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Heme and Lymph > Red Blood Cell and Bleeding Disorders > Flashcards

Red Blood Cell and Bleeding Disorders Flashcards

1
Q

What are microcytic hypochromic anemias dur to?

A

disorders of Hb synthesis

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2
Q

What are macrocytic anemias due to?

A

things that impair maturation of erythroid precursors in marrow

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3
Q

What are the principal clinical features of extravascualr hemolytic anemias?

A

anemia, splenomegaly, and jaundice

  • patients need splenectomy
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4
Q

What are the principal clinical features of intrinsic hemolytic anemias?

A

anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice.

*do not need splenectomy

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5
Q

What morphology is seen in hemolytic anemias regardless of cause and type?

A
  • increased # of erythroid precursors
  • Prominent reticulocytosis
  • hemosiderosis in spleen, liver, LN
  • Extramedullary hematopoiesis
  • bilirubin gallstones
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6
Q

What is the name of the disorder in which there are intrinsic defects in the red cell membrane?

A

Hereditary Spereocytosis

*autosomal dominant 75% of the time

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7
Q

What proteins are defective in hereditary sperocytosis?

A

ankyrin, spectrin, band 3, protein 4.2

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8
Q

What tests are positive in hereditary sphercytosis?

A

osmotic fragility +

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9
Q

What are the presenting symptoms of hereditary spherocytosis?

A

Anemia, splemomegaly, and jaundice

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10
Q

What are the labs on hereditary spherocytosis?

A

increased MCHC, increased RDW

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11
Q

What are patients with hereditary spherocytosis at risk of?

A

aplastic crisis that is triggered by parvo B19 virus

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12
Q

Treatment of hereditary spherocytosis?

A

splenectomy

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13
Q

What is the inheritance of G6PD?

A

autosomal recessive- males at risk!

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14
Q

Why is G6PD needed?

A

To reduce NADP-> NADPH which is needed to regenerate reduced glutathione.

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15
Q

What two populations are affected by G6PD most often?

A

black- modest reduciton in G6PD

mediterrean- marked reduction in G6PD activity

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16
Q

What are the common triggers for oxidant stress?

A

Infection, foods (fava beans), drugs

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17
Q

What cells will a peripheral smear show in G6PD?

A
  • RBSc with Heinz bodies and bite cells.

- Heinz cells are removed by splenic macrophages that result in bit cell formation

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18
Q

What are the genetics of sickel cell anemia?

A

Autosomal recessive

  • point mutation glu-> val in the sixth codon of beta-globin
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19
Q

Sickel cell trait is protective against what?

A

Falciparum malaria

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20
Q

Why are newborns with sickle cell disease initially asymptomatic?

A

HbF is protective and no HbS is present for first few months of life

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21
Q

What is the pathogenesis of sickle cell disease?

A

low O2, dehydration, or acidosis precipitates sickling, which results in anemia and vaso-occclusive disease

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22
Q

What does peripheral blood smear show in a patient with sickle cell disease?

A

sickled cells, reticulocytosis, and target cells, howell-jolly bodies

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23
Q

What are the sites of extramedullary hematooiesis?

A
  • skull and facial bones “crew-cut appearance and chipmunk facies”
  • -hepatomegaly
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24
Q

What are patients with SSD and infection with parvo B19 at risk for?

A

aplastic crisis

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25
Q

SSD patients with autosplenectomy are at risk for what?

A
  • bacterial infections from encapsulated organisms.
  • Strep and H. influ (most common cause of death in children)
  • Salmonella osteomyelitis
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26
Q

How does acute chest syndrome associted with SSD present?

A
  • vaso-occlusion in pulmonary microcirculation.

- chest pain, SOB, lung infiltrates

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27
Q

What is a common presenting symptom in a young individual with SSD?

A

dactylitis-> painful hand swelling due ot vaso-occlusion

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28
Q

How is the diagnosis of SSD confirmed?

A

Metabisukfite reagent that induces sickling

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29
Q

What is the name of the disease that results in a decrease in synthesis of globin?

A

Thalassemia

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30
Q

What type of anemia do the thalassemias cause?

A

microcytic, hypochromic

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31
Q

What are the mutations that cause beta thalassemia?

A

point mutations in splice sites and promoter sequences.

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32
Q

What are the two types of beta thalassemia and which is more common?

A

Major and minor

Minor more common

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33
Q

What will a peripheral smaer in a patient with beta thalassemia minor show?

A

hypochromia, microcytosis, basophilic stippling and target cells.

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34
Q

How does beta thalassemia major present?

A

within 6-9 months of birth as Hb switches from HbF to HbA

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35
Q

What will a peripheral smear show in a patient with beta thalassemia major?

A
  • severe red cell abnormalities, microcytosis and hypochromia
  • Target cells, basophilic stippling, and fragmented red cells
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36
Q

Where can the extramedullary hematopoiesis be seen in patients with beta thalassemia major?

A
  • spleen/liver-> HSM

- skull-> “crewcut” appearance on xray

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37
Q

What do almost all patients with beta thalassemia major develop?

A

secondary hemochromotosis

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38
Q

What is the genetic cause of alpha thalassemias?

A

inherited deletions.

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39
Q

Where is beta thalassemia most common?

A

Mediterranean countries, parts of Africa, and SW Asia

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40
Q

What is the name of the unpaired gamma chains that form tetramers in newborns with alpha thalassemia?

A

Hemoglobin barts

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41
Q

How is the clinical syndrome of alpha thalassemia classified?

A

By the number of alpha globin genes that are deleted

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42
Q

What do one and two alpha globin gene deletions produce?

A
  • one: asymptomatic silent carrier

- two: alpha thalassemia trait , mild anemia, cis deletion increases risk of severe thalassemia in offspring

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43
Q

What does three alpha globin gene deletions produce?

A
  • Hemoglobin H disease, tetramers of HbH form, tissue hypoxia develops, intracellular inclusions form that promote red cell sequestration.
  • moderately severe anemia
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44
Q

What does four alpha globin gene deletions produce?

A
  • Hydrops fetalis->lethal in utero.

- fatal unless intrauterine transfusions are performed.

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45
Q

What is the mutation and what protein is affected in Paroxysmal Nocturnal Hemoglobinuria?

A
  • mutations in the PIGA gene that encode for the GPI anchor

- three proteins that make up the anchor are deficient;DAF (CD55), C8 binding protein, CD59.

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46
Q

What is causing the RBC damage and lysis is Paroxysmal Nocturnal Hemoglobinuria?

A

compliment

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47
Q

Why do RBCs have a tendency to lyse at night is Paroxysmal Nocturnal Hemoglobinuria?

A

shallow and slower respirations cause an increase in CO2 and decrease in pH causing the cells to become damaged and lysed by compliment

48
Q

What do patients develop with paroxysmal nocturnal hemoglobinuria?

A

mild to moderate anemia with hemosiderinuria that will eventually lead to iron deficiency.

49
Q

What are patients at risk for with paroxysmal nocturnal hemoglobinuria?

A

thrombosis–> major cause of death

50
Q

What does the indirect Coombs test for?

A
  • tests for presence of antibodies in the patients serum.

- agglutination will occur if serum antibodies are present

51
Q

What does the direct Coombs test for?

A
  • test for presence of antibody or complement coated RBCs.

- agglutination will occur if antibodies or complement have coated RBCs already

52
Q

What is the warm antibody type of immunohemolytic anemia?

A
  • IgG mediated, coats RBCs
  • Partially removed in the spleen-> spherocytes
  • moderate splenomegaly
  • penicillin and methyldopa
53
Q

What is the cold antibody type of immunohemolytic anemia?

A
  • IgM antibodies bind to RBCs at lower temperatures.
  • following mycoplasma pneumonia, EBV, CMV, influenza, HIV
  • fingers, toes, ear, nose
54
Q

What can cause microangiopathic hemolytic anemia?

A

-prosthetic heart valves, HUS, TTP, malignant HTN, and SLE

55
Q

What is the common theme among megaloblastic anemias?

A

impairment of DNA synthesis that leads to ineffective hematopoiesis.

56
Q

What is the morphology in megaloblastic anemia?

A
  • RBCs will be macrocytic and oval shaped “macro-ovalocyte”

- lack central pallor

57
Q

What is anemia due to a B12 deficiency called?

A

pernicious anemia

58
Q

What are some underlying causes of B12 megaloblastic anemia?

A

autoimmune gastritis, achlorhydria, gastrectomy, EPD, ileal reseciton, tape worms, B12 deficiency

59
Q

What are the lab findings in B12 megaloblastic anemia?

A
  • hypersegmented granulocytes
  • decreased B12
  • increased homocysteine
  • increased methylmalonic acid
60
Q

What is the most common cause of B12 megalobalstic anemia?

A

Pernicious anemia caused by autoimmune gastritis.

61
Q

What is a complication of B12 deficiency if not treated?

A
  • Subacute combined degeneration of the spinal cord.
  • peripheral neuropathy
  • dorsal column (proprio/ vibra)
  • LCST (spasticity)
62
Q

What are the three main causes of anemia of folate deficiency?

A

decreased intake, increased requirement, impaired utilization

63
Q

What can interfere with folate absorption?

A

oral contraceptives and phenytoin

64
Q

What are the labs of anemia of folate deficiency?

A

-decreased folate, increased homocysteine, normal methymalonic acid

65
Q

What type of anemia is Iron deficiency anemia?

A

Microcytic, hypochromic

66
Q

Who is most commonly affected by Iron deficiency anemia?

A

toddles, adolescent girls, and women of childbearing age

67
Q

What are the common etiologies of Iron deficiency anemia?

A

Chronic bleeding (GI, menorrhagia), malnutrition/ absorption, increased demand (pregnancy)

68
Q

What are the lab findings in iron deficiency anemia?

A

decreased ferritin, increased TIBC, decreased serum Fe, decreased % saturation.

69
Q

What does a peripheral smear in a patient with Iron deficiency anemia show?

A

Microcytic, hypo chromic RBCs, with central pallor.

70
Q

What can long term Iron deficiency cause?

A

Koilonychias, alopecia, glossitis, intestimal malabsorption

71
Q

What syndrome can occur with iron deficiency anemia and what is it characterized by?

A

Plummer-Vinson Syndrome: anemia, dysphagia, and esophageal webs.

72
Q

What are some initial presenting symptoms in Iron deficiency anemia?

A

Fatigue, conjunctival pallor

73
Q

What is anemia of chronic disease?

A

anemia associated with chronic inflammation or cancer.

74
Q

What is released in anemia of chronic disease that causes the sequestration of Fe?

A

Acute phase reactants are released from the liver, including hepcidin, which results in the sequestration of Fe in cells.

75
Q

What specific cytokine is associated with stimulation of hepcidin?

A

IL-6

76
Q

What are the lab values in anemia of chronic disease?

A

increased ferritin, decreased TIBC, decreased serum Fe, decreased % saturation.

77
Q

What is the underlying cause of aplastic anemia?

A

Damage to hematopoietic stem cells, resulting in pancytopenia.

78
Q

What are the common etiologies of aplastic anemia?

A

drugs, chemicals, viral infections, and autoimmune damage.

79
Q

What does the bone marrow show in aplastic anemia?

A

hypocellular marrow with fatty infiltration

80
Q

What are the most common viral agents associated with aplastic anemia?

A

Parvo B19, HIV, HCV, EBV

81
Q

What are some of the clinical findings in a patient with aplastic anemia?

A
  • progressive weakness, pallor, dyspnea.
  • ecchymoses and petechiae
  • bacterial infections
82
Q

What is red cell aplasia?

A

primary marrow disorder in which only erythroid precursor cells are suppressed.

83
Q

What is myelophthisic anemia?

A

form of marrow failure in which space-occupying lesson replace normal marrow elements

84
Q

What is the cause of a relative polycythemia?

A

hemoconcentration due to decreased plasma volume (dehydration, burns)

85
Q

What is the underlying cause of absolute polycythemia?

A

increase in total red cell mass, high EPO levels, decreased O2 saturation–> lung disease, congenital heart disease, high altitude

86
Q

What does PT assess and what factors are in this pathway?

A

Common and Extrinsic pathway, factors I,II,V,VII,and X

87
Q

What does PTT assess and what factors are in this pathway?

A

Common and Intrinsic pathway, factors XII,XI, X, IX, VIII, V, II, I

88
Q

How is thrombocytopenia defined?

A

platelet count less than 100,000

89
Q

What are the 4 major ways in which thrombocytopenia can develop?

A

decreased platelet produciotn, decreased platelet survival, sequestration, and dilution

90
Q

What do the autoantibodies attack in immune thrombocytopenic purpura?

A

IgG antibodies attack platelet antigens, specifically GPIIb-IIIa

91
Q

What does the marrow show in immune thrombocytopenia purpura?

A

increased number of megakarycytes

92
Q

what are the lab findings in immune thrombocytopenic purpura?

A

decreased platelet count, increased megakaryocytic, normal PT/PTT

93
Q

How is Immune thrombocytopenic purpura diagnosed?

A

diagnosis of exclusion.

94
Q

What is the major cause of acute immune thrombocytopenic purpura?

A
  • occurs in children after a viral illness-> 1-2weeks later.

- self limited and resolves

95
Q

What is the pathogenesis of HIT?

A
  • development of IgG antibodies against heparin bound platelet factor 4 (PF4) leading.
  • Heparin-PF4 complex activates platelets leading to thrombosis and thrombocytopenia
96
Q

What are the symptoms of TTP?

A

fever, transient neurologia deficits, microangiopathic anemia, thrombocytopenia, renal failure

97
Q

What are the symptoms of HUS?

A

microangiopathic hemolytic anemia and thrombocytopenia,

98
Q

What is the distinguishing factor between TTP and HUS?

A

HUS does not have neurological symptoms and frequently occurs in children

99
Q

What is the genetic mutation associated with TTP?

A

ADAMTS13- normally degrades vWF

100
Q

What is cause of “typical” HUS?

A

infection with E.coli O157:H7

101
Q

What is Bernard-Soupier Syndrome?

A
  • defective adhesion of platelets to subendothelial matrix.

- Inherited deficiency in GpIb-IX

102
Q

What is Glanzmann Thrombasthenia?

A

-defect in platelet aggregation due to deficiency in GpIIb-IIIa

103
Q

What are the presenting symptoms of Von Willebrand Disease?

A

Spontaneous bleeding from mucous membranes, excessive bleeding from wounds, menorrhagia

104
Q

What are the genetics of vWD?

A

autosomal dominant

105
Q

What is the role of vWF?

A
  • vFW acts to carry/ protect factor VIII

- vFW deficit does not allow normal platelet adhesion

106
Q

What are the labs in vWD?

A

normal platelet count
increased bleeding time
can have increase in PTT

107
Q

What is Hemophilia A?

A

Factor VIII deficiency

108
Q

What are the genetics of Hemophilia A?

A

X-linked Recessive

109
Q

What are the labs in Hemophilia A?

A

Prolonged PTT

Normal PT

110
Q

What is Hemophilia B?

A

Christmas Disease, Factor IX deficiency

111
Q

What are the genetics of Hemophilia B?

A

X-linked Recessive

112
Q

What are the labs of Hemophilia B?

A

Prolonged PTT

Normal PT

113
Q

What is DIC?

A

Excessive activation of coagulation and the formation of thrombi in the microvasculature

114
Q

What are the labs in DIC?

A

Decreased platelet count
increased bleeding time
increased PT/PTT

115
Q

What is released in sepsis that is a mediator of DIC?

A

TNF

116
Q

What are some of the common causes of DIC?

A

obstetric complications*
endothelial injury
malignant neoplasms
major trauma

117
Q

What are some common presenting symptoms of DIC?

A

-Microangiopathic hemolytic anemia, dyspnea, cyanosis, respiratory failure, convulsions and coma, sudden or progressive circulatory failure

Heme and Lymph (4 decks)

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