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Pathophys Semester 2 > Hematopathology- Schoenwald > Flashcards

Hematopathology- Schoenwald Flashcards

1
Q

RBC disorders: Describe the very basic pathology of anemia.

A

A decrease in RBCs

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2
Q

RBC disorders: What are the three mechanisms of anemia?

A
  • Blood loss (acute or chronic)
  • Decreased production of RBC
  • Increased destruction of RBC
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3
Q

RBC disorders: Describe hemolysis

A

Destruction of RBCs

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4
Q

RBC disorders: How do you evaluate for anemia?

A

Size and hemoglobin concentration (MCV and MCHC)

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5
Q

Classification of anemias: In blood loss (acute) what will you see on evaluation of the RBCs? (initially and later).

A
  • Initially: normocytic normochromic

- Later: Hypochromic, reticulocytosis

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6
Q

Ex’s of blood loss (acute) that can cause anemia? (list 3)

A
  • Acute volume depletion.
  • RBC reduction due to fluid influx
  • Rapid RBC regeneration
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7
Q

classification of anemias: RBCs of blood loss (chronic)?

A

Hypochromic (means the RBC’s have less Hgb than normal)

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8
Q

Classification of anemias: Characteristics of blood loss (chronic)?

A

Reduced Iron stores

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9
Q

lassification of anemias: What types of anemia are under the category of increased destruction?

A
  • Hemolytic anemias
  • Immunologic
  • Mechanical
  • Hereditary
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10
Q

Classification of anemias: Characteristics of hemolytic anemias?

A
  • Features of hemolysis

- Splenomegaly

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11
Q

Classification of anemias: Characteristics of immunologic anemias?

A
  • Autoantibodies against RBC
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12
Q

Lab finding (pertaining to cells) associated with immunologic anemias?

A
  • Reticulocytosis
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13
Q

Classification of anemias: RBCs of Mechanical anemias?

A

Shistocytes

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14
Q

classification of anemias: examples of mechanical anemias?

A
  • HUS (hemolytic uremic syndrome)
  • TTP (thrombocytopenic purpura)
  • Trauma-valve
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15
Q

Classification of anemias: RBC (types) of hereditary anemias? (list 5)

A
  • Spherocytosis
  • Elliptocytosis
  • Sickle cell
  • Hypochromic
  • Microcytic
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16
Q

Classification of anemias: Characteristics of hereditary anemia?

A
  • RBC membrane and cytoskeleton deficiency
  • Hemoglobinopathies: sickle cell, thalassemia
  • Enzyme deficiencies
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17
Q

Classification of anemias: What types of anemia are categorized as deficient erythropoiesis?

A
  • “Stem cell” defect

- Maturation defect

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18
Q

Classification of anemias: RBCs of “stem cell” defect anemia?

A
  • Normocytic, normochromic

- Normocytic, mildly macrocytic

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19
Q

Classification of anemias: Examples of “stem cell” defect anemias (list 2)

A
  • Aplastic anemia

- Pure red cell aplasia

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20
Q

Classification of anemias: RBCs of maturation defect anemia?

A
  • Megaloblastic
  • Anisocytic
  • Hypochromic
  • Poikilocytic
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21
Q

Classification of anemias: Characteristic of maturation defect anemia?

A
  • Pernicious anemia
  • Folate deficiency
  • Iron deficiency
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22
Q

Mechanisms of anemia: Examples of blood loss that cause anemia?

A
  • Acute: trauma

- Chronic: GI, malignancy, menstrual bleeding

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23
Q

What are schistocytes?

A

fragmented RBCs

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24
Q

Mechanisms of anemia: Examples of external factors that could increase destruction of RBCs?

A
  • Ab mediated transfusion Rx
  • Erythroblastosis fetalis
  • Autoimmune
  • Trauma to RBC
  • Infection
  • Sequestration in spleen.
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25
Q

Mechanisms of anemia: Examples of hereditary internal factors that could increase destruction of RBCs?

A
  • Cytoskeleton defect (hereditary spherocytosis)
  • Structurally abnormal Hgb (sickle cell, thalassemia)
  • Enzyme deficiency (G6PD)
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26
Q

Mechanisms of anemia: Other than external and hereditary internal factors, what are other sources of increased destruction of RBCs?

A
  • Acquired defect
  • Paroxysmal nocturnal hemoglobinuria (= an acquired, life-threatening disease in which hemolysis, thrombosis, and anemia occur)
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27
Q

Describe paroxysmal nocturnal hemoglobinuria

  • Etiology?
  • characterized by which 3 things?
A

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired, life-threatening disease of the blood. The disease is characterized by destruction of red blood cells (hemolytic anemia), blood clots (thrombosis), and impaired bone marrow function (not making enough of the three blood components).

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28
Q

Paroxysmal nocturnal hemoglobinuria: Red cells are more prone to lysis by what mechanism?

A

Complement

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29
Q

Paroxysmal nocturnal hemoglobinuria: Condition results in what venous condition?

A

venous thrombosis

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30
Q

Paroxysmal nocturnal hemoglobinuria: CLinical signs?

A
  • Episodic hemoglobinuria, 1st morning urine.
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31
Q

Paroxysmal nocturnal hemoglobinuria: How does venous thrombosis manifest?

A
  • Hepatic
  • Portal
  • Mesenteric
  • Cerebral veins. (Harry potter makes cake)
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32
Q

Mechanisms of anemia: How can decreased production of RBCs occur?

A
  • Disturbance of stem cells
  • Defective heme production
  • Defective DNA production
  • Destruction of bone marrow
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33
Q

Mechanisms of anemia: What is a condition that can cause a disturbance of stem cells?

A

aplastic anemia

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34
Q

Mechanisms of anemia: What are conditions that can cause a defective heme production? (list 2 ex’s)

A
  • IDA

- thalassemia

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35
Q

Mechanisms of anemia: What is a condition that would lead to defective DNA production?

A
  • Vitamin B12 deficiencies

- Folic acid deficiencies

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36
Q

Mechanisms of anemia: What is a condition that could lead to a destruction of bone marrow?

A

metastatic tumor

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37
Q

Sites of removal of RBC from circulation: Extravascular site(s)?

A
  • Phagocytic systems of spleen and liver
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38
Q

Sites of removal of RBC from circulation: Complications?

A
  • Hemosiderin deposition in organs

- Jaundice and gallstones

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39
Q

Sites of removal of RBC from circulation: Why could jaundice and gallstones occur as a complication?

A

Results from elevated unconjugated bilirubin associated with hemolysis

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40
Q

Sites of removal of RBC from circulation: Intravascular source?

A
  • Destruction of RBC in the vessel
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41
Q

Sites of removal of RBC from circulation: Intravascular complications?

A
  • Acute tubular necrosis

- Jaundice and gallstones –> can occur due to increased unconjugated bilirubin

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42
Q

Why could acute tubular necrosis occur as a complication of intravascular removal of RBCs?

A

Due to hemoglobinemia

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43
Q

Lab findings in hemolysis of RBCs: Hgb/Hct?

A

Hgb decreased, Hct decreased

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44
Q

Lab findings in hemolysis of RBCs: Haptoglobin?

A
  • Initially: increase

- Over time (net): Decreased

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45
Q

Lab findings in hemolysis of RBCs: Wy does haptoglobin decrease over time?

A

Because it keeps binding to that free heme circulating.

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46
Q

Lab findings in hemolysis of RBCs: LDH?

A

Increased lactate dehydrogenase (LDH) (it’s a liver enzyme and its inside RBCs)

-and increased unconjugated bilirubin

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47
Q

Lab findings in hemolysis of RBCs: K+?

A

Increased potassium (since cells are breaking down and releasing potassium)

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48
Q

What conditions might you see spherocytes with?

A
  • Hereditary spherocytosis

- Immune hemolysis

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49
Q

What conditions might you see schistocytes with?

A
  • TTP

- HUS

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50
Q

What conditions might you see target cells with?

A

hemaglobinopathies

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51
Q

What conditions might you see teardrops with?

A

Myelofibrosis

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52
Q

What conditions might you see sickle cells with?

A
  • Sickle cell

- Thalassemia minor with sickle cell

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53
Q

What conditions might you see Rouleaux with?

A

Rouleaux= “stacking of coins”–> - due to Increased globins or decreased albumin (multiple myeloma)

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54
Q

Clinical presentation of anemia?

A
  • Pale skin
  • Fatigue
  • Shortness of breath
  • Chest pain
  • Syncope and dizziness
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55
Q

Lab values associated with an initial anemia assessment?

A
  • MCV

- Reticulocyte count

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56
Q

What does an increased reticulocyte count imply?

A
  • Bone marrow intact and able to produce RBC

- Acute blood loss or hemolysis

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57
Q

What does a decreased reticulocyte count imply?

A
  • Primary bone marrow disorder
    OR
  • Deficiency in building blocks (iron, B12, folate)
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58
Q

MCV values: What are the categories of MCV?

A
  • Microcytic
  • Normocytic
  • Macrocytic
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59
Q

MCV values: Microcytic?

Normocytic? Macrocytic?

A

Micro- < 80 fl
Normo- 80-100 fl
Macro- >100 fl

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60
Q

What are the microcytic anemias?

A
  • Iron Deficiency
  • Thalassemia
  • Late stage anemia of chronic disease
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61
Q

Iron deficiency anemia: MCC in US? (list MC cause of blood loss in the US vs outside the US)

A
  • GI blood loss or menstrual blood loss

- -Outside of US- due to poor nutrition

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62
Q

Iron deficiency anemia: Additional causes of IDA?

A
  • Malabsorption

- Increased demand (pregnancy)

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63
Q

IDA labs: What would you look at?

A
  • Ferritin
  • Serum iron
  • Iron stores in bone marrow
  • TIBC
  • RDW
  • Transferrin saturation
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64
Q

IDA Labs: Results

A
  • Decreased ferritin (less than 30 is diagnostic of IDA))**
  • Decreased serum Iron
  • Decreased iron stores in bone marrow
  • Decreased transferrin saturation(ratio of serum iron to TIBC
  • Increased TIBC
  • Increased RDW
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65
Q

IDA labs: What is normal transferrin saturation?

A

> 20%

66
Q

IDA labs: What would the transferrin saturation look like in IDA?

A

<10%

67
Q

Thalassemia–> is characterized by a deficient production of Hgb due to ______

A

–> mutation or complete loss of globin chain

-There are 4 copies of the alpha globulin chain and 2 copies of beta globulin.

68
Q

Alpha Thalassemia: Due to the complete loss of ____?

A

—>1 or more of the 4 copies of the alpha globulin chain gene

69
Q

Alpha thalassemia:

-Loss of 1 copy of the gene= ?

A

silent (carrier)

70
Q

Alpha thalassemia: loss of 2 copies=

A

alpha thalassemia trait (asymptomatic but MCV low)

71
Q

Alpha thalassemia: loss of 3 copies=

A 
HbH disease (marked anemia)
**these pts have Sx
72
Q

Alpha thalassemia: loss of 4 copies=

A

hydrops fetalis (stillborn)

73
Q

Alpha thalassemia:

-demographic?

A

Prevalent in Africans and Asians

74
Q

T/F: alpha thalassemia usually requires blood transfusions

A

False!! usually does not require blood transfusion

75
Q

Describe Beta Thalassemia

A

Malfunction of gene due to mutation which result in **complete loss of the Hgb protein
**Alpha chains are insoluble and result in intravascular hemolysis and ineffective erythropoiesis

76
Q

Demographic: Beta thalassemia

A

Mediterranean descent

77
Q

Beta thalassemia: Major

  • describe this condition?
  • requires ?
  • Onset?
A

=Skeletal abnormalities due to bone marrow expansion

  • -Usually onset after 6 months of age due to loss of Hgb F and transition to Hgb A
  • Tx: Requires life-long blood transfusions due to severe anemia
78
Q

Beta Thalassemia: Minor

  • Describe this condition? (hgb and MCV values)?
  • Requires?
A

Hgb and MCV disproportionate-hgb 10/MCV 55

-Usually does not require transfusions

79
Q

Beta Thalassemia: Labs?

A
  • Decreased MCV (microcytic!!!! less than 80 fl)
  • Decreased hgb
  • Anisocytosis (change in size)
  • Poikilocytosis(change in shape)
80
Q

Beta Thalassemia: Poikilocytosis shows?

A

-microcytes, target cells

81
Q

Anemia of Chronic disease: anemia that occurs in the background of ______

A

chronic disease (ie Lung carcinoma, Hodgkin lymphoma, rheumatoid arthritis, infection-TB)

82
Q

Anemia of chronic disease: Labs- initially? Late stage?

A

Initially, normocytic,

-late stage can be microcytic

83
Q

Anemia of Chronic Disease: Labs (elevated lab values and decreased)

A

Elevated: levels of storage iron, **increased Ferritin

Decreased: serum iron & decreased TIBC

84
Q

Describe Anemia of chronic disease:

  • what happens with iron?
  • EPO?
A
  • ->Cannot transfer iron from phagocytic cells to erythroid precursors
  • Through TNF and interferons erythropoietin is decreased
85
Q

In a Pt with anemia of chronic disease, what protein is stimulated?

A

**Hepcidin is stimulated

Hepcidin= protein that is key regulator of the entry of iron into circulation
–>**impairs release of iron from storage

86
Q

IDA: lab values

A

Elevated: TIBC
Decreased: **Ferritin, Iron, Transferrin saturation

87
Q

Thalassemia: lab values

A

All normal

**thalassemia is NOT an iron issue, it’s a Hgb issue

88
Q

Anemia of chronic disease: lab values

A

Elevated: Ferritin, transferrin saturation

Decreased: Iron, TIBC

89
Q

Macrocytic Anemia: describe this condition

  • Vitamin ___ deficiency?
  • MCV?
  • describe what happens to DNA
  • DNA shows ______
A

**Vitamin B12 deficiency

-MCV >100
=Impaired DNA synthesis involving all bone marrow precursors, not just RBC
-Impairment of
DNA slows nuclear maturation but NOT cytoplasmic

90
Q

Vit. B12 is needed for?

A

(cyanocobalamin)
=for regeneration of tetrahydrofolate–> giving folate will correct a B12 anemia but does not treat the neurologic findings

91
Q

A Pt presents with MCV >100. what labs should you check?

A

for macrocytic anemia work up you must check B12 levels, homocysteine and MMA levels

92
Q

vit. B12 deficiency causes elevated levels of?

A

**homocysteine and methyl-malonic acid (MMA)

93
Q

Complications of B12 deficiency

A
  • Anemia, thrombocytopenia and leukopenia
  • Ineffective erythropoiesis
  • Neurologic deficiencies
94
Q

Describe why B12 deficiency causes ineffective eythropoiesis

A

Adequate number of RBC precursors but many of them undergo apoptosis in bone marrow due to impaired nuclear maturation

95
Q

List specific neurologic deficiencies that stem from B12 deficiency

A

=Peripheral neuropathy and spinal cord pathology

**note: this is NOT seen in Folate deficiency

96
Q

Causes of B12 deficiency

A
  • **Pernicious anemia
  • Nutrition (rarely)
  • Gastrectomy
  • Ileal disease
  • Diphyllobathrium latum infection –fish tapeworm
97
Q

What is Pernicious Anemia

A

=Condition is due to antibodies against parietal cells or blocking antibodies or antibody against B12-IF(intrinsic factor) complex
-Autoimmune gastritis, loss of parietal cells that causes loss of intrinsic factor

(Parietal cells are in the stomach (so if they are attached, you don’t have as much B12 absorption In the stomach)

98
Q

Clinical presentation of B12 deficiency

A
  • **Pancytopenia (Defect is not just limited to RBC, you will see a decrease in all cells growing rapidly)
  • Loss of intestinal cells–> diarrhea and malabsorption
  • Peripheral neuropathy
99
Q

B12 deficiency: KEY Lab findings

A

-Hypersegmented neutrophils on CBC (due to DNA impairment)

100
Q

Folate deficiency: important differences from B12 deficiency

A

aka vitamin B9 deficiency–> megaloblastic anemia

  • Folate deficiency does NOT cause neurologic deficits
  • **Often due to dietary deficiency(US)
  • ->**alcoholism
  • Folate deficiency is NOT associated with pernicious anemia
101
Q

Aplastic Anemia: describe this condition

A

=Decreased Production of cells, -it’s not associated with MCV

  • Anemia is due to absence of RBC in bone marrow
  • Also involves other hematopoietic cells
  • Likely due to suppression of stem cell function
102
Q

T/F: splenomegaly is not seen with aplastic anemia

A

True, there is NO splenomegaly

103
Q

What is a big problem associated with aplastic anemia?

A

Can convert to leukemia

104
Q

Aplastic anemia: causes

A

-Idiopathic (50%)

  • Secondary to drug therapy:
  • Alkylating agents- ie chemotherapy
  • Chloramphenicol-antibiotic (old)
  • Benzene
  • Infections: parvovirus B19 (slpa cheek appearance), Epstein Barr Virus, HIV
  • Radiation
  • Genetic
105
Q

Hemolytic Anemias ALL have:

A
  • Increased RBC destruction(intravascular or extravascular
  • Increased erythropoiesis
  • Increased iron deposition in tissues-spleen and liver
  • Some cases have pigment gallstones (from increase in bilirubin
106
Q

Hemolytic Anemias: Intravascular Hemolysis–> Key lab findings

A

-Decreased haptoglobin

  • Increased unconjugated(indirect) bilirubin
  • Increased LDH-markedly
  • **Positive urine hemosiderin and urine hemoglobin (brown urine)
107
Q

Hemolytic Anemias: Extravascular Hemolysis–> Key lab findings

A
  • Normal to slightly decreased haptoglobin (not as marked as with intravascular)
  • Increased unconjugated (indirect)bilirubin-usually more than intravascular
  • Normal to slightly increased LDH
  • **Negative urine hemosiderin and urine hemoglobin
108
Q

What is the mechanism of increased unconjugated bilirubin in hemolysis?

A

Macrophages phagocytize RBCs–> inside the macrophage the RBC is degraded in lysosomes (heme + globin–> AA’s) Heme–> Fe and Porphyrin. Porphyrin–> unconjugated bilirubin–> exits macrophage and combines with albumin–> goes to hepatocyte–> unconjugated bilirubin–> is converted to conjugated bilirubin—> exits into biliary system(= gallbladder and bile ducts inside and outside the liver)
-these Pts have discoloration of stool and urine

Unconjugated and conjugated= total bilirubin

109
Q

haptoglobins = _______ produced by the liver

A

Glycoproteins produced by liver

110
Q

Haptoglobin: fx

A

Powerful free hemoglobin binding proteins

111
Q

What happens to haptoglobin in hemolytic anemias?

A
  • In hemolytic anemia, large release of free Hgb by breakdown of RBC, haptoglobin quickly binds to free hgb, and is rapidly broken down
  • ->***Results in decrease in haptoglobin as liver is unable to rapidly compensate to make more haptoglobin
112
Q

Mechanism of Hemolytic anemia: Congenital?

A
  • Enzyme deficiency (G6PD)
  • Defects of cytoskeleton (sperocytosis)
  • hemoglobinopathies (sicke cell and thalassemia)
113
Q

Mechanism of Hemolytic anemia: Acquired?

A
  • Antibody induced

- mechanical

114
Q

Coombs Testing: Direct Coombs

A

aka direct antiglobulin test

  • Antibodies causing hemolysis are on the surface of the RBC
  • In lab, add antibody against IgG(on RBC)=agglutination of cells
115
Q

Difference b/w direct coombs and indirect coombs test

A
  • Direct coombs tests the Pts RBCs looking for hemolytic anemia, while Indirect coombs checks the Pts plasma, checking for antibodies that will react with transfusions
  • Direct coombs is more important for hemolytic anemias (the test will be Positive— indicating there are antibodies causing hemolysis on the surface of the RBC)

How to perform a direct coombs: in a lab, add antibody against IgG (onto RBCs)- if there is agglutination of the cells= + test

116
Q

Describe and Indirect Coombs Test

A

-aka indirect antiglobulin test

  • Antibodies causing hemolysis are in the plasma but not on the RBC surface
  • In lab, serum tested with known RBC ag that antibody reacts to then antibody added=agglutination

-Indirect coombs: antibodies causing hemolysis are in the PLASMA–> these tests are checking antibodies in the Pts plasma that could react with a transfusion (blood transfusion)

117
Q

Antibody mediated destruction of RBCs: Isohemagglutinin

A

isohemagglutinin= what happens in RBC transfusion reactions (ie ABO or other type incompatibility, Rh positive

  • RBC transfusion reactions-ABO or other blood type incompatibility
  • Ertyhroblastosis fetalis–> RH incompatibility
118
Q

Antibody mediated destruction of RBCs: Autoimmune (list 2 examples of anemias)

A
  • Warm autoimmune anemia

- Cold autoimmune anemia

119
Q

ABO transfusion reaction: antibody type is ____ mediated

A

IgM

120
Q

An ABO transfusion reaction indicates an _____ _____

A

Intravascular hemolysis

121
Q

ABO transfusion reaction leads to: (list complications)

A
  • Intravascular hemolysis
  • Hemoglobinemia–> leading to acute renal failure & HIGH mortality rate

(all the Hgb that was released, and decreased heme carrying ability leads to acute renal failure and high mortality)

122
Q

ABO transfusion reaction: which antibodies are naturally occuring?

A

Anti A and Anti B IgM naturally occurring

123
Q

Review Slide 43

A

on ABO typing

124
Q

Non ABO transfusion reaction: antibody type?

-is usually which type of hemolysis?

A
  • usually IgG
  • **Extravascular hemolysis–> antibody binds to RBC then complex is removed by spleen
  • Usually requires previous exposure to non ABO antigen (example Rh factor)
125
Q

What Sx is associated with Non ABO transfusion reaction?

A

Jaundice

(my notes: Non ABO transfusion reactions= usually not as lethal as ABO transfusion reactions BUT you do see a lot of jaundice)

126
Q

Erythroblastosis Fetalis: describe this condition

A

=Maternal antibody against a fetal red
-blood cell antigen
Rh factor (made up of DCE ag) D potential for most potent reaction

  • Mom Rh factor(D) negative, 1st child D+=exposure, 1st child is okay
  • **Second child D+=erythroblastosis fetalis due to IgG present in mom, able to cross placenta and bind to fetal RBC=hemolysis
127
Q

Erythroblastosis fetalis: clinical presentation

A 
**=fetal hemolytic anemia
Sx: -Hydrops fetalis 
-**Positive direct coombs
-Unconjugated hyperbiliribinemia (severe)
-Kernicterus
128
Q

Kernicterus=

A

is a very rare type of brain damage that occurs in a newborn with severe jaundice. –> **causes mental retardation and DEATH

129
Q

Hydrops fetalis=

A

SEVERE fetal edema

130
Q

Erythroblastosis fetalis: Prevention

A
  • Rh (D-) mothers are given RhD(anti D antibody) =Rhogam, during pregnancy
  • When fetal rbc cross into maternal circulation, Rhogam binds to them, masks the D ag from mom and prevents forming ab to it.
131
Q

Warm Autoimmune Anemia=

A

=IgG vs self antigen reaction at 37⁰ C acts as opsonin

  • RBC removed by phagocytosis
  • Extravascular hemolysis
132
Q

Warm Autoimmune Anemia: Sx

A

Present with anemia, jaundice,+/-splenomegaly

133
Q

Warm Autoimmune Anemia: Labs

A
  • Direct Coombs test positive,

- elevated retic count

134
Q

Warm Autoimmune Anemia: etiology (primary and secondary)

A
  • Primary-60% idiopathic

- Secondary: drugs (Penicillin, methyldopa, immune complex formation with drugs), B-cell neoplasm, lupus

135
Q

Cold Autoimmune Hemolytic Anemia: describe this condition

A

-IgM binds to RBC at 30⁰ with complement, at 37⁰ IgM releases, cleaves C3b>opsonin

136
Q

DIFFERENCE b/w warm and cold autoimmune anemia ?

A

-***Direct coombs is NEGATIVE in cold autoimmune hemolytic anemia & spherocytes are present

137
Q

Cold Autoimmune Hemolytic Anemia: Causes–> Acute-self limited?

A

Mycoplasma pneumonia, EBV

138
Q

Cold Autoimmune Hemolytic Anemia: Causes- Chronic?

A
  • Idiopathic

- B cell neoplasms

139
Q

Other causes of Hemolytic Anemia

A
  • Mechanical valves
  • ITP, TTP, DIC
  • Malaria
  • Babesia
140
Q

Babesia=

A

=an intracellular parasite that is endemic to the midwestern US (usually transmitted by a tick bite)

141
Q

Hereditary causes of destruction of RBC: Categories (list 3)

A
  • defect in cytoskeleton
  • structurally abnormal hemoglobins
  • enzyme deficiency
142
Q

Hereditary Spherocytosis: describe this condition

A

=Defect in cytoskeleton of RBC

  • Mutation of gene of one of the proteins responsible for cytoskeleton matrix
  • RBC not as flexible and becomes trapped in spleen
143
Q

Hereditary Spherocytosis: Sx

A

Jaundice and splenomegaly

144
Q

Hereditary Spherocytosis: Labs

A

-direct coombs negative, increased osmotic fragility test(cell cannot swell in hypotonic solution),increased unconjugated bilirubin

145
Q

Hereditary Spherocytosis: definitive tx?

A

Splenectomy resolves anemia

146
Q

Structurally abnormal hemoglobin: Sickle Cell anemia

Homozygous vs Heterozygous?

A

HbS= sickle cell disease (when they have all HgS)= Homozygous

Sickle cell trait (NOT sickle cell dz)= Heterozygous–> 50% HbS

147
Q

Sickle cell MC demographic

A

Africans 8% have trait(malaria protection)

-0.2% have disease(anemia)

148
Q

AA substitution in Sickle Cell anemia (KNOW**)

A

“sticky” hemoglobin due to valine substitution for glutamic acid in Beta globin chain!!!!

149
Q

What environments or conditions promote sickling in sickle cell anemia?

A

Dehydration and altitude promote sickling

150
Q

Complications of Sickle Cell:

A
  • anemia
  • Microvascular obstruction=ischemia and infarction
  • Increased erythropoiesis
  • Renal and pulmonary failure
151
Q

Complications of sickle cell disease associated with Microvascular obstruction=ischemia and infarction

A
  • Painful vaso-occlusive crisis caused by necrosis in bone marrow
  • Necrotic bone marrow can embolize to the lung= acute chest syndrome
  • Autosplenectomy (in some Pts spleen becomes non functioning)-increased risk of infection with encapsulated organisms
  • Priapism (a prolonged erection of the penis), cerebral infarcts
152
Q

G6PD deficiency-Enzyme: describe this condition

A

=X linked recessive

  • Most common enzyme deficiency to cause hemolysis
  • **RBC unable to regenerate glutathione-normally helps to reduce oxidized substances
153
Q

G6PD deficiency-Enzyme: demographic

A

Prevalent in Middle East and African descent

154
Q

G6PD deficiency-Enzyme: Sources of oxidants (that can trigger an anemic episode in Pts)

A

-Antimalarial (primiquine), sulfonamides, aspirin, nitrofurantoin, viral hepatitis and fava beans

155
Q

G6PD deficiency-Enzyme: key lab findings

A

-Heinz bodies and Bite cells

(**Think Heinz ketchup (glucose 6-d) and bite cells

156
Q

Polycythemia: describe this condtion

A
  • Increased number of RBCs

- hgb>60%

157
Q

Polycythemia: sx

A

red faces, headaches,

158
Q

Polycythemia: Relative

A

hemoconcentration

159
Q

Polycythemia: Primary

A

Polycythmia rubra vera=proliferation of RBC

160
Q

Polycythemia: secondary

A

(ie occurring 2/2) Lung dx, cyanotic heart dx, erythropoietin producing tumors

Pathophys Semester 2 (12 decks)

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