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Hematology > Test 5 (Units 18-22) > Flashcards

Test 5 (Units 18-22) Flashcards

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1
Q

What are the two hallmark findings of hemolysis?

A
  • Decrease in lifespan of RBCs

- Increase in reticulocytes

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2
Q

Extravascular vs intravascular hemolysis: differences in lab findings

A

Extra: NEG for free hemoglobin and methemoglobin; spherocytes often present

Intra: POS for free hemoglobin and methemoglobin; schistocytes often present; decrease in haptoglobin, increase in LD

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3
Q

Classifying hemolytic anemias: Acute vs chronic

A 
Acute- Rapid onset
	-Isolated
	-Episodic 
	-Paroxysmic
Chronic-BM compensates
	-No symptoms
	-Can have hemolytic crises
	-BM may not be able to compensate chronically
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4
Q

Classifying hemolytic anemias: Inherited vs Acquired

A

Inherited- Mutant gene

Acquired- develop in previously non-hemolytic patients

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5
Q

Classifying hemolytic anemias: Intrinsic vs Extrinsic

A

Intrinsic- Abnormal RBCs (membrane defects, enzyme deficiencies, hemoglobinopathies)

Extrinsic- External agents (non-immune or immune)

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6
Q

Classifying hemolytic anemias: Intravascular vs extravascular

A

Intravascular- fragmentation

Extravascular- not in bloodstream; engulfed by macrophages

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7
Q

RBC destruction: laboratory findings

A
  • CBC: Spheroctyes, schistocytes, parasites, sickle cells, target cells and microcytes
  • Increased bilirubin and LD, especially in intravascular hemolysis
  • Decreased haptoglobin (intra) and hgb A1C
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8
Q

Increased erythropoiesis: laboratory findings

A
  • Increased retics
  • CBC: polychromasia, NRBCs, increased MCV (due to retics)
  • BM exam: usually not necessary; erythroid hyperplasia
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9
Q

Hereditary Spherocytosis: Etiology

A

-Lipid bilayer is disconnected from cytoskeleton in some places due to vertical interruption

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10
Q

Hereditary Spherocytosis: Pathophysiology

A

-Loose spots get picked out by spleen; loss of membranealtered surface rationspherocytesextravascular and intravascular hemolysis

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11
Q

Hereditary Spherocytosis: Clinical features

A
  • Level of anemia varies
  • Some splenomegaly
  • Jaundice
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12
Q

Hereditary Spherocytosis: Lab findings

A
  • Spherocytes
  • Polychromasia
  • Inc. retics
  • Inc. bilirubin
  • Inc RDW
  • Inc LD
  • Possibly inc MCHC
  • Dec haptoglobin
  • DAT neg
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13
Q

Hereditary Elliptocytosis and pyropoikilocytosis: Etiology

A

-Defect in cytoskeleton that cause lateral interruptions

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14
Q

Hereditary Elliptocytosis and pyropoikilocytosis: Pathophysiology

A

-Weakened stability stress from tight spaceselongation

Subtype: cells rupture at 41-45C instead of 49C (normal)sever anemia; will also see schistocytes and low MCV

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15
Q

Hereditary Elliptocytosis and pyropoikilocytosis: Clinical features

A
  • Mostly asymptomatic
  • Mod-severe in 10%
  • Number of elliptocytes does not correlate with severity
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16
Q

Hereditary Elliptocytosis and pyropoikilocytosis: Lab findings

A
  • Inc. retics
  • Inc. bilirubin
  • Inc LD
  • Dec haptoglobin
  • DAT neg
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17
Q

Hereditary Ovalocytosis: Etiology

A

-Band3 and ankyrin are very tight together

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18
Q

Hereditary Ovalocytosis: Pathophysiology

A

Increased rigidity resistance to invasion from Malaria

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19
Q

Hereditary Ovalocytosis: Lab findings

A

-Some RBCs have transverse ridges

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20
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Etiology

A

-CD55 and CD59 unable to protect cell from lysis by complement

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21
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Pathophysiology

A

Acquired stem cell mutation in GPI anchor proteins (X)lack of CD55/59no inhibition of complement spontaneous lysis of RBCs

Coexistence
Mosaicism
BM disfunction

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22
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Clinical features

A
  • Intravasular hemolysis (mildsevere)
  • Thrombophilia  risk of stroke and heart attack
  • concomitant with BM failure
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23
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Lab findings

A
  • Hemoglobinuria
  • Hemoglobinemia
  • Dec in haptoglobin
  • Inc in bilirubin (esp. unconj.)and LD
  • Hemosiderinuria
  • DAT neg
  • Iron deficiency anemia, folate deficiency due to increased need
  • Pancytopenia
  • Confirmation via flow cytometry: Type 1 cells- normal CD59, Type 2 cells- partial deficiency, Type 3 cells-no CD59
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24
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Types

A

Classic PNH: Over 50% neutrophils affected and marked hemolysis
PNH in setting of another specific BM disorder: <30% neutrophilia and mild-mod hemolysis

Subclinical PNH: <1% neutrophils, concomitant BM failure but no hemolysis

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25
Paroxysmal Nocturnal Hemoglobinuria (PNH): Treatment
- ECULIZUMAB (drug)-monoclonal ab that binds to complement to prevent spontaneous lysis but still have increased platelets and risk of BM failure) - Stem cell transplant - Supportive therapy: antibiotics, anticoagulants - Median survival: 10 more years - Cause of death: complications of thrombosis or pancytopenia ; also at risk for cancer
26
Overhydrated Hereditary Stomatocytosis
Increase in cationspuddles/ Increase in cell volume  decrease in viscosity; cells are fragile; NO SPLENECTOMIES (will lead to thromboembolic events)
27
Dehydrated Hereditary Stomatocytosis (Xerocytosis)-
K+ leaks out because the membrane is more permeable to K+ water followsincreased viscosity; More common; mild to moderate; usually no treatment; NO SPLENECTOMIES
28
Neuroacanthocytosis and types
neurologic symptoms; acanthocytes (never normal) - Abetalipoproteinemia- No b lipoprotein in bloodfat malabsorption and lipid transfer issues decrease in triglycerides and cholesterol fluid loss - McCleod syndrome- X linked; mild - Chlorea acanthocytosis- issues with genes that code for choreinproblems trafficking proteins; rare
29
Acquired stomatocytosis
Acute alcoholism, some medictions; 3-5% in all fields
30
Spur cell anemia
- Severe liver disease,severe hemolytic anemia excess cholesterol On membrane picked out by spleen spikes=acanthocytesjaundice, Splenomegaly
31
G6PD deficiency: Etiology
-X linked disorder that results in 140 different mutations which provides malaria protection and is often asymptomatic
32
G6PD deficiency: Pathophysiology
-Hemoglobin oxidized to methemoglobin -Inclusions made of denatured hemoglobin: Heinz bodies intravascular and extravascular hemolysis; preferential destruction
33
G6PD deficiency: Clinical features (types)
-Mostly asymptomatic 3 clinical manifestations: 1) Acute hemolytic anemia-abrupt onset after oxidative stress due to infections, certain drugs, fava beans 2) Neonatal jaundice (hyperbilirubinemia)-no symptoms at birth but 2-3 days later; can cause kernicterustoo much bilirubin build upmental retardation; give phototherapy, exchange transfusion 3) Chronic nonshperocytic hemolytic anemia-symptomatic all the time, rare
34
G6PD deficiency: Lab findings
- Mod to extremely sever hemolytic anemia - Some have aniso and poik, spherocytes, schistocytes - Heinz bodies - up to 30% retics - dec haptoglobin - inc in WBCs, indirect bili and LD - Hemoglobinuria - Hemoglobinemia - G6PD quantitative assays NOT during episode
35
Pyruvate Kinase deficiency: Etiology
- No pyruvate kinase - Most common deficiency in glycolysis pathway - Amish
36
Pyruvate Kinase deficiency: Pathophysiology
- Embden-Meyerhof pathway interrupted - Cannot turn glucose into lactate - mechanism unclear - Increase in 2,3 DPG (good thing)
37
Pyruvate Kinase deficiency: Clinical features
- Gallstones (constant inc flow in tubues) - Jaundice - Splenomegaly
38
Pyruvate Kinase deficiency: Lab findings
-Dec Hgb -In retics -Aniso and poik -Burr cells _measure enzyme levels -Molecular assays Treatment: transfusion or splenectomy
39
Microangiopathic hemolytic anemia: general pathophysiology
mechanical shearing RBC fragmentation schistocytes
40
Microangiopathic hemolytic anemia: Characteristic RBC morphology
- schistocytes occasionally, helmet cells
41
Microangiopathic hemolytic anemia: Clinical lab findings-
``` RBC fragmentation Hemolytic anemia Dec in H+H and haptoglobin Inc in retics, unconjugated bilirubin Urine urobilinogen ```
42
Thrombotic Thrombocytopenic Purpura (TTP): pathophysiology
VWF needed for high turbulence but Adams 13 is not there/able to cut after long stands block pathwaysplatelets attach Microthrombi-little clots
43
Thrombotic Thrombocytopenic Purpura (TTP): clinical symptoms
- Abrupt onset - Ischemia (organs starving bc not getting oxygen due to RBCs being shredded) - Hemolysis - Adults, more in females - Acquired and familial
44
Thrombotic Thrombocytopenic Purpura (TTP): laboratory findings
- Dec in platelets (often below 20) - Big inc in LD bc it is released by organs and RBCs - normal coag tests
45
Hemolytic Uremic Syndrome (HUS): pathophysiology
Shiga toxin from Ecoli0157 endothelium damage in kidneyacute renal failure
46
Hemolytic Uremic Syndrome (HUS): clinical symptoms
- Acute renal failure following gastroenteritis - Usually children - Supportive care and symptoms resolve spontaneously
47
Hemolytic Uremic Syndrome (HUS): laboratory findings
- Dec in platelets - Inc in creatine - Granular casts in urine - Proteinuria - Hemoglobinuria - Normal coag tests
48
HELLP Syndrome: pathophysiology
Placental vasculature abnormal (epithelial cells)platelets aggregate and deposit in vasculature and liverhemolysis
49
HELLP Syndrome: clinical symptoms
- Associated with pregnancy, pre-eclampsia - Deposits in liver - Delivery ASAP
50
HELLP Syndrome: laboratory findings
- elevated liver enzymes - Dec in platelets - Inc LD - Inc AST - Schistocytes - normal coag tests
51
What are the diagnostic lab results for HELLP Syndrome?
- Dec in platelets - Inc LD - Inc AST
52
Disseminated Intravascular Hemolysis (DIC): pathophysiology
Widespread abnormal activation of coagulation systembleeding and clotting all overischemia
53
Disseminated Intravascular Hemolysis (DIC): clinical symptoms
- Ischemia | - Associated with cancer, brain injury, crash injuries
54
Disseminated Intravascular Hemolysis (DIC): laboratory findings
- Depleted platelets and clotting factors - Schistocytes in 50% - D dimer POS (inc) - inc PT/PTT - dec fibrinogen
55
macroangiopathic hemolytic anemias: list 2
Traumatic Cardiac Hemolytic Anemia Exercise-Induced Hemoglobinuria
56
Traumatic Cardiac Hemolytic Anemia
-Cardiac valve disease or improperly working prosthetic heart valve causes shearing
57
Exercise-Induced Hemoglobinuria
- RARE - caused by long-distance running and strenuous hand drumming - Doesn’t usually cause anemia unless hemoglobinuria is severe - Diagnosis of exclusion - No treatment, minimize physical impact
58
Infectious agents capable of causing hemolytic anemias
- Malaria - Babesiosis - Clostridial sepsis - Bartonellosis SCHISTOCTYES AND SPHEROCYTES
59
RBC injuries capable of causing hemolytic anemias
- Drugs/chemicals - Venoms - Extensive burns
60
Immune hemolytic anemia definition
antibody mediated (allo, auto or against drugs) anemia; complement activation possible (classical pathway)
61
Immune hemolytic anemia general lab findings
- Dec hgb and haptoglobin - Inc retics, indirect bilirubin and LD - Normal to slightly increased MCV due to retics - Polys - Spherocytes (IgG) - Agglutination (IgM) - NRBCs - occ. schistocytes
62
Antibody general associations
- IgM: complement generally needed; usually extra and intra vascular hemolysis - IgG: generally extravascular
63
Immune hemolytic anemia: Factors influencing severity
- Ab characteristics (can it activate complement?, titer, specificity, affinity) - Ag it self (how many? Immunogenicity) - Patient (age, immune response, BM health)
64
Warm Autoimmune Hemolytic Anemia (WAIHA)
- Most common of this type - Idiopathic - Secondary to lymphoproliferative diseases, nonlymphoid neoplasms, autoimmune disorders, etc - IgG; extravascular
65
Warm Autoimmune Hemolytic Anemia (WAIHA): lab
-DAT POS in 95% -Blood bank: pan reactive -RBC lifespan as short as 5 days -Polychromasia and spherocytes Chronicsplenectomy Secondaryunderlying treatment
66
Cold Agglutinin Disease: what is it?
-Polyclonal antibodies in healthy individuals OR -Pathologic: high titers, monoclonal, can react at body temp -Idiopathic -Secondary to leukemia Acute CAD secondary to infectious mono or mycoplasma pneumo -IgM; extravascular; C -cold: IgM and C bound to RBC -warm: IgM disassociates -Mostly liver takes care of
67
Cold Agglutinin Disease: clinical finding
-Acrocyanosis (bluish nose and fingers)
68
Cold Agglutinin Disease: lab
- DAT (I) POS * *prewarm before analysis on instrument -50% need blood transfusion
69
Paroxysmal cold hemoglobinuria
- Severe but self-limiting - After respiratory infection - median age: 5 - Donath-Landsteiner ab - Auto anti-P - Biphasic - IgG; intravascular; C - Binds when cold and complement destroys intravascularly when temp increases
70
Paroxysmal cold hemoglobinuria: lab
- DAT POS for C3d | - NO TRANSFUSIONS
71
Mixed-Type Autoimmune Hemolytic Anemia
- Rare | - IgG and IgM;extravascular;C
72
Mixed-Type Autoimmune Hemolytic Anemia: lab
-DAT POS with IgG and C3d
73
Drug Induced Hemolytic Anemia
-Very rare; suspect after sudden dec in hgb (after put on new drug)
74
Drug Induced Hemolytic Anemia: mechanisms
Mechanisms: 1. Drug adsorption-destroyed by spleen or reshaped to sphero 2. Drug-RBC membrane protein immunogenic complex-intravascular 3. RBC autoantibody formation
75
Drug Induced Hemolytic Anemia: lab
- If drug-dependent, need drug or metabolite present for in-vitro detection - If non-immune, no ab produced membrane modification method - Discontinue drug use - If severe: transfusion or plasma exchange
76
Hemolytic Transfusion Reactions
``` Acute -within minuteshours -Most common cause: ABO incompatibility -complement; intra Delayed -often anamnestic response -Extravascular ```
77
Hemolytic Transfusion Reactions: lab
Acute: Hemoglobin and haptoglobin dec
78
Hemolytic Disease of the Newborn (HDN)
- IgG - Crosses placenta binds to fetal RBCs whch are pos for ag to which ab has been produced Severe anemia and possibly hydrops fetalis

Hematology (5 decks)

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