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Hematology / Oncology > Pathophysiology: Hematology > Flashcards

Pathophysiology: Hematology Flashcards

1
Q

Hemoglobinopathy

A

Disorder affecting the structure, function, or production of hemoglobin; inherited; range fr asymptomatic to deadly.

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2
Q

Hemolysis / Hemolytic

A

Destruction of RBCs –> hemoglobin is liberated

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3
Q

Coagulopathy

A

Bleeding disorder affecting clotting of the blood

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4
Q

Polycythemia

A

Elevated hemoglobin

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5
Q

Apparent Polycythemia

A

Plasma level is down so hemoglobin level seems elevated

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6
Q

Aplasia

A

Incomplete, defective, or a stop in the usual regenerative process of blood

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7
Q

Cytosis

A

More than the usual # of cells are circulating in the blood

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8
Q

Cytopenia

A

Reduction in number of cells in the blood

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9
Q

Anisocytosis

A

Variation in size and shape of RBCs

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10
Q

Poikilocytosis

A

Presence of poikilocytes in the blood

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11
Q

Poikilocytes

A

Abnormally shaped red cells

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12
Q

Anemia

A

Hematocrit is less than 41% in males and less than 36% in women… i.e. hemoglobin is less than 13.5 g/dL in men or less than 12 g/dL in women

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13
Q

Pathway to a platelet

A

pluripotential cell –> myeloid multipotential cell –> megakaryocyte colony forming cell –> Megakaryoblast –> Megakaryocyte –> Platelet

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14
Q

Thrombocytopenia

A

Low Platelets

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15
Q

Pathway to B or T Cell

A

Pluripotential Cell –> Lymphoid Multipotential Cell –> Lymphoid Colony Stimulating Factor –> Lymphoblast –> B and T lymphocytes

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16
Q

To check for low folic acid, what lab should you draw?

A

Homocysteine

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17
Q

To check for low B-12, what lab should you draw?

A

Methylmalonic Acid

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18
Q

Mean Cell Volume for Macrocytic Anemia

A

100 and Above (Hemaglobin & Hematocrit will be low)

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19
Q

MCV for Normocytic Anemia

A

MCV = 80-100 (Hemaglobin & Hematrocrit

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20
Q

MCV for Microcytic Anemia

A

Under 80

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21
Q

Anemias by Morphology

A

Macrocytic, Microcytic, Normocytic

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22
Q

Microcytic Anemias

A

Iron Deficiency, Thalassemia (major/minor)

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23
Q

Iron deficiency pathophysiology

A

Problem synthesizing hemoglobin b/c iron component is lacking

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24
Q

Iron deficiency is _____________________

A

blood loss until proven otherwise

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25
Q

Etiology of iron deficiency (5):

A

1) blood loss (GIB, menstrual, blood donation), 2) decreased absorption (post gastrectoy, celiac disease, h.pylori), 3) increased demand (pregnancy), 4) dietary deficiency, 5) iron sequestration

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26
Q

Iron deficiency signs:

A

smooth tongue, brittle nails, koilonychias, cheilosis

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27
Q

Iron deficiency symptoms:

A

tachycardia, tachypnea on exertion, pica, fatigue, palpitations

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28
Q

Iron deficiency treatment:

A

Take oral iron (with Vitamin C)

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29
Q

Thalassemia

A

Defect in the globin part of the hemoglobin

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30
Q

Thalassemia Etiology / Types

A

Genetic / Alpha and Beta

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31
Q

Thalassemia Major / Minor

A

Major = homozygous, Minor = heterozygous

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32
Q

Thalassemia Treatment

A

NO IRON; don’t over-treat, if necessary, blood transfusions

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33
Q

Alpha Thalassemia is common in:

A

SE Asia, China, ME, Africa; Asia can get Major, Africa will not

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34
Q

Beta Thalassemia is common in:

A

Mediterranean origin (lesser extent Asia/Africa)

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35
Q

Macrocytic Anemias:

A

Folic Acid Deficiency, B-12 Deficiency, Liver Disease, Myelodysplasia

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36
Q

Megaloblasts / Megaloblastic Anemias

A

Large, Immature Red Cells / Low B-12, Folic Acid

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37
Q

Pathophys of Megaloblastic Anemias

A

DNA synthesis is inhibited; cell grows, but does not go through division.

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38
Q

Folic Acid Deficiency Etiology

A

Malnutrition, alcoholism, pregnancy (increased demand)

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39
Q

B-12 Deficiency Etiology

A

Disruption in intestinal mucosa absorption: surgery, alcoholism, celiac disease

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40
Q

Pernicious Anemia

A

Body can’t absorb enough B-12

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41
Q

Signs and Symptoms of B12/Folic Acid deficiency

A

Pallor, glossitis, diarrhea, paresthesia, memory impairment

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42
Q

Side Effects of All Anemias

A

Tachycardia, tachypnea, fatigue w/ exercise, palpitations

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43
Q

Myeloid Dysplastic Syndrome

A

Stem cell disorder involving myeloid lineage of blood cells; nuclear and cytoplasmic maturation in blood cells do not correspond

44
Q

Myeloid Dysplastic Syndrome Etiology

A

Idiopathic or Toxic

45
Q

Myeloid Dysplastic Blood looks like:

A

Tapioca pudding under a microscope

46
Q

Treatment for Myeloid Dysplastic Syndrome:

A

Give EPO or blood transfusion

47
Q

Thalassemia Complication

A

Iron overload!

48
Q

Signs of Thalassemia in children

A

Facial bone changes

49
Q

Round Macrocytes

A

Liver disease or Myeloid Dysplastic

50
Q

Oval Macrocytes

A

Megaloblastic

51
Q

Normocytic Anemia:

A

Anemia of Chronic Disease

52
Q

Etiology of Anemia of Chronic Disease:

A

Chronic inflammation/conditions lead to reduced # of RBCs, i.e.: Autoimmune disease, cancer, chronic kidney disease, HB, HC, HIV / lack of EPO

53
Q

Who often gets Anemia of Chronic disease?

A

Elderly / sick

54
Q

What are the components of a CBC? (10)

A

RBC count, WBC count, Platelet Count, Hemoglobin, Hematrocrit, Reticulocyte Count, RDW, MPV, MCV, MCHC

55
Q

RDW

A

RBC Distribution Width

56
Q

Hemoglobin

A

Iron containing oxygen transport metalloprotien in RBCs

57
Q

Hematrocrit

A

Volume RBCs / Plasma = %

58
Q

MPV

A

Mean Platelet Volume

59
Q

MCHC

A

Mean Corpuscular Hemoglobin Concentration - measure of the concentration of hemoglobin in a given volume of packed RBCs

60
Q

Polythemia

A

Increased hemoglobin and hematocrit

61
Q

Leukocytosis

A

High WBCs

62
Q

Leucopenia

A

Low WBCs

63
Q

Thrombocytosis

A

High Platelet Count

64
Q

Thrombocytopenia

A

Low Platelet Count

65
Q

Hemolytic Anemia

A

Abnormal breakdown of RBCs

66
Q

Intrinsic Hemolytic Anemia / Examples (2)

A

Defects in component of RBC (i.e. membrane, enzyme, hemoglobin) / Ex: Sickle Cell, G6PD deficiency

67
Q

Extrinsic Hemolytic Anemia

A

Immune or microangiopathic hemolytic anemia / agent like Clostridium or Plasmodium attacking

68
Q

List the Hemolytic Anemias (7):

A

Sickle Cell (Hb SS disease), Hemoglobin S-C disease, Sickle Cell Trait, Hereditary Spherocytosis, G6PD Deficiency, Autoimmune Hemolytic Anemia, Cold Agglutinin Disease

69
Q

Sickle Cell Anemia SS’s genetic inheritance

A

Autosomal recessive

70
Q

Etiology of Sickle Cell SS Anemia

A

Base substitution of valine for glutamine leads to unstable production of hemoglobin that leads to mis-shapen, “sickled” RBCs

71
Q

Signs and symptoms of Sickle Cell SS

A

Onset in 1st year of life; jaundice, gallstones, splenomegaly, poor healing

72
Q

Sickle Cell SS labs

A

Chronic low hematocrit, reticulocytes, elevated WBCs, thrombocytosis, high bilirubin

73
Q

Sickle Cell SS treatment

A

EPO, folic acid, transfusions

74
Q

Hemoglobin S-C disease etiology

A

Base substitution at same place as Sickle Cell SS but instead it is valine for glutamine

75
Q

Hemoglobin S-C disease is __________ than Sickle Cell SS

A

Milder

76
Q

In hemoglobin S-C, RBCs will look _______ in a smear

A

sickled

77
Q

Consistency of sickled cells

A

sticky

78
Q

Sickle Cell Trait Genetics

A

Heterozygous

79
Q

Between Sickle Cell Trait, Sickle Cell SS, Hemoglobin S-C, which is asymptomatic?

A

Sickle Cell Trait

80
Q

How does Sickle Cell Trait appear on a blood smear?

A

Totally normal - no sickling; no RBCs on smear.

81
Q

Complications of Sickle Cell Trait:

A

Possible sudden cardiac death and rhabdomylosis during exercise, increase in thromboemolism, chronic kidney disease

82
Q

Etiology of Hereditary Spherocytosis

A

Autoimmune Disorder of RBC membrane leading to chronic hemolytic anemia; decrease in surface to volume ration result in spherical shaped cell.

83
Q

Hereditary Spherocytosis Genetics

A

Autosomal Dominant

84
Q

Hereditary Spherocytosis lab

A

Always high reticulocytes, spherocytes always present, Microcytosis

85
Q

Hereditary Spherocytosis Treatment

A

Splenectomy

86
Q

G6PD Deficiency Genetics

A

x-linked recessive

87
Q

How G6PD deficiency causes hemolytic anemia

A

B/c of lack of G6PD enzyme, exposure to oxidative drugs/food causes episodes of hemolytic anemia

88
Q

G6PD Deficiency Complications

A

Kidney/Liver failure

89
Q

Autoantibody Hemolytic Anemia etiology

A

IgG binds to the outside of RBCs and coats them, making them look like a foreigner. Body attacks RBC, leaving spirochete which gets stuck in the spleen; likes warmth.

90
Q

Autoantibody Hemolytic Anemia S&S

A

Rapid onset anemia - LIFE THREATENING

91
Q

Autoantibody Hemolytic Anemia Lab Results:

A

Reticulocytes, spirochetes

92
Q

Autoantibody Hemolytic Anemia Management:

A

Splenectomy

93
Q

Cold Agglutinin etiology

A

IgM antibodies attach to RBCs in areas of lower temp; when RBCs move to higher temp, IgM dissociates & signals Kupffer cells to sequester RBCs, causing hemolytic anemia

94
Q

Cold Agglutinin S&S

A

Exposure to cold

95
Q

Hemochromatosis Genetics

A

Autosomal Recessive; HFE gene mutation

96
Q

Hemochromatosis

A

Decrease in hepcidin which regulates iron, causing increased iron absorption / over load

97
Q

Hemochromatosis prevention

A

Patients should avoid foods rich in iron

98
Q

Conditions of Bone Marrow Failure (3):

A

Aplastic anemia, pure red cell aplasia, myelodysplasia

99
Q

Aplastic Anemia

A

Bone marrow is not producing mature blood cells from hematopoietic stem cells

100
Q

Hemochromatosis genetics

A

Autosomal Recessive; mutation of HFE gene that expresses hepcidin

101
Q

Consequences of Hemochromatosis

A

Iron builds up in kidneys, liver, lung, pancreas, heart, adrenals, pituitary

102
Q

Treatment for Hemochromatosis

A

“Donate blood” frequently except you just throw away the excess pints

103
Q

Treatment for Aplastic Anemia

A

EPO or myeloid growth factor

104
Q

Pure Red Cell Aplastic Anemia

A

Rare autoimmune disorder mediated by T lymphocytes causing RBCs to be formed incorrectly.

105
Q

Treatment for Pure Red Cell Aplastic Anemia

A

Immunosuppressive therapy

106
Q

Myeloid Dysplasia

A

Morphologic abnormalities in 2 or more hematopoietic cell lines

107
Q

Myeloid Dysplasia on labs

A

Left shift in CBC; particularly presence of dwarf megakaryocytes w/ unilobed nucleus; MCV elevated

Hematology / Oncology (9 decks)

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