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PAS 512 Clinical Medicine 1 > Anemias > Flashcards

Anemias Flashcards

1
Q

anemia

A

decreased absolute number or decreased quality of circulating RBCs which reduces the oxygen carrying capacity of blood

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2
Q

anemia is most commonly measured by

A

decreased Hgb (<13 g/dl in men; <12 g/dL in women)

decreased Hct (<41% in men; <36% in women)

decreased RBC

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3
Q

anemia classification by severity

A

mild: Hgb ~10-13.5 g/dL
moderate: Hgb ~8-10 g/dL
severe: Hgb <7-8 g/dL

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4
Q

anemia classification by pathophysiologic mechanisms

A

decreased erythropoiesis/hypoproliferative

ineffective erythropoiesis

increased RBC destruction

blood loss

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5
Q

decreased erythropoiesis/hypoproliferative pathophysiologic mechanism

A

decreased stimulation (anemia of chronic inflammation, renal dz)

mild iron deficiency

marrow damage (myelofibrosis, aplastic anemia)

bone marrow suppression (drugs)

hypometabolic states (protein malnutrition, endocrine deficiencies)

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6
Q

ineffective erythropoiesis pathophysiologic mechanism

A

sever iron deficiency

megaloblastic anemia

thalassemia

myelodysplastic syndrome

sideroblastic anemias

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7
Q

increased RBC destruction pathophysiologic mechanism

A

congenital causes (sickle cell, thalassemias, GP6D deficiency)

acquired causes (autoimmune hemolytic anemia)

hypersplenism

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8
Q

anemia classification by RBC morphology

A

size (macrocytic, microcytic, normocytic)

degree of hemoglobination (normochromic and hypochromic)

shape

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9
Q

anemia classification by timing

A

acute (blood loss, hemolysis)

chronic

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10
Q

anemia general clinical presentation

A 
variable dependent on severity and onset
dyspnea (exertional --> rest)
fatigue, weakness, malaise
pallor
hypoxia
signs and symptoms of hyperdynamic state
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11
Q

signs and symptoms of hyperdynamic state

A 
palpitations
roaring pulsatile sound in ear
increased HR
bounding pulses
systolic flow murmur
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12
Q

anemia is never normal and etiology should be sought

A

evaluate for increased RBC destruction

evaluate for marrow suppression

evaluate for nutritional deficiencies (iron, folate, B12)

evaluate for bleeding

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13
Q

anemia diagnostic evaluation

A 
Hgb/Hct, RBC count, RBC indices
platelets
WBC/WBC differential
reticulocyte count
peripheral smear
evaluate for iron deficiency
evaluate for hemolysis
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14
Q

evaluate for iron deficiency

A

iron
TIBC/transferrin
transferrin saturation
ferritin

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15
Q

evaluate for hemolysis

A 
LDH
indirect bilirubin
haptoglobin
plasma/urinary Hgb
urinary hemosiderin
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16
Q

anemia treatment

A

treat underlying etiology

treat anemia

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17
Q

anemia prognosis

A

risk factor for increased mortality in association with CKD, malignancy, heart failure, older adults, hospitalized adults

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18
Q

normocytic anemias

A 
acute blood loss anemia
anemia of chronic disease
aplastic anemia
hemolytic anemias
early iron deficiency anemia
endocrine dysfunction: hypothyroidism, hypopituitarism
chronic kidney disease
leucoerythroblastic blood picture
pure RBC aplasia
protein starvation
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19
Q

acute blood loss anemia etiologies

A

trauma

GI tract, lung, kidney, uterine disorders

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20
Q

acute blood loss anemia clinical presentation

A

hypovolemia:

  • vascular instability (increased HR, decreased BP, decreased organ perfusion)
  • hypovolemic shock (confusion, dyspnea, diaphoresis, increased HR, decreased BP)
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21
Q

acute blood loss anemia diagnostic evaluation

A

Hgb/Hct normal initially

after correction of hypovolemia, Hgb/Hct will decrease

normal RBC indices

inadequate reticulocyte response

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22
Q

acute blood loss anemia treatment

A

supportive care (transfusion)

treat underlying condition

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23
Q

anemia of chronic disease

A

seen in association with a variety of conditions (i.e. infection, inflammatory, neoplastic disease, tissue injury, etc.)

hypoproliferative-primarily due to decreased erythropoiesis

typically normochromic, normocytic, mild severity

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24
Q

anemia of chronic disease clinical presentation

A

history of chronic condition

usually minimal symptoms due to anemia

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25
Q

anemia of chronic disease laboratory evaluation

A

usually mild anemia (Hgb 10-11 g/dL)

usually MCV, MCH normal

decreased reticulocyte response

acute phase reactants (ESR, CRP) may be increased

iron studies

peripheral smear- normochromic, normocytic (not routinely needed)

bone marrow studies (not routinely needed)

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26
Q

anemia of chronic disease iron studies

A

serum Fe decreased

TIBC (transferrin) normal or decreased

TIBC saturation normal or decreased

serum ferritin normal or increased

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27
Q

if diagnosis of anemia of chronic disease is uncertain, consider additional tests

A

serum EPO –> would be low

reticulocyte response–> would be inappropriately low

soluble transferrin receptor

soluble transferrin receptor/ferritin ratio

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28
Q

anemia of chronic disease treatment

A

correction of underlying disorder

usually no specific treatment needed for anemia- transfusion or EPO-stimulating agent for severe/symptomatic anemia

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29
Q

anemia of critical illness

A

acute-event related anemia

develops following major event (surgery, trauma, MI, sepsis)

characterized by decreased RBC survival

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30
Q

anemia of critical illness labs

A

decreased Fe

increased ferritin

blunted EPO response

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31
Q

aplastic anemia

A

diminished or absent hematopoietic precursors in bone marrow

usually due to injury to pluripotent stem cell

causes pancytopenia

can be congenital or acquired

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32
Q

congenital palastic anemia

A

Fanconi anemia

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33
Q

acquired causes of aplastic anemia

A

idiopathic

drugs/chemicals

ionizing radiation

viral infection

others

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34
Q

aplastic anemia clinical presentation

A 
can have abrupt or insidious onset
may have history of inciting event
symptoms due to thrombocytopenia
symptoms due to anemia
symptoms due to profound neutropenia
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35
Q

symptoms due to thrombocytopenia

A

mucosal hemorrhage

petechiae

increased menstrual flow

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36
Q

symptoms due to anemia

A 
fatigue
pallor
lassitude
weakness
shortness of breath
pounding sensation in ear
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37
Q

symptoms due to profound neutropenia

A

recurrent infections

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38
Q

aplastic anemia diagnostic evaluation

A 
CBC: pancytopenia
RBC indices: usually normocytic
decreased reticulocyte response
peripheral smear: decreased reticulocytes; abnormal cells NOT present
bone marrow biopsy (usually required)
tests to evaluate underlying etiology
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39
Q

aplastic anemia treatment

A

hematology referral

identify and treat underlying etiology if possible

supportive care for cytopenias (minimize bleeding risk, transfusions, aggressive infection prevention, antibiotics)

patients with moderate disease may be followed supportively without initiating curative treatment

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40
Q

aplastic anemia treatment for severe disease

A

immunosuppressive therapy: 1st line therapy for patients without available donor, older patients, patient preference

hematopoietic cell transplantation: 1st line therapy for majority of patients <50 yrs with available donor

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41
Q

aplastic anemia prognosis

A

untreated associated with >70% 1 year mortality

improved survival with availability of hematopoietic cell transplant

increased risk of developing clonal hematologic disorder

increased transfusions leads to risk of iron overload

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42
Q

macrocytic anemias

A 
vitamin B12 deficiency
folate deficiency
hemolytic anemias
ethanol abuse
myelodysplastic syndromes
acute myeloid leukemias
drug induced anemia
liver disease
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43
Q

megaloblastic anemias

A

impaired DNA synthesis leading to large erythroid precursors and red cells

major causes: vitamin B12 deficiency (including pernicious anemia) and folate deficiency

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44
Q

vitamin B12 physiologic actions

A

required for growth/division of most cells

activates folic acid to allow it to participate in DNA synthesis/cell maturation

only source for humans is animal products

absorption can be passive or active (requires intrinsic factor)

body stores typically last 3-4 yrs

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45
Q

vitamin B12 deficiency

A

deficiency characterized by hematologic, neurologic, mucosal changes

can coexist with folate deficiency

increased frequency in older age

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46
Q

vitamin B12 deficiency etiologies

A

inadequate absorption

inadequate dietary intake

medications (PPI, H2 receptor antagonists, metformin)

HIV infection

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47
Q

vitamin B12 deficiency caused by inadequate absorption

A

pernicious anemia

gastrointestinal disease

gastric surgery

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48
Q

pernicious anemia

A

vitamin B12 deficiency due to lack of intrinsic factor

auto-antibodies destroy gastric parietal cells which produce intrinsic factor leading to atrophic gastritis +/- neutralize intrinsic factor

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49
Q

risk factors for pernicious anemia

A

Caucasians of northern European ancestry

increased age

autoimmune disease

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50
Q

vitamin B12 deficiency clinical presentation

A

slow in onset (years)

anemia: may have sings/symptoms related to pancytopenia if severe

glossitis, vaginal atrophy, malabsorption may be present

neurologic changes: neuropathy, balance disturbance, neuropsychiatric symptoms

increased risk of osteoporosis

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51
Q

vitamin B12 deficiency diagnostic evaluation

A

decreased Hgb/Hct - not all patients develop anemia, if anemia severe may develop pancytopenia

RBC indices: increased MCV, normal MCH

reticulocyte count normal to decreased

decreased serum vitamin B12

metabolite testing

peripheral smear

bone marrow biopsy usually not required

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52
Q

vitamin B12 deficiency metabolite testing

A

increased serum/urinary methylmalonic acid

increased serum homocysteine levels (also increased in folic acid deficiency)

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53
Q

vitamin B12 deficiency peripheral smear

A

macroovalocytes

hypersegmented neutrophils

anisocytosis

poikilocytosis

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54
Q

vitamin B12 deficiency diagnostic evaluation other tests (not commonly needed)

A

increased iron

increased indirect bilirubin

increased LDH

decreased haptoglobin

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55
Q

vitamin B12 deficiency diagnostic evaluation for pernicious anemia

A

antibodies to intrinsic factor

increased serum gastrin levels

decreased serum pepsinogen I levels

decreased ratio pepsinogen I :: pepsinogen II

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56
Q

vitamin B12 deficiency treatment

A

referral to hematologist not usually required

transfusion rarely required

rule out concurrent folate deficiency

vitamin B12

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57
Q

vitamin B12 dosing for deficiency

A

pernicious anemia: typically treated with parenteral (IM/subcu) vitamin B12

oral supplementation: generally recommended to use after vitamin B12 status has been normalized with parenteral treatment

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58
Q

vitamin B12 deficiency prognosis

A

Hgb increases within 10 days and returns to normal within 8 weeks

neurologic changes improve over 3 months (max improvement ~6-12 months)

patients with pernicious anemia appear to have increased risk of GI tract malignancies

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59
Q

vitamin B12 deficiency prevention

A

at risk populations may benefit from B12 supplementation- vegetarians, status post gastric surgery

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60
Q

folate physiologic actions

A

required for many tissue reactions

activated folic acid involved in DNA synthesis

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61
Q

folate deficiency

A

deficiency characterized by hematologic changes and pregnancy complications/neural tube defects

can coexist with vitamin B12 deficiency

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62
Q

folate deficiency risk factors

A

increased age

chronic alcohol use

certain medications

pregnancy/lactation

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63
Q

folate deficiency etiologies

A

most common cause is nutritional and/or increased requirements

nutritional due to inadequate intake (developing countries) and/or alcoholism

conditions associated with increased requirements: pregnancy, lactation, chronic hemolytic anemia

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64
Q

folate deficiency clinical presentation

A

quicker onset (4-5 months)

anemia: may have signs/symptoms related to pancytopenia if severe deficiency

pregnancy complications/neural tube defects

neurologic manifestations are rare

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65
Q

folate deficiency diagnostic evaluation

A

decreased Hgb/Hct
RBC indices: increased MCV, normal MCH
pancytopenia may develop if deficiency is severe
reticulocyte count normal to decreased
decreased serum folate levels/RBC folate
metabolite testing: increased homocysteine levels
peripheral smear
bone marrow aspiration/biopsy not usually required

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66
Q

folate deficiency peripheral smear

A

macroovalocytes
hypersegmented neutrophils
anisocytosis
poikilocytosis

67
Q

folate deficiency treatment

A

referral to hematologist not usually necessary

folic acid (1-5 mg/day orally) x 1-4 months

rule out concurrent B12 deficiency

transfusion rarely required

68
Q

folate deficiency prevention

A

supplemental folic acid recommended in pregnancy, low-weight premature neonates

69
Q

microcytic anemias

A

iron deficiency anemia
sideroblastic anemia
thalassemia

70
Q

degree of microcytosis

A

mild: MCV >70 fL
severe: MCV <70 fL

71
Q

mild microcytosis conditions

A 
iron deficiency
thalassemias
sideroblastic anemia
lead toxicity
anemia of chronic disease (usually normocytic)
72
Q

severe microcytosis conditions

A 
thalassemias
iron deficiency (severe)
73
Q

iron

A

major physiologic role to carry O2 in Hgb
present in meats, eggs, iron-fortified cereal, beans
absorbed in stomach, duodenum, upper jejunum
circulates in plasma bound to transferrin
stored as ferritin (and hemosiderin)
effective recycling mechanisms to conserve iron
no mechanism for iron excretion
nutritional requirements vary with development and physiologic processes

74
Q

iron deficiency characterized by

A

hematologic changes

alterations within fast-growing tissues

75
Q

iron deficiency anemia etiologies

A 
blood loss (overt or occult)-menstrual losses, GI losses
dietary deficiency
reduced GI absorption
increased requirements (growth, pregnancy, lactation)
76
Q

iron deficiency anemia clinical presentation

A

may have underlying condition that increases likelihood of deficiency

variable onset

signs/symptoms of anemia (weakness, headache, irritability, fatigue/exercise intolerance, pallor, increased HR)

severe deficiency can lead to smooth tongue, brittle nails, spooning of nails, cheilosis, hair loss, pagophagia, restless leg syndrome

neurodevelopment delay in children

77
Q

iron deficiency anemia diagnostic evaluation

A

decreased Hgb/Hct
RBC indices: decreased/normal MCH and MCV
+/- increased platelets
decreased reticulocyte response
peripheral blood smear
serum/plasma ferritin, iron, TIBC (transferrin), TIBC saturation
bone marrow biopsy rarely required
search for source of blood/iron loss (medical history, GI evaluation)

78
Q

iron deficiency anemia peripheral smear

A 
anisocytosis
microcytosis
poikilocytosis
hypochromia
target cells with severe deficiency
79
Q

iron deficiency anemia iron studies

A

serum iron decreased
TIBC (transferrin) normal to high
Transferrin saturation decreased
plasma ferritin decreased

80
Q

iron deficiency anemia treatment

A

identify and treat etiology

iron (oral/IV) continue for 3-6 months after normalization of Hgb and correction of underlying etiology

severe anemia may require RBC transfusion

81
Q

iron deficiency anemia prognosis

A

pagophagia often disappears as soon as iron therapy begun
improved sense of wellbeing within 1st days of treatment
modest reticulocytosis (peaks at 7-10 days)
Hgb rises slowly and will return to normal ~6-8 weeks

82
Q

iron deficiency anemia prevention

A

iron supplementation for exclusively breast-fed infants, iron fortified formula for formula fed infants

universal lab screening for infants 9-12 months of age

boys should be screened at least once during peak growth period

girls aged 12-18 yrs and non-pregnant women of childbearing age should have screening every 5-10 yrs

pregnant women should receive low dose iron and routine screening for presence of iron deficiency anemia

83
Q

sideroblastic anemia

A

congenital or acquired defects leading to impaired incorporation of iron into heme molecule

primarily laboratory diagnosis based on identification of siderocytes in peripheral blood with Wright-Giemsa stain or sideroblasts on bone marrow examination with Prussian blue stain

84
Q

siderocyte

A

abnormal RBC in peripheral circulation with Pappenheimer bodies

85
Q

sideroblast

A

erythroblast with stainable deposits of iron in cytoplasm

86
Q

ring sideroblast

A

abundant iron deposits in erythroblast that form ring around nucleus

87
Q

sideroblastic anemia diagnostic evaluation

A

moderate anemia (Hct 20-30%

decreased MCV most common

associated decreased in WBC, increased/decreased platelets may be present if associated with myelodysplastic syndrome

peripheral smear shows siderocytes with pappenheimer bodies

diagnosis confirmed with demonstration of sideroblasts on bone marrow examination with prussian blue stain

88
Q

siderocytes with pappenheimer bodies

A

hypochromic RBCs with basophilic iron deposits

89
Q

sideroblastic anemia treatment

A 
remove underlying etiology if able
supportive care (including transfusion if needed)
90
Q

sideroblastic anemia prognosis

A

variable based on underlying etiology

major causes of death are secondary hemochromatosis due to transfusions and leukemia

91
Q

anemia of old age

A

Hgb values in healthy older adults generally lower than younger adults

differences in Hgb between males and females decreasing with age

currently not uniformly accepted definition of anemia in older adult

92
Q

pediatric anemias

A 
anemia of prematurity
pathologic anemia (birth-3 months)
physiologic anemia (6-9 weeks)
pathologic anemia (3-6 months)
pathologic anemia (toddlers-adolescence)
93
Q

pathologic anemia: birth - 3 months

A

blood loss
immune hemolytic disease
congenital infection
congenital hemolytic anemia

94
Q

physiologic anemia: 6-9 weeks

A

most common cause of anemia in young infants

95
Q

pathologic anemia: 3-6 months

A

hemoglobinopathy

96
Q

pathologic anemia: toddlers - adolescence

A

acquired causes more likely (i.e iron deficiency)

anemia due to lead poisoning

97
Q

anemia dur to lead poisoning

A

can occur due to decreased Hgb synthesis and hemolysis

usually mild and normocytic

98
Q

hemolytic anemia

A

anemia due to shortened RBC survival

characterized by increased EPO levels, increased reticulocyte response, accumulation of Hgb degradation products

99
Q

various classifications of hemolytic anemia

A

acquired vs, congenital
acute or chronic
mild or severe
compensated or uncompensated
pathophysiologic
site of hemolysis (intravascular vs extravascular)
intrinsic vs. extrinsic process (intracorpuscular vs. extracorpuscular defects)

100
Q

intravascular hemolysis

A

usually caused by mechanical injury, complement fixation, intracellular parasites, exogenous factors, toxic factors

101
Q

examples of intravascular hemolysis

A

microangiopathic hemolytic anemia (thrombotic microangiopathy, leaky prosthetic heart valves)

paroxysmal nocturnal hemoglobinuria

transfusion reaction (ABO incompatibility

infection (clostridial sepsis)

102
Q

extravascular hemolysis (more common)

A

RBC destruction within phagocytes in spleen, liver, bone marrow

103
Q

examples of extravascular hemolysis

A

hemoglobinopathy
enzyme deficiencies
membrane defect
autoimmune hemolytic anemia

104
Q

intracorpuscular (intrinsic RBC defect) process

A

hemoglobinopathy (sickle cell disease, thalssemia, unstable Hgb)

enzyme deficiencies (G6PD deficiency)

membrane defect (hereditary spherocytosis, elliptocytosis)

paroxysmal nocturnal hemoglobinuria

105
Q

extracorpuscular (process extrinsic to RBC) process

A 
autoimmune hemolytic anemias (warm or cold-reacting, drugs)
microangiopathic hemolytic anemia
liver disease
hypersplenism
infections
oxidant agents
IV immune globulin infusion
106
Q

hemolytic anemia clinical presentation

A

symptoms of anemia

symptoms of hemolysis (extravascular or intravascular) - gallstones

severe forms may be associated with skeletal changes due to overactive bone marrow

107
Q

clinical presentation of extravascular hemolysis

A

associated with jaundice and hepatosplenomegaly

108
Q

clinical presentation of intravascular hemolysis

A

associated with jaundice and dark urine

109
Q

hemolytic anemia diagnostic evaluation

A

decreased Hgb/Hct

peripheral smear: macrocytes, polychromasia, nucleated RBCs may be seen

increased reticulocyte response: may be reduced (especially in intravascular hemolysis); leads to increased MCV

increased LDH

decreased haptoglobin (more significant decreased w/ intravascular hemolysis)

increased unconjugated bilirubin, increased urobilinogen

hemoglobinemia, hemoglobinuria, hemosiderinuria (intravascular hemolysis)

variable iron studies

further eval based on most likely etiology

110
Q

hemolytic anemia treatment

A

based on underlying etiology

splenectomy often helpful for patients with extravascular hemolysis

111
Q

congenital hemolytic anemias

A 
G6PD deficiency
hereditary spherocytosis/elliptocytosis
sickle cell disease
thalassemias
thrombotic microangiopathy (TTP, complement-mediated thrombotic microangiopathy)
112
Q

G6PD deficiency

A

genetic mutation leads to reduced half-life of G6PD

x-linked recessive disorder –> males at higher risk for symptomatic disease

associated with extravascular (an intravascular-rare) hemolysis

113
Q

G6PD enzyme

A

important in maintaining RBC integrity

protects against oxidative stress

114
Q

G6PD deficiency can lead to a wide variety of hemolytic syndromes based on severity of the enzyme deficiency

A

there are class I, class II, class III, and class IV variants

115
Q

G6PD deficiency class I variants

A

most severe

chronic hemolysis

congenital non-spheocytic hemolytic anemia

116
Q

G6PD deficiency class II variants

A

severe

intermittent episodes of hemolysis

G6PD Mediterranean (prevalent in Middle East)

117
Q

G6PD deficiency Class III variants

A

mild-moderate severity

intermittent episodes of hemolysis

G6PD A- (present in ~10% of American blacks)

118
Q

G6PD class IV variants

A

not associated with hemolysis

G6PD B

119
Q

G6PD deficiency triggers for hemolysis

A

infections (bacterial, viral, fungal)

medications (anti-infectives, analgesics)

foods (fresh fava beans, bitter melon)

DKA

120
Q

G6PD deficiency clinical presentation- neonatal hyperbilirubinemia

A

may develop with all variants (more likely to occur with more severe variants)

yellowing of skin, sclera, mucous membranes

encephalopathy may occur in severe cases

121
Q

G6PD deficiency clinical presentation class I variants

A

anemia and jaundice usually observed in newborn period

after infancy: chronic hemolytic manifestations subtle and variable- pallor uncommon, scleral icterus intermittent, splenomegaly rare

periods of increased hemolysis in association with triggers (drugs, infections, metabolic abnormalities)

122
Q

G6PD deficiency clinical presentation class II, III variants

A

asymptomatic in steady state

periods of hemolysis in association with triggers (drugs, infections, metabolic abnormalities)

123
Q

G6PD deficiency diagnostic evaluation class I variants

A

mild to moderate anemia at baseline (Hgb 8-10 g/dL)

increased MCV

increased reticulocyte count as baseline (10-15%)

124
Q

G6PD deficiency diagnostic evaluation

A

acute hemolysis characterized by:
decreased Hgb/Hct
increased reticulocyte response
evidence of hemolysis (increased bilirubin, decreased haptoglobin, increased LDH)
if intravascular hemolysis occurs hemoglobinemia/hemoglobinuria may be present
peripheral blood smear

125
Q

G6PD deficiency diagnostic evaluation peripheral blood smear

A 
red cell fragments (schistocytes)
microspherocytes
eccentrocytes/bite cells
anisocytosis
poikilocytosis
polychromasia
Heinz bodies
126
Q

G6PD deficiency treatment

A 
neonatal hyperbilirubinemia treated similarly to other cases or neonatal jaundice
avoid exposure known to trigger hemolysis
supportive care for acute hemolytic events
class I variants: folic acid supplementation
127
Q

G6PD deficiency prognosis

A

associated with protective effect against malaria

acute renal failure can develop during times of acute hemolysis

class II variants: hemolysis will continue well after trigger is discontinued

class III variants: even with continued exposure to trigger, acute hemolytic process ends after ~1 week (with the reversal of anemia)

128
Q

G6PD deficiency screening

A

all personnel being deployed to malaria endemic regions must be screened

all personnel entering military service

universal newborn screening in many malaria-endemic regions

newborn screening in US state dependent (MI not involved)

129
Q

hereditary spherocytosis/elliptocytosis

A

inherited forms of hemolytic anemia with presence of spherocytes or elliptocytes in peripheral circulation

heterogenous genetic pattern and clinical manifestations (presentations vary greatly)

130
Q

hereditary spherocytosis/elliptocytosis diagnostic evaluation

A

presence of spherocytes or elliptocytes on peripheral smear

characteristic feature is increased MCHC

131
Q

hereditary spherocytosis/elliptocytosis treatment

A

includes splenectomy in severe cases

132
Q

acquired hemolytic anemias

A 
autoimmune hemolytic anemia
gold agglutinin disease
paroxysmal nocturnal hemoglobinuria
drug-induced hemolytic anemia
infection
mechanical RBC destruction
thrombotic microangiopathy
133
Q

acquired hemolytic anemia caused by presence of IgG auto antibodies

A

autoimmune hemolytic anemia

134
Q

autoimmune hemolytic anemia

A

50% of cases are idiopathic; can also develop in association with SLE, CLL, lymphoma

auto-antibodies bind RBC membrane most avidly at body temperature

auto-antibody coated RBC leads to extravascular hemolysis in spleen and liver

occasionally intravascular hemolysis may occur due to complement activation

135
Q

autoimmune hemolytic anemia clinical presentation

A

generally rapid onset

symptoms of anemia (fatigue, dyspnea, etc.)

symptoms of hemolysis (jaundice, splenomegaly, dark urine if predominant intravascular hemolysis)

136
Q

autoimmune hemolytic anemia diagnostic evaluation

A

decreased Hgb/Hct (can be severe with Hct <10%)

decreased platelets (minority of cases)

increased reticulocyte response (takes 3-5 days)

evidence of hemolysis (increased bilirubin and LDH, decreased haptoglobin)

peripheral smear: spherocytes, nucleated RBCs

positive direct antiglobulin test (direct Coombs’ test)

137
Q

autoimmune hemolytic anemia treatment

A

referral to hematologist

transfusions- unlikely that fully compatible blood will be found but transfused RBCs will survive ~as long as patients own RBCs

immune suppression: prednisone 1-2 mg/kg/day

splenectomy

plasmapheresis (in setting of rapid hemolysis)

138
Q

autoimmune hemolytic anemia prognosis

A

patients generally do well

death due to cardiovascular collapse in setting of rapid hemolysis may occur

139
Q

acquired hemolytic disease (usually extravascular) caused by presence of IgM autoantibodies

A

cold agglutinin disease

140
Q

cold agglutinin disease etiologies

A

usually idiopathic

can occur in association with Waldenstorom, macroglobulinemia, lymphoma, CLL

acute post-infectious cold agglutinin disease can develop after mycoplasma pneumonia or viral infection

141
Q

cold agglutinin disease

A

IgM autoantibodies react with RBCs at low temperatures

IgM attaches to RBC membranes in cooler parts of circulation (fingers, nose, ears) which causes complement binding and fixation

when RBCs return to warmer circulation, IgM dissociates, but complement remains bound

RBCs are sequestered and destroyed in the liver

142
Q

cold agglutinin disease clinical presentation

A

mottled fingers/toes, acrocyanosis, episodic low BP, dark colored urine in response to cold exposure

anemia rarely severe

143
Q

cold agglutinin disease diagnostic evaluation

A

decreased Hgb/Hct (usually mild)
increased reticulocyte response
intermittent evidence of hemolysis (increased bilirubin and LDH, decreased haptoglobin)
peripheral smear (made at room temp): agglutinated RBCs
direct antiglobulin test (direct Coombs’ test) positive for complement only
serum cold agglutinin titer available
protein electrophoresis often reveals monoclonal IgM

144
Q

cold agglutinin disease treatment- supportive

A

avoid exposure to cold

transfusions should be administered through an in-line blood warmer

145
Q

cold agglutinin disease treatment- more severe forms

A

can be difficult to manage

plasmapheresis or IV immunoglobulin may be used until immunosuppressive agents take effect

Rituximab treatment of choice to reduce Ab production

146
Q

paroxysmal nocturnal hemoglobinuria

A

rare acquired hematopoietic stem cell disorder-may appear de novo or in setting of aplastic anemia

intrinsic RBC abnormality leads to unregulated formation of complement membrane attack complexes and ultimately to intravascular hemolysis

147
Q

paroxysmal nocturnal hemoglobinuria clinical presentation

A 
may be asymptomatic
episodic intravascular hemolytic anemia (reddish brown urine-more pronounced in am)
venous thrombosis (especially in hepatic, mesenteric, CNS, and skin veins)
148
Q

paroxysmal nocturnal hemoglobinuria diagnostic evaluation

A

decreased Hgb/Hct (variable severity and frequency)
variable RBC morphology
decreased WB/ decreased platelets may be present
normal to increased reticulocyte response during times of hemolysis
intermittent evidence of hemolysis
decreased iron +/- decreased folate common
peripheral smear: polychromasia
flow cytometry demonstrates decreased CD55 and CD59
may require diagnostic evidence based on symptoms

149
Q

paroxysmal nocturnal hemoglobinuria treatment

A

usually referred to hematologist
supportive care for mild cases (serum folate and iron supplementation prn)
eculizumab may be used if severe hemolysis or thrombosis present
hematopoietic cell transplant may be required in severe cases
thromboses require anticoagulation

150
Q

paroxysmal nocturnal hemoglobinuria prognosis

A

life expectancy for severe forms 10-15 yrs

major cause of death is thrombosis

possible progression to myelodysplasia or AML

151
Q

drug-induced hemolytic anemia

A

drugs can elicit immune reaction resulting in hemolysis- will cause positive Direct Coombs test

drugs with oxidative potential can cause hemolysis

drugs can cause hemolysis via non-oxidative, unknown mechanisms

152
Q

infectious causes of hemolytic anemia

A

malaria common cause of acquired hemolytic anemia in endemic regions

Shiga toxin-producing E. coli can cause Shiga toxin hemolytic uremic syndrome

Clostridium perfringens sepsis

153
Q

mechanical destruction of RBCs

A

microangiopathic hemolytic anemia

March hemoglobinuria

154
Q

microangiopathic hemolytic anemia

A

descriptive term for non-immune intravascular hemolysis

can occur due to abnormalities in microvasculature, intravasculature devices

155
Q

March hemoglobinuria

A

acute hemolytic anemia secondary to mechanical RBC damage in marathon runners, prolonged barefoot dancing

156
Q

hemolytic disease of the fetus and newborn

A

destruction of neonatal or fetal RBCs by maternal IgG Ab

Rh(D) hemolytic disease is the most sever

can also have ABO hemolytic disease and other blood group Abs

157
Q

hemolytic disease of the fetus and newborn clinical presentation

A

variable

mild-moderate disease: hyperbilirubinemia within 1st 24 hrs of life; +/- symptomatic anemia

hydrops fetalis

158
Q

hemolytic disease of the fetus and newborn diagnostic evaluation antenatal

A

maternal ABO testing, screening for Ab to RBC antigen

159
Q

hemolytic disease of the fetus and newborn diagnostic evaluation postnatal

A

demonstration of incompatible blood types between infant and mother

demonstration of hemolysis

positive direct or indirect antiglobulin test (Coombs test)

160
Q

hemolytic disease of the fetus and newborn treatment antenatal

A

monitoring of Ab titers and fetal condition

fetal RBC transfusions prn

161
Q

hemolytic disease of the fetus and newborn treatment postnatal

A

treat anemia with supportive care +/- PRBC transfusion, EPO agents

treat hyperbilirubinemia with supportive care +/- phototherapy, exchange transfusion

162
Q

hemolytic disease of the fetus and newborn prevention

A

Rh(D) typing and Ab screen should be performed at 1st prenatal visit

administration of anti-D immune globulin to Rh(D) negative women exposed, or at high risk of being exposed, to Rh(D) positive RBCs

163
Q

Rh(D) typing and Ab screen should be performed at 1st prenatal visit

A

mothers with positive screen should be assessed for risk of clinically significant hemolytic disease of fetus/newborn and managed accordingly

Rh(D) negative mothers with negative initial screening should have repeat Ab screen at 28 weeks gestation and delivery

164
Q

administration of anti-D immune globulin to Rh(D) negative women exposed, or at high risk of being exposed, to Rh(D) positive RBCs

A

routinely administered at 28 weeks gestation and within 72 hours of delivery to Rh(D) positive newborn

administration following any event associated with placental trauma or disruption of fetomaternal interface

PAS 512 Clinical Medicine 1 (3 decks)

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