Hematology part 2 DIT - part 1 in endo Flashcards Preview

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Flashcards in Hematology part 2 DIT - part 1 in endo Deck (179):
1

protein responsible for the flexibility of RBCs

Spectrin

2

Anisocytosis

RBCs of varying size

3

Poikilocytosis

RBCs of varying shape

4

erythrocytosis

same thing as polycythemia

5

Life span of a RBC

120 days

6

Incompatible blood transfusion is due to what type of process

symptoms?

Type II HTN

hemolysis
renal failure
shock
death

7

erythroblastosis fettles is due to

prevented by? timeline of treatment (3)

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

acanthocytes are?

Seen when? (2)

irregularly spiked RBCs (vs regularly spiked RBCs in echinocytes)

liver disease
abetalipoproetinemia

9

Liver disease see what 2 changes to the RBCs

acanthocytes

target cells

10

Basophilic stippling of the RBCs indicates what (3)

Lead poisoning*

Thalassemia
anemia of chronic disease

11

Bite cells are seen in

G6PD deficiency - Macrophages removing oxydized hemoglobin (heinz bodies)

12

Elliptocytes are?

seen in

pencil shaped cells

hereditary elliptocytosis

13

schistocytes are?

seen in (3)

cut up RBCs

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

14

sideroblasts are?

differ from ringed siderblasts how?

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

Causes of ringed sideroblasts (4)

lead poisoning
drugs
genetic conditions
myelodysplastic syndromes

16

Sherocytes commonly seen in

hereditary spherocytosis

17

teardrop cells seen in

bone marrow infiltration; like myelofibrosis (squeezed out)

18

Target cells are seen in ((4)

THAL

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

19

Heinz bodies are?

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

Can be "bite out"

20

Howell jolly bodies are seen?

nucleated RBCs that are normally removed by the spleen

Seen in apslenic patients

21

Asplenic patients you may see(2)

howell jolly bodies
Target cells

22

HbA composition

normal adult hemoglobin - 97%
2 alpha
2beta

23

HbA2 composition

2 % of normal Hgb
2 alpha
2 delta

24

HbF composition

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

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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

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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

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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