q

HbA composition

A

normal adult hemoglobin - 97%
2 alpha
2beta

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23
Q

HbA2 composition

A

2 % of normal Hgb
2 alpha
2 delta

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24
Q

HbF composition

A

2 alpha
2 gamma chains

higher affinity for oxygen, less affinity for 2,3 DPG (allows placental transfer)

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25
Q

HbS composition

A

sickle cells
2 alpha
2 beta (glu -> valine)

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26
Q

HbC composition

A

hemoglobin C
2 alpha
2 beta (glu -> lys)

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27
Q

Hb barts composition

A

Severe thalassemia
no alpha
4 gamma

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28
Q

HbH compostion

A

Severe thalassemia
4 beta
no alpha

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29
Q

Fetal erythropeies occurs where (4)

timeline

A

young liver synthesizes blood

Yolk sac - 3-8 weeks
Liver* 6-birth
Spleen 15-30 wks
Bone marrow 22 wks - adult

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30
Q

Late adolesent/ adult erythropeisis occurs where specifically (3)

A

axilla skeleton

  • vertebra
  • pelvis
  • ribs
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31
Q

Early childhood erthyropoeisis occurs where specifically?

A

flat and long bones

-sternum, pelvis, ribs, cranial bones, vertebra, long bones

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32
Q

Lead poisoning interferes w/ these 2 enzymes

see what build up?

A

ferrochelatase
-protoporphyrin in the urine

aminolevulinic dehydratase (ALA dehydratase)
- aminolevulinic acid
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33
Q

can be given to inhibit porphyries and stop heme synthesis(2)

A

glucose and heme

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34
Q

induces heme synthesis

rate limiting step

A

alchohol, barbituates, seizure drugs, rifampin, metoclopramide

aminolevulinic acid synthase

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35
Q

acute intermittent porphyria is due to a defect in what enzyme

presents as (5)

A

pophobilinogen deamninase

Acute abdomen; acute neuropathy (heme substrates are neurotoxic)

Painful abdomen
port wine -colored urine
polyneuropathy
psychological disturbances
precipitated by drugs
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36
Q

patient’s started on metclopramide for GERD and begins to have a painful abdomen, and dark urine, also polyneuropathy and agitation

A

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
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37
Q

Porphyria cutanea tarda presents as ?

Due to defect where?

A

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

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38
Q

Presentation of lead poisoning?

A 
global ecephalopathy
memory loss
delirium
mental deterioration 
colicky pain
lead lines in gingiva and bone
foor/wrist drop
microcytic anemia
basophilic stippling
sideroblastic anemia
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39
Q

Causes of polycythemia (3)

A

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

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40
Q

4 tumors that can lead to ectopic EPO -> polycythemia

A

pheo
renal cell
hepatocellular carcinoma
hemangioblastoma

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41
Q

what test can be used to diagnose beta thalassemia minor

A

hemoglobin electrophoresis

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42
Q

Lab values of thalassemia minor

Ferritin
TIBC
Serum Fe

A

All are normal

may see target cells in peripheral smear

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43
Q

microcytic anemia, swallowing difficulties and glossitis

A

Plummer Vinson Syndrome

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44
Q

Microcytic anemia and >3.5% HbA2 level

A

Thalassemia minor

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45
Q

Megaloblastic anemia not correctible with B12 or folate

A

orotic aciduria (w/o the hyper ammonia)

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46
Q

Microcytic anemia reversible by B6

A

sideroblastic anemia

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47
Q

HIV positive patient w/ macrocytic anemia

A

AZT; zidovudine

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48
Q

normocytic anemia and elevated creatine

A

chronic renal disease

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49
Q

Hypersegmented nuetraphils be suspicious of

A

Folate or B12 deficiency

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50
Q

Causes of hyposchromic, microcytic anemia(4)

A

Fe deficiency
Thalassemia
lead poisoning
Sideroblastic anemia

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51
Q

Iron deficiency can be due to? (3)

A

chronic bleeding - CA-
malnutrition/absorbtion
increased demand -pregnant

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52
Q

Lab findings w/ Fe deficiency

Serum Fe
Ferritin
Transferrin
TIBC

A

low Fe in serum
low Ferritin
Increased transferrin ( trying to mobilize limited stock)
Decreased TIBC (not saturated at all)

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53
Q

Alpha thalassemia presentation (4) due to?

A

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 )

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54
Q

Populations at increased risk for alpha thalassemia

A

Africans - trans mutation

Asians - cyst mutation

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55
Q

beta thalasemia presentation (2)

A

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
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56
Q

2 diseases w/ marrow expansion

A

crew cut appearance on X ray

Sickle cell and Beta thalassemia

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57
Q

Population at risk for beta thalassemia

A

Mediterranean

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58
Q

Sideroblastic anemia due to ?

A

defect in heme synthesis -> ringed sideroblasts ( iron laden macrophages)

  • hereditary - X linked
  • reversible: alcohol, lead, INH, Rifampin

Rx w/ pyridoxine

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59
Q

Anemia due to chronic disease lab values

Serum Fe
Ferritin
Transferrin
TIBC

A

Serum - low

Ferritin - high (acute phase reactant)

transferrin- decreased

TIBC - normal

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60
Q

Hemachromatosis lab values

Serum Fe
Ferritin
Transferrin
TIBC

A

Serum - High
Ferritin - High
Transferrin - Low (body senses a lot and doesn’t want to mob)
TIBC - High ( a lot of saturation)

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61
Q

Pregnancy and OCP - Fe lab values

Serum Fe
Ferritin
Transferrin
TIBC

A

Serum - normal
Ferritin - normal
Transferrin - increased w/ estrogen
TIBC - Lower w/ excess binding sites

Transferrin production ramped up

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62
Q

Sideroblastic anemia Fe lab values

Serum Fe
Ferritin
Transferrin
TIBC

A

Serum - Increased (not using the Fe w/ defect in heme synth)

Ferritin - normal or increased
Trasnferrin -decreased, senses high serum
TIBC - is thus increased

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63
Q

Meagloblastic macrocytic anemia causes ( 3)

A

Impaired DNa synth -> increasing cytoplasm w/ ineffective nucleus maturation

B12 deficiency
Folate deficiency
Orotic aciduria

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64
Q

Folate deficiency due to (4)

Presentation (4)

A

malnutrition
-alcoholic
malabsorbtion

antifolates

  • methotexate
  • trimethoprin

increased requirement
-pregnanat

Presents w/

  • megaloblastic/macrocytic anemia
  • hyper seg PMNs
  • glossitis
  • homocystinuria w/o MMA increase
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65
Q

B12 deficiency due to (5)

Presentation

A 
inefficent uptake ( vegans)
malabsorbtion - crohns
pernicious anemia
Diphyllobothrium datum
PPIs

Hyperseg PMNs
glossitis
homocystinuria w/ increased MMA
Neuro symptoms (B12 used in myelin synth)

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66
Q

Which vitamin Def presents w/ neuro symptoms and megaloblastic anemia

A

B12 deficiency
- Neuro: Peripheral neuropathy, posterior columns, lateral corticospinal, dementia

need to be aware and not just give folic acid w/ megaloblastic anemia

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67
Q

Orotic acid in the urine and fatigued

Hyperammonia?

A

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

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68
Q

Non megaloblastic - Macrocytic anemia causes ((3)

A

DNA synthesis is unimpaired

Liver disease
Alcoholism - direct effect
Drugs
- 5 FU
- AZT
-hydroxyurea
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69
Q

Nonhemolytic normocytic anemia due to (3)

A

anemia of chronic disease

Aplastic anemia

Chronic kidney disease

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70
Q

Anemia due to chronic disease is due to what acute phase reactant pathology

A

an increase in hepcidin binds to ferroportin preventing the release of Fe out of macrophages

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71
Q

Aplastic anemia causes (4)

A

Radiation *
Viral: B19, EBV, HIV, HCV - especially if sickle cell
Fanconi’s Anemia - DNA repair defect
Idiopathic

-> pancytopenia

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72
Q

Pancytopenia bone histology(2)

A

hypocellular

bone marrow w/ fatty infiltration

73
Q

Symptoms of aplastic anemia (3)

A

anemia

  • fatigue
  • malaise
  • pallor

Leukoplakia
- Infection

thrombocytopenia
- purpura
-mucosal bleeding
petechiae

Stop the offending agent, transfuse, give G-CSF or GM -CSF

74
Q

Rx for anemia due to chronic kidney disease

A

EPO

75
Q

Basophilic nuclear remnants in RBCs

A

Howell Jolly body

Post splenctomy

76
Q

Increased LDH indicates

A

either extravascular or intravascular hemolysis

Decreased haptoglobin also -> intravascular
Increased uncongugated bilirubin -> extravascular

77
Q

Haptoglobin is?

A

molecule that binds to free hemoglobin found in the vasculature

decreased in intravascular hemolysis due to complex formation

78
Q

Intravascular hemolysis examples and lab findings

A

See decreased haptoglobin and increased LDH

Due to mechanical destruction, autoimmune, paroxysmal nocturnal hemoglobinuria

79
Q

Extravascular hemolysis examples and lab findings

A

RBCs removed from circulation and lysed
Examples: G6PD, hereditary spherocytosis, pyruvate kinase deficiency, sickle cell,

Increased LDH and uncongugated billirubin

80
Q

Hereditary Spherocytosis presentation (5)

Rx?

A

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
Q

aplastic crisis due to parvo B19 for example

A

Hereditary spherocytosis

Sickle cell

82
Q

G6PD deficiency is?

A

intravascular/Extravascular** normocytic anemia

X linked defect w/ less glutathione regeneration -> susceptibility to oxidative stress -> hemolytic anemia

83
Q

Pyruvate kinase deficiency pathophys and presentation

A

normocytic hemolytic anemia

Auto recessive defect leads to decreased ATP production and inability to maintain Na/K pump -> rigid cells that are lysed

84
Q

Hemolytic anemia in a newborn think of

A

pyruvate kinase deficiency

also could be IgG crossing over and attacking Rho +

85
Q

HbC mutation vs HbS mutation

A

glutamic acid -> valine on chrom 6 = sickle cell

glutamic acid -> lysine = HbC

86
Q

Paroxysmal nocturnal hemoglobinuria pathophys

Rx?

A

intravascular hemolytic anemia due to increased lysis by complement - lack CD 55/59 which is protective

eculizumab

87
Q

hemolytic anemia, pancytopenia and venous thrombosis

hink red urine in the morning

A

Paroxysmal nocturnal hemoglobinuria

88
Q

HAMs test is useful for

A

test for paroxysmal nocturnal hemoglobinuria

Low pH activates compliment

89
Q

Lack of CD55/59 leads to

A

paroxysmal nocturnal hemoglobinuria and red urine in the morning

90
Q

Why are newborns asymptomatic w/ sickle cell?

A

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
Q

splenic sequestration crisis in HbS is

A

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
Q

Presentation of sickle cell/ complications(6)

A 
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
Q

Rx for suckle cell (2)

A

bone marrow transplant

hydroxyureas=

94
Q

Extrinsic causes of normocytic hemolytic anemia(4)

A

Cause is outside of the RBC (Intrinsic means somethings wrong w/ the RBC)

Autoimmune
Microangiopathic anemia
Macroangiopathic anemia
infections

95
Q

Infections that may cause hemolytic anemia(2)

A

malaria

Babesia

96
Q

Macroangiopathic anemia causes (2)

A

extrinsic anemia -> schistocytes

prosthetic heart valves or aortic stenosis; big vessels cause

97
Q

Microangiopathic anemia causes

A

extrinsic anemia

Due to DIC, TTP-HUS, SLE and maligant HTN

(shear stress) or fibrin leads to schistocyte formation w/ obstructed narrowed lumens

98
Q

Cold agglutins vs Warm agglutins - autoimmune anemia

A

Cold
- IgM
Due to: mycoplasma or EBV or CLL

Warm
-IgG
Due to SLE, malignancies (CLL, non Hodgkins), congenital immune compl, drugs

99
Q

Cold agluttin anemia due to ? (3)

A
  • IgM
    Due to: mycoplasma or EBV or CLL

iGm bonds to RBCs -> complement fixation -> MAC lysis and opsinization

100
Q

Warm agglutin anemia due to? (4)

A

Warm
-IgG
Due to SLE, malignancies (CLL, non Hodgkins), congenital immune compl, drugs

101
Q

overlap in warm and cold anemia agglutinin causes?(2)

A

CLL

EBV

102
Q

Direct coombs test is?

Useful for(3)

A

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
Q

Indirect Coombs test is?

Useful for (2)

A

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
Q

platelets derived from ?

last?

A

megalokaryocytes

no nucleus

come preformed w/ ganules containing: Ca, vWF, COX, TXA2, thrombin, fibrinogen, PDGF, lysosome enzymes

Lasts 8-10 days

105
Q

vWF receptor

fibrinogen receptor

A

GpIb

GpIIb/IIIa

106
Q

Von willibrand factor is found where?(2)

2 jobs?

A

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
Q

3 steps in platelet plug formation

A

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
Q

Endothelial cells contain(4)

A

vWF*
thromboplastin
tPA
PGI2

109
Q

ASA roll on PT and PTT

A

none just irreversible blocks COX1 promotion of TXA2 formation decreasing platelet plug formation and increasing bleeding time

110
Q

ADP receptor inhibitors (4)

A

Clopidogrel
Ticlopidine
prasugrel
ticagrelor

111
Q

Uses for ADP receptor inhibitors (4)

A

acute coronary syndrome
post stent
reduce incidence or recurrence of stroke
ASA allergy?

112
Q

GP IIb /IIIa inhibitors (3)

A

abcliximab

eptiFIBate
tiroFIBan

think about use in non ST elevated MI

113
Q

Uses of G IIb/IIIa use

A

Non ST elevated MI

percutaneous transluminal coronary angioplasy

114
Q

Platelet disorders are characterized by what symptoms(4)

A

microhemorrage

mucous membrane bleeding,
epistaxis
petechia - >purpura
increased bleeding time

115
Q

Immune Thrombocytopenic purpura pathophys?

presentation?

A

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
Q

Rx for ITP?

A

immune suppression
splenectomy - more platelets in circulation
IVIG
steriods

117
Q

Thrombotic thrombolytic purpura (TTP) 5 clinical manifestations

A

Nasty Fever Torched His Kidneys

Neuro changes
Fever
Thrombocytopenia*
Hemolysis* -microangiopathic due to fibrin/Complement act
Kidney failure*
  • seen in hemolytic uremia syndrome
118
Q

How is HUS related to TTP

Occurs when?

A

Similar presentation of thrombocytopenia, Uremia/kidney failure, hemolysis

NO neurological changes or fever as in TTP

associated w/ post O127 H7 in kids

119
Q

Pathogenesis of TTP

A

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
Q

Schistocytes, fever, petchial rash and increased bleeding time be concerned w/

A

Thrombotic Thrombocytopenic Purpura

121
Q

Bernard Soulier syndrome pathogenesis

labs?

A

defect in GpIb -> prevention of initial platelet adhesion

decreased platelet count due to slightly enlarged platelets being removed and increased bleeding time

122
Q

Glanzman thrombasthenia

labs?

A

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
Q

von willebrand disease pathogenesis

Labs?

PC
BT
PT
PTT

A

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
Q

Rx for vWF deficiency

A

Desmopressin - (ADH analog) increase the release vWF from the enthelium

125
Q

Pathogenesis of DIC

Labs
PC
BT
PT 
PTT
A

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
Q

schistocytes and increased bleeding time disorders (2)

A

TTP

DIC

127
Q

causes of DIC (6)

A

STOP Making Thrombin

Sepsis
Trauma
Obstetrical problems - placenta abruptous/amniotic emboli
Pancreatitis (acute)
Malignancy
Transfusions
128
Q

most common lymphoma in the US?

A

Diffuse B cell lymphoma

129
Q

lymphoma associated w/ EBV(2)

A

burkitts lymphoma (NHL)

50% w/ hodgkins

130
Q

Lymphoma associated w/ long term celiac disease

A

intestinal T cell lymphoma

131
Q

Lymphoma equivalent to CLL

A

Small lymphocytic lymphoma

132
Q

Most common non hodgkins lymphoma in kids?

A

lymphoblastic lymphoma

133
Q

lymphoma associated w/ sjogrens, hashimotos and H pylori

A

marginal cell maltoma

134
Q

cervcal painless LAD of the neck w/ fever, weightless and Nightsweats

A

hodgkins lymphoma

135
Q

Leukemia vs Lymphoma

A

Leukemia originates/involves the bone marrow and tumor cells found in the peripheral blood

Lymphoma arises from lymph nodes

136
Q

LAD presentation in hodkins lymphoma

A

painless non-tender LAD in a single group of nodes, can also be in the mediastinum; firm and rubbery

contiguous spread

137
Q

Age distribution of hodgkins lymphoma vs nonhodgkins lymphoma

A

Hodgkins - bimodal 20s and 60s

Nonhodgkins is more variable 20-60

138
Q

Constitutinal signs/ B symptoms are (3) associated w?

A

hodgkins lymphoma

Fever, weight loss light sweats

puritis also may be an issue

139
Q

Spread and presentation of nonhodgkins lymphoma

A

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
Q

Reed stern berg cells are seen in?

Derived from?

A

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
Q

Subtypes of hodgkin’s lymphoma (4)

A

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
Q

nodular sclerosing subtype of hodgkins lymphoma

histology

A

most common subtype of bodkins

Bands of collagen, sclerosis and fibrosis

143
Q

Non hodgkin lymphoma and mutation

t8: 14
t11: 14
t14: 18

A

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
Q

Burkett lymphoma associated w?

3 types

A

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
Q

Diffuse large cell B lymphoma

A

most common in adults

most common overall in the US

146
Q

Mantle cell Lymphoma translocation

A

t11:14; cyclin D

poor prognosis, B cell

147
Q

Follicular cell lymphoma translocation

A

t14:18; bcl 2

adults; indolent course, hard to Rx

148
Q

T cell non- hodgkins lymphoma(3)

A

adult t cell lymphoma aw/ HTLV 1

Mycosis fungoides -> sezary

Intestinal T cell lymphoma

149
Q

Cutaneous lesion that is aggressive, patient is from Japan and has a history of HTLV1 infection

A

Adult T cell lymphoma

can also be caribbean and west african in origin

150
Q

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?

A

Mycosis fungoides

sezary

151
Q

Acute leukemia characterized by?

smear?

bone marrow?

A

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
Q

ALL presentation

PAS?

age?

A

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
Q

TdT positive in what leukemia?

A

ALL

PAS + also

154
Q

AML presentation

PAS?

Age

A

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
Q

Risk factors for AML

A 
Radiation
benzene
alkylating agents
myeloproliferative disease (like polycythemia vera)
mlyelodysplastic syndrome 
aplastic aneima
downs as well (ALL)
156
Q

Chroic leukemia characterisics

A 
insiderous onset
mature cells (<5%blasts)

associated w. hepatospelnomegaly and LAD

prominent infiltration of bone marrow and peripheral WBC counts may be high

157
Q

CLL presentation

characteristic feature?

Age

A

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
Q

Smudge cells are seen in

A

CLL

159
Q

CML

age and presentation

Charcteristic feature

A

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
Q

Leukocyte alkaline phosphatase (-) cells; numerous w/ mature basophils and PMNS

A

CML

161
Q

t15:17 translocation

rx?

A

M3 AML

See more Auer rods

Rx al trans retinoic acid

162
Q

Monoclonal antibody spike think of 3 things

A

Multiple myeinoma
Waldenstroms macroglobinemia
MGUS - monoclonal gammopathy of undetermined significance

163
Q

leukemia commonly presents w/ bone pain

A

ALL

164
Q

myleodysplastic syndrome

can see?

A

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
Q

Tartate resistant acid phosphatase

A

stain that is positive in hairy cell leukemia,

a maute b cell tumor in the elderly

166
Q

imatinib is used to treat?

A

CML

167
Q

Myeloproliferative disorders(4)

A

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
Q

Jak 2 mutation is associated w?

A

myeloproliferative disorders

especially polycythemia vera

169
Q

Symptoms of polycythemia vera(5)

EPO?

A 
plethora - red face
itching after a shower
splenomegaly
hypervisous blood
erythromyalgia

LOW epo - not stimulated by outside

170
Q

Essential thrombocytosis is?

presentation

A

myeloproliferative disorder of megalokaryocytes

have LOW thrombopoeitin
bleeding w/ poor platelet function despite having so many or thrombosis

171
Q

Myelofibrosis is

A

fibrotic obliteration of bone marrow due to myeloproliferative disorder

see tear drop cells

172
Q

Leukemoid reaction

key lab dif from CML

A

Acute inflammatory response to infection or other stimuli

increase WBC w/ PMNs and neutrophil precursors

HIGH leukocyte alkaline phosphatase vs CML

173
Q

Multiple myelinoma usually produces large amounts of what?

4 major findings

A

IgG usually, some IgA

Hypercalcemia
renal failure (casts of Ig block the tubule)
Anemia (plasma cells interfere w/ production)
bone lytic lesions

174
Q

rbc’s stacked like poker chips

A

rouleaux formation - in multiple myelinoma

175
Q

patient presents w/ hypercalcemia, lower back pain and fatigue. What might you find in the urine

A

Bence jones proteins ( but only if you do a urine protein (UPEP)

Multiple myelinoma

176
Q

what is found on protein electrophoresis when patient has increased susceptibility to infection and primary amyloidsos (AL)

A

M spike

177
Q

Waldenstroms macroglobulinemia

A

M spike and hyperviscosity w/ no lytic lesions

178
Q

MGUS - monoclonal gammopathy of undetermined significance

A

monoclonal expansion of plasma cells w/ M spike

symptomatic precursor to multiple myelinoma

watch

179
Q

Plasmocytoma

A

solid tumor of plasma cells

2 types 
-solitary plasmocytoma of bone
extramedulary plasmocytoma (head neck and nose)

NO lytic lesion

USLME Kiss my ass and pray (33 decks)

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