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Hematology > Other hemolytic anemias > Flashcards

Other hemolytic anemias Flashcards

1
Q

Defects in RBC membrane

Cation permeability

A

Hereditary Stomacytosis
Hereditary Xerocytosis

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2
Q

Defects in RBC membrane

Cytoskeleton

A

Herditary Spherocytosis

Hereditary Elliptocytosis

Hereditary Pyropoikolocytosis

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3
Q

Hereditary spherocytosis

A
  • Most common
  • Spectrin deficiency (cytoskeleton)
  • Problem: Loss of surface area - decreased surface to volume ratio
  • Clinical manifestations
    1. splenomegaly
    2. Jaundice
    3. Anemia
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4
Q

Hereditary Spherocytosis

LAB

A
  • Decreased Haptoglobin
  • Normal or increased MCHC
  • Increased osmotic fragility
  • Increased reticuolcytes
  • Increased bilirubin (indirect and total)
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5
Q

Hereditary spherocytosis

Morphology + treatment?

A

Morphology - spherocytes

Treatment - Total or Partial splenecotomy

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6
Q

Cause of hereditary elliptocytosis

A

defective or deficienct spectrin (cytoskeleton)

  • Weakened cytoskeleton cannot go back to original form after shear stress
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7
Q

Clinical subtypes of Elliptocytois

A
  • Common Hereditary Elliptocytosis
  • Southeast Asian Ovalocytosis
  • Spherocytic Hereditary Elliptocytosis
  • Hereitary Pyropoikilocytosis
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8
Q

Hereditary elliptocytosis

severity + Morphology?

A

Severity - anemia severity varies from asymptomatic to severe between types.

Morphology - Elliptocytes, some HE clinical subtypes exhibit ovalocytes

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9
Q

Hereditary Pyropoikilocytosis

Cause?

Problem?

A

RARE

Cause - Reduced assembly of spectrin + increased spectrin degradation

Problem - Severely weakened cytoskeleton that cannot go back to original form and may fragment after shear stress.

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10
Q

Hereditary pyropoikilocytosis
symptoms

A
  • severe hemolysis
  • Poor growth
  • Bone expansion - facial abnormalities
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11
Q

morphology of pyropoikilocytosis

A

Schistocytes !

Other fragmented cells, spherocytes, elliptocytes, bizarre RBCs

RBCs heat sensitive and can fragment at body temperature (pyro)

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12
Q

Hereditary Stomacytosis (permeability)

Cause?
Problem?

A

Cause - Deficiency in stomatin - a protein that regulates ion transport across RBC membrane

Problem?
- increased permability to sodium (Na+)
- Na+ influxes into cell
- Water follows and influxes into cell

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13
Q

Hereditary Stomacytosis - Lab findings

A

High MCV
Low MCHC

Morphology: stomatocytes
Treatment: Splenectomy

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14
Q

Hereditary Xerocytosis

Cause?
Problem?

A

Cause - unknown, but due to defect in membrane permeability

Problem
- Increased permeability to Potassium (K+)
- K + OUTfluxes from cell
- Water follows outfluxing
- Results in cell dehydration with hemoglobin concentrating in one area

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15
Q

Herditary Xerocytosis Lab findings

A

High MCHC
Low Osmotic Fragility - more resistant

morphology - Target cells, crenated cells and Xerocytes

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16
Q

Hexose monophosphate pathway

A

G6PD deficiency

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17
Q

Glycolytic pathway

A

Pyruvate Kinase Deficiency

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18
Q

Methemoglobin Reductase Pathway

A

Methemoglobin reductase deficiency

19
Q

Hexose monophosphate pathway aka phosphogluconate pathway /PPP

A
  • Uses 5-10% of RBC glucose
  • Forms NADPH
  • NADPH is reduced and forms reduced Glutathionene (GSH)
  • GSH protects hemoglobin from oxidative denaturation
20
Q

G6PD Deficiency

A

X linked recessive disorder (more prevalent in males)
- Decrease in G6PD

Hgb becomes susceptible to oxidation, followed by denaturation and precipitation
- increased Heinz bodies

21
Q

In G6PD deficiency ..heinz bodies are

A

Removed in the spleen, leaving (helmet cells) aka. bite cells

22
Q

G6PD deficiency, RBC loses deformability ..

A

tough time getting through microvasculature

  • leads to premature destruction in the circulation = intravascular hemolysis
23
Q

G6PD clinical manifestiations

A

Majority is asymptomatic
- only 20% of normal G6PD activity is required for normal RBC function

Clinical manifestations occur in states of oxidation:
1. oxidative drugs
2. Infections
3. Ingestion of fava beans

24
Q

G6PD - Favism

A

Fava beans contain chemicals that destroy gluthione

Always want to pair with a nice Chianti

25
Q

G6PD - CBC, Smear, Chemistry

A
  • Decreased Hgb, HCT
    1. normocytic, normochromic
  • Hemoglobinemia
    1. Visual hemolysis
  • Increased reticulocytes
  • Increased Bite cells
  • Increased Serum indirect bilirubin
  • Decreased/absent Haptoglobin
  • Increased LDH
26
Q

G6PD - urine

A
  • hemoglobinuria
  • Urine may be darker/black (coke or port wine color)

Due to oxidized RBCs

27
Q

G6PD - Heinz body prep

A

Heinz bodies

  • wright stain
  • supravital stain
28
Q

Glycolytic pathway aka Embden-Meyerhod Pathway

A

Non oxidative

Generates 90% of ATP needed by RBCs

Forms 2 net ATP

29
Q

PK deficiency

A

Hereditary mutation (different autosomal forms not sex-linked)
- Decreased pyruvate kinase (PK)
- decreased PK leads to decreased ATP generated
- Decreased ATP leads to RBC membrane rigidity and fragility

Rigid RBCs are sequestered by the spleen = extravascular hemolysis

30
Q

PK deficiency - Clinical Manifestations

A

Hemolytic anemia can range from mild to severe
- dependent on type of mutation inherited

More pronounced with concurrent infections or other stress states

Severe cases may require lifelong repeated transfusions

Splenectomy may help to increase RBC life span

31
Q

PK deficiency Lab findings

A

*CBC + Peripheral smear *
- decreased Hgb and Hct

Accelerated Erythropoiesis
- Polychromasia
- nRBCs

*Chemistry *
- increased serum indirect bilirubin
- decreased/absent haptoglobin

Diagnosis is done using a specialized fluorescent screening test

32
Q

Importance of methemoglobin reductase pathway

A
  • Important in maintaining heme iron in reduced (Fe2+, ferrous) form
33
Q

NADH - Methhemoglobin Reductase Deficiency

A

Autosomal recessive inheritance

Not able to keep iron in reduced form

Leads to increased methemoglobin
- methemoglobinemia

34
Q

NADH - Methhemoglobin Reductase Deficiency

Main lab finding
Major clinical feature
Treatment

A

Main lab finding
- Methemoglobinemia
Major clinical feature
- Cyanosis (not able to carry oxygen)
Treatment
- Intravenous Methylene Blue
1. Artifically activates NADH - Methemoglobin reductase system

35
Q

Paroxysmal Nocturnal hemoglobinuria - PNH

Acquired Intracorpuscular defect - hemolytic anemia

A

X Linked PIGA mutation in hematopoietic stem cell

  • Deficiency in GPI anchor proteins
  • ^Decreased GPI anchor proteins = sensitive to complement-mediated hemolysis

Complement enhanced with acidity

Blood pH slightly decreased at night - increased hemolysis
- or with infection, surgery, transfusion

36
Q

PNH - CBC and differential

A

Low RBC cpunt, Hgb, Hct

Low WBC - Leukopenia
- decreased Neutrophils - Neutropenia

37
Q

PNH reticulocyte count

A
  • Increased Reticulocyte count, but not enough to compensate

Can lead to aplastic anemia = bone marrow failure - decreased reticulocyte count

38
Q

PNH peripheral smear

A
  • Normocytic, normochromic
  • occasional increase in macrocytosis and polychromasia
    1. due to increased reticulocyte
    2. reverse if aplastic anemia is developed
39
Q

PNH - BM

A

Erythroid hyperplasia
- responding to chronic hemolysis
- reverse if aplastic anemia is developed

Usually decreased iron
- Leading to development of IDA

40
Q

PNH - Urinalysis

A
  • Possible dark urine
  • Hemoglobinruia
  • Hemosiderinuria (iron)
  • All due to chronic hemolysis
41
Q

Ham’s Test (PNH)

A

RBC incubated at 37 degrees
- acidified to 6.5-7.0

Normal RBC are resitant to acidification

PNH RBCs are sensitive to acidification and lyse from activated complement

Flow cytometry is considered current gold standard

42
Q

PNH treatment

A

Bone marrow transplant is curative

Long-term tranfusions for anemia

Monoclonal Ab Treatment - Eculizumab
- Binds C5 - prevents complement mediated hemolysis

43
Q

Extravascular defects

A
  • Immune hemolytic anemias
  • infections
  • Exposure to chemicals and toxins
  • Exposure to physical agents
  • Microangiopathic and macroangiopathic hemolysis
  • General system disorders
43
Q

Extravascular defects

A
  • Immune hemolytic anemias
  • infections
  • Exposure to chemicals and toxins
  • Exposure to physical agents
  • Microangiopathic and macroangiopathic hemolysis
  • General system disorders

Hematology (36 decks)

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