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Heme > Anemias (lecture 1&2) > Flashcards

Anemias (lecture 1&2) Flashcards

1
Q

What is anemia?

A

Decrease in RBC mass

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2
Q

Changes in red cell mass is inferred from what two things?

A

Hemoglobin concentration or hematocrit

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3
Q

What is another description of anemia?

A

Insufficient red cell production and/or oxygen delivery

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4
Q

What are the WHO guidelines for men and women with anemia?

A

Hemoglobin <13 g/dL in men

Hemoglobin <12 g/dL in women

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5
Q

The kinetics of RBC production and destruction discloses what?

A

The etiology of anemia

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6
Q

How is anemia graded?

A

1-4

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7
Q

What is another way to grade anemia?

A

Mild (1), moderate (2), severe (3), life-threatening (4)

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8
Q

What is the Hgb for mild/grade 1 anemia?

A

Hgb 10g/dL to lower limit of normal

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9
Q

What is the Hgb for moderate/grade 2 anemia?

A

Hgb 8.0-9.9 g/dL

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10
Q

What is the Hgb for severe/grade 3 anemia?

A

Hgb 6.5-7.9 g/dL

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11
Q

What is the Hgb for life-threatening/grade 4 anemia?

A

Hgb <6.5 g/dL

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12
Q

The RBC comprises what % of whole blood product?

A

45%

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13
Q

RBC function

A

Specialized to carry oxygen to tissues and organs, full of hemoglobin, no organelles.
Facilitate CO2 elimination

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14
Q

What is the RBC life span?

A

100-120 days

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15
Q

How are RBCs destroyed?

A

Sensecence-hemolysis

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16
Q

Retired RBCs are removed via what?

A

The RES, reticulate endothelial system

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17
Q

What maintains our iron stores?

A

Scavenging by hemopexin and Haptoglobin

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18
Q

New RBC production is managed by what?

A

EPO and HIF

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19
Q

Hemoglobin molecule is comprised of what?

A

2 alpha-globin chains and 2 beta-globin chains

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20
Q

The alpha-and beta-globin chains are bound to what?

A

Heme group which contains iron

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21
Q

Hemoglobinopathies can result from what?

A

Mutations in the amino acid sequence that form the globin chains

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22
Q

Production of RBCs is dependent upon raw materials including what?

A

Iron, B12, folate

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23
Q

What type of stem cell do RBCs come from?

A

Pluripotent stem cells

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24
Q

What is a committed cell?

A

Proerythtoblasts

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25
Q

What type of cells help with Hgb synthesis and accumulation?

A

Erythroblast

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26
Q

What type of cell expels its nucleus once the Hgb concentration reaches approximately 34%?

A

Normoblast

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27
Q

Reticulocytes contain what?

A

Still contain some ribosomes which are degraded by intracellular enzymes 1-2 days after being released into the bloodstream

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28
Q

What happens when there is low blood oxygen?

A

Liver and kidney release erythropoietin into the blood stream which goes to red bone marrow and tells it to increase number of RBCs to increase the oxygen carrying-capacity

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29
Q

EPO acts as what?

A

The messenger to the bone marrow to enhance red cell production

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30
Q

EPO increasing RBC production leads to what?

A

An increase in committed cells (proerythroblasts) and subsequently reticulocytes

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31
Q

Aging RBCs become what?

A

Less flexible and more fragile

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32
Q

Where are aging RBCs phagocytized?

A

But he macrophages in the liver, spleen and bone marrow

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33
Q

What happens to the RBCs once they are engulfed by macrophages?

A

Hemoglobin is broken down into heme group and protein component (globin)

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34
Q

Heme group is broken down into what?

A

Iron is recycled and remainder gets degraded to bilirubin

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35
Q

What are some ways to evaluate the RBC indices?

A

MCV, MCH, MCHC, RDW

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36
Q

What is the MCV?

A

Average size of the RBC

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37
Q

What are the classifications of MCV?

A

Microcytic: <80fl
Normocytic: 80-100fl
Macrocytic: >100fl

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38
Q

What is MCH?

A

Average weight of hemoglobin in RBCs

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39
Q

What is MCHC?

A

Average concentration of hemoglobin in RBCs

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40
Q

What are the classifications of MCHC?

A

Hypochromic, Normochromic

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41
Q

What is RDW?

A

Calculation of variation in RBC size

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42
Q

When are basophilic stippling seen?

A

Burn and sepsis

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43
Q

Under normal conditions RBC loss =?

A

RBC production

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44
Q

When reticulocyte activity is increased in setting of anemia…

A

It indicates bone marrow is attempting to keep up with RBC loss/destruction

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45
Q

Low reticulocyte activity can indicate what 3 things?

A
  1. Marrow infiltration
  2. Marrow failure
  3. Deficiency in raw materials
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46
Q

What are some examples of marrow infiltration?

A

Leukemia, lymphoma, malignancy

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47
Q

What are some examples of marrow failure?

A

Pure red cell aplasia, MDS, fibrosis

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48
Q

What raw materials can be deficient to cause low reticulocyte activity?

A

B12, iron, folate

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49
Q

As anemia worsens, what happens to reticulocytes?

A

Leave the bone marrow sooner and are in circulation before they are mature, polychromatic seen on smear

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50
Q

What are the 4 types of MICROCYTIC anemia?

A
  1. Iron deficiency
  2. Anemia of chronic disease
  3. Thalassemia
  4. Siderobalstic
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51
Q

What is the most common cause of anemia world wide?

A

Iron deficiency anemia

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52
Q

What is the most common cause of iron deficiency anemia?

A

Bleeding, GI or menstrual

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53
Q

What are the different stages of iron deficient anemia?

A

Depletion of iron stores without anemia, then anemia with nl RBC size, then anemia with reduced RBC size

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54
Q

The average American diet contains how much iron?

A

10-15 mg, only 10% absorbed

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55
Q

Where is most dietary iron absorbed?

A

Under acidic conditions in the stomach, duodenum and jejunum

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56
Q

How much iron do males and non-menstruating females need?

A

1mg daily

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57
Q

How much iron do menstruating females need?

A

3-4mg/day

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58
Q

How much iron do pregnant females need?

A

2-5mg/day

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59
Q

Bleeding results in a loss of how much iron?

A

50mg Fe in 100cc of whole blood

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60
Q

What are the 4 different causes of iron deficiency?

A
  1. Deficient dietary intake/decreased absorption
  2. Increased requirement
  3. Blood loss
  4. Other
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61
Q

Deficient dietary intake/ Decreased absorption of iron

A

Celiac spruce, zinc deficiency

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62
Q

Increased requirement of iron?

A

Pregnancy, lactation

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63
Q

What “other” can cause iron deficiency?

A

Hemoglobinuria, idiopathic, iron sequestration

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64
Q

What can iron deficiency anemia initially present as?

A

Normocytic/normochromic and progress to microcytic/hypochromic

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65
Q

What are some Hx clues to iron deficient anemia?

A

Diet, Pica, phagophagia, glossitis, mouth soreness, angular chilitis, koionychia, dysphagia

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66
Q

What does the work up for Fe deficient anemia consist of?

A

Serum iron, TIBC, transferrin, ferritin

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67
Q

What are the signs and symptoms of Fe deficiency anemia?

A

Easily fatigued, conjunctival pallor, tachy, palpitations, DOE, pica-consumption of non-nutritive substances like ice clay or chalk, smooth tongue, brittle nails, koilonychia, cheilosis

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68
Q

What lab results would show Fe deficient anemia?

A

Low MCV and MCH, peripheral smear: RBCs hypochromic microcytic

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69
Q

What will iron studies show for someone with Fe deficiency anemia?

A

Low serum iron, low transferrin saturation, low serum ferritin, high TIBC

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70
Q

Identifying the cause of what is crucial for Fe deficient diagnosis?

A

BLEED! Where is it coming from?

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71
Q

What is the treatment for Fe deficiency?

A

Ferrous sulfate 325mg TID, 10mg is absorbed, take with VitC to increase absorption

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72
Q

What are some side effects of Ferrous sulfate?

A

GI upset, constipation

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73
Q

When do labs and iron studies need to be repeated if pt is put on Ferrous sulfate?

A

2-3 weeks of therapy-values should normalize but continue treatment for 3-6 mos

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74
Q

What inhibits iron absorption?

A

Fiber, dairy products, phosphates, and tea (tannins)

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75
Q

Who is parenteral iron an option for with Fe deficiency?

A

If they do not tolerate PO iron, refractories to PO iron, GI disease which limits absorption, continued blood loss

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76
Q

What are the two parenteral iron options?

A 
Iron sucrose (venofer) given IVP over 2-5 mins OR
Sodium ferric gluconate (Ferrlicit) given IV over 30 mins
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77
Q

When should a patient with Fe deficiency be referred?

A

Not necessary unless the etiology is not clear or pts if refractory to treatment

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78
Q

Anemia of chronic disease-problem with what?

A

Problem with iron utilization and bone marrow unable to respond to EPO

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79
Q

Anemia of chronic disease is seen with what?

A

Chronic inflammation, endocrine disorders and infection

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80
Q

What are some examples of chronic diseases causing anemia?

A

Chronic liver, connective tissue disorders (SLE, RA), AI, hypothyroid, hypopituitarism, hyperparathyroidism, hyperthyroidism, endocarditis, viral, bacterial, parasitic, fungal infections, cancers (liquid and solid tumors)

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81
Q

What is anemic of chronic disease NOT seen in?

A

DM, COPD, CHF, or HTN

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82
Q

Chronic diseases produce massive amounts of what?

A

Inflammatory cytokines-IL-6 which stimulate hepatocytes to make massive amounts of hepcidin

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83
Q

What does the massive amounts of hepcidin do?

A

Prevents the release of iron from macrophages and liver stores (AKA access to iron is obstructed)

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84
Q

“You cant make chocolate chip cookies without chocolate chips”

A

Anemia of chronic disease: no iron because its stuck in storage

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85
Q

How is anemic of chronic disease different than anemia of CKD?

A

The underlying pathology impairs the kidneys to produce adequate EPO in CKD anemia

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86
Q

What are some lab results for chronic disease anemia?

A

CBC: normo, low reticulocyte count (low EPO levels), low production, normal or elevated serum ferritin

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87
Q

What will the iron studies show for anemia of chronic disease?

A

Decrease serum iron, decreased or normal TIBC with low saturation, FERRITIN IS UP OR NORMAL

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88
Q

What other lab levels can be elevated in anemia of chronic disease?

A

Increased ESR or CRP

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89
Q

How do you treat anemia of chronic disease?

A

Treat underlying condition, once treat the cause of inflammation, then anemia will resolve

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90
Q

What is EPO

A

CSF: coronary stimulating factor and ESF: erythropoiesis stimulating factor, stimulate production of RBCs

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91
Q

What is needed before starting EPO therapy?

A

Get baseline Hct/Hgb, baseline iron studies, ongoing lab monitoring important, educate pt on side effects, dont use in cancer pts if transplant candidate

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92
Q

Initiate EPO if Hgb is what?

A

<10 g/dL

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93
Q

Resolution of stabilization of anemia can be seen with EPO therapy is Hgb is what?

A

> 12g/dL or there is a resolution of symptoms

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94
Q

What are the side effects of EPO?

A

BBW for pts with renal disease, HTN, edema, HA, SOB, stroke or TIA, N/V, arthralgia, myalgia, pruritis/rash, pain at injection site

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95
Q

What is a heterogeneous group of disorders associated with decreased or absent synthesis of alpha and beta globin chains?

A

Thalassemia

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96
Q

Severe thalassemia is when the disease occurs when?

A

Early in life

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97
Q

Thalassemia is often confused with what?

A

Iron deficiency anemia because of decreased MCV

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98
Q

What can be helpful in evaluation of thalassemia?

A

Hemoglobin electrophoresis

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99
Q

Severity of thalassemia is dependent upon what?

A

The number of genes with mutations

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100
Q

Alpha thalassemia is primarily due to gene what?

A

Deletions (16) causing reduced alpha globin chain synthesis

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101
Q

Beta thalassemia is usually caused by what?

A

Point mutations rather than deletions

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102
Q

Defective globin chains in beta-thalassemia causes what?

A

Decreased normal Hb production as well as relative excess alpha chains

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103
Q

What type of thalassemia is usually seen in people form southeast Asian and china?

A

Alpha thalassemia

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104
Q

How many copies of the alpha globin chain is considered normal?

A

4

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105
Q

If someone with alpha thalassemia only has 3 copies of the alpha globin chain…

A

They are considered a silent carrier

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106
Q

If someone with alpha thalassemia only has 2 copies then what?

A

Considered alpha thalassemia trait (aka thalassemia minor)

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107
Q

If someone with alpha thalassemia has 1 copy then what?

A

Called HbH disease

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108
Q

If someone with alpha thalassemia has 0 copies, then what?

A

Called hydros fetalis

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109
Q

What symptoms are associated with 3 alpha chains?

A

Make enough Hb, no symptoms

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110
Q

What symptoms are associated with 2 alpha chains?

A

Mild symptoms

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111
Q

What symptoms are seen with only 1 copy of the gene?

A

Ischemia, Target cells, Heinz bodies

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112
Q

0 alpha copies

A

Fatal

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113
Q

Alpha thalassemia signs and symptoms

A

Carriers- no symptoms, fatigue, weakness, pallor, splenomegaly

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114
Q

What labs should be drawn to test for alpha thalassemia?

A

CBC, CMP, smear, Ferritin, Hb Electrophoresis

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115
Q

What will be the results of the blood work for alpha thalassemia?

A

CBC: hypochromic, microcytic
Smear: normal to target cells and Heinz bodies
Ferritin: to rule out iron deficiency

116
Q

What are the treatments for someone who is an alpha thalassemia carrier/trait?

A

No treatment, transfusions as needed, folic acid and iron supplementation

117
Q

What are the treatments if someone has HbH disease?

A

Transfusions as needed, consider iron chelation for pts who might have iron overload

118
Q

What are some other treatment options for alpha thalassemia?

A

Hemolytic episodes might be triggered by infection or drugs, can do splenectomy and SCT (stem cell transplant), offer genetic counseling

119
Q

Beta thalassemia primarily affects who?

A

People of Mediterranean decent

120
Q

Beta thalassemia subgroups?

A

Major (homozygous) and Minor (heterozygous)

121
Q

Beta (0) thalassemia mutation results in what?

A

Absence** of production of beta globin. Pts homozygous or doubly heterozygous cannot make normal beta chains and thus are unable to make any hemoglobin A

122
Q

Beta (+) thalassemia mutation results in what?

A

Decreased** production of beta globin. Pts homozygous are able to make some hemoglobin A, and are generally less severely affected than those homozygous for beta (0) genes

123
Q

Beta thalassemia major

A

No effective production or severely limited production of beta globin

124
Q

Beta thalassemia minor

A

AKA beta thalassemia trait, reduced beta globin production

125
Q

What are the signs and symptoms for beta thalassemia minor (heterozygous)?

A

Most are asymptomatic, smear shows hypochromic, microcytic anemia, note target cells, dacrocytes, basophilic stippling

126
Q

What can be seen on smear for beta-thalassemia minor?

A

Hypochromic, microcytic anemia, target cells, dacrocytes, basophilic stippling

127
Q

What are the signs and symptoms for beta-thalassemia major?

A

Pallor, irritability, growth retardation, abdominal swelling

128
Q

What will the smear show for beta-thalassemia major?

A

Poikilocytosis, hypochromic, microcytic, target cells, basophilic stippling

129
Q

What labs should be drawn for beta-thalassemia?

A

CBC, RDW, CMP, Ferritin, Hb Electrophoresis

130
Q

What will the results show for beta-thalassemia major?

A

CBC: microcytic hypochromic cells, target cells, dacrocytes, basophilic stippling, poikilocytosis
Ferritin: usually normal

131
Q

What is the treatment for beta-thalassemia major?

A

Transfusions as needed, chelation if iron overloaded, splenectomy, SCT, supplemental folic acid, genetic counseling

132
Q

When are transfusions needed for beta-thalassemia major?

A

Most required ruing periods of rapid growth, pregnancy, growth spurts, and infection

133
Q

Bone marrow makes ringed sideroblasts in what?

A

Sideroblastic anemia

134
Q

What is seen in a workup for sideroblastic anemia?

A

Elevated iron, ferritin, and RDW, hypochromic RBCs, iron overload

135
Q

Sideroblastic anemia is associated with what?

A

Cu deficiency

136
Q

What drugs can cause sideroblastic anemia?

A

Chloramphenicol, Linezolid

137
Q

The body has plenty of iron but cannot incorporate it into hemoglobin in what?

A

Sideroblastic anemia

138
Q

What are some signs and symptoms of sideroblastic anemia?

A

Pale, fatigue, dizziness, hepatosplenomegaly

139
Q

What labs should be done for siderobalstic anemia?

A

CBC, CMP, iron studies, peripheral smear, lead level, copper level

140
Q

What will the lab results show for sideroblastic anemia?

A

CBC: microcytic, hypochromic RBCs
CMP: liver and kidney abnorms due to iron overload

141
Q

What will iron studies show for sideroblastic anemia?

A

Increased serum iron, decreased TIBC

142
Q

What will a peripheral smear show for sideroblastic anemia?

A

Abnormal cell shapes, basophilic stippling, target cells, Pappenheimer bodies

143
Q

What is the treatment for sideroblastic anemia?

A

Transfuse as needed, chelate as needed, replete copper, does NOT respond to EPO because the bone marrow is making abnormal RBCs

144
Q

In INH induced sideroblastic anemia, the treatment is what?

A

B6 depletion

145
Q

In severe cases of sideroblastic anemia, the treatment is what?

A

Bone marrow transplant

146
Q

Hemolytic anemias main facts

A

Normocytic in size, color seems to be normal, no longer a production issue! Its full on destruction

147
Q

Anemia = ?

A

Decrease in RBCs or RBCs dont have enough hemoglobin

148
Q

Hemolytic anemia is caused by what?

A

High rate of cell destruction, not a production problem

149
Q

The treatment for hemolytic anemia depends on what?

A

Severity of disease and whether inherited or acquired

150
Q

What is the classification for hemolytic anemia?

A

Intrinsic: defect intrinsic to RBC
Extrinsic: External factors causing anemia

151
Q

What types of defects can cause inherited intrinsic hemolytic anemia?

A

Membrane defects, enzyme defects, and hemoglobinopathies

152
Q

What type of membrane defects can cause hemolytic anemia?

A

Hereditary spherocytosis, hereditary elliptocytosis

153
Q

What enzyme defect can cause hemolytic anemia?

A

G6PD deficiency

154
Q

What types of hemoglobinopathies can cause hemolytic anemia?

A

Sickle cell syndrome, thalassemia

155
Q

What can cause acquired and extrinsic hemolytic anemia?

A

Immune, miroangiopathic, infections, hypersplenism, and burns

156
Q

Microangiopathic causes for hemolytic anemia

A

TTP, HUS, DIC, valve hemolysis

157
Q

What types of infections can cause hemolytic anemia?

A

Plasmodium, Clostridium, Borrelia

158
Q

What are the main subtypes of hemolytic anemia?

A

Hereditary spherocytosis, G6PD deficiency, paroxysmal nocturnal hemoglobinuria, autoimmune hemolytic anemia, sick cell disease

159
Q

What are the signs and symptoms fo hemolytic anemia?

A

Varies based on underlying condition
Fatigue, weakness, pallor, easy bruising, thrombus, petechiae and purpura, yellow discoloration of skin/eyes, abdominal pain, dark urine

160
Q

What are some physical exam findings for hemolytic anemia?

A

Pallor, jaundice, sclera icterus, dark/discolored urine, splenomegaly, petechiae, purpura, ecchymosis, abdominal tenderness

161
Q

Bilirubin is a breakdown of what portion of RBC?

A

Heme portion

162
Q

What are the types of bilirubin?

A

Total bilirubin, indirect bilirubin (unconjugated), direct bilirubin (conjugated), urobiliongen

163
Q

What are some lab studies common to all hemolytic anemia?

A

Elevated reticulocytosis with stable or falling hemoglobin, decreased haptoglobin positive urine hemosiderin, increased indirect bilirubin, increased total bilirubin increased serum LDH increased methemalbuminemia, increased hemoglobinemia

164
Q

What are some other lab studies to be drawn?

A

Direct and indirect Coombs test, hemoglobin Electrophoresis, Heinz body stain, osmotic fragility, peripheral smear, flow cytometry, cold agglutin titer, bone marrow biopsy

165
Q

Crystallizing hemoglobin is found in what?

A

Sick cell disease

166
Q

What are Heinz body formations seen in?

A

Unstable hemoglobin variants, G6PD, enzyme defect

167
Q

What can sphereocytes be seen in?

A

HS, result of spleen or partial phagocytosis when part of membrane removed

168
Q

What is the treatment for membranopathies?

A

Splenectomy in some moderate and most severe cases

169
Q

What is used to diagnose membranopathies?

A

Spherocytes, family history, negative DAT

170
Q

What is the treatment for enzymopathies?

A

Withdrawal of offending drug, treatment of infection

171
Q

How can you diagnose enzymopathies?

A

Low G6PD activity measurement

172
Q

What is the treatment for hemoglobinopathies?

A

Folate, transfusions

173
Q

How do you diagnose hemoglobinopathies?

A

Hemoglobin Electrophoresis, genetic studies

174
Q

How do you treat immune-mediated hemolytic anemia?

A

Treat underlying disorder, removal of offending drug: steroids, splenectomy, IV gamma globulin, plasmapheresis, cytotoxic agents, or Danocrine, avoidance of cold

175
Q

How do you diagnose immune-mediated hemolytic anemia?

A

Spherocytes and positive DAT

176
Q

What is the treatment for microangiopathic hemolytic anemia?

A

Treatment of underlying disorder

177
Q

How do you diagnose microangiopathic hemolytic anemia?

A

Schistocytes

178
Q

Normal RBCs are what shape?

A

Biconcave shaped disc with membrane built to last 120 days

179
Q

RBCs travel through where?

A

Capillaries and spleen so need a flexible and formidable membrane

180
Q

What are the 2 proteins that the RBC membrane is made out of?

A

Spectrin and Actin they are the scaffolding of the membrane

181
Q

In hereditary spherocytosis, what is abnormal?

A

Spectrin and Ankyrin are ABNORMAL

182
Q

What is the result of abnormal spectrin and ankyrin in spherocytosis?

A

Small, hyperchromic, inflexible cells that cant navigate through the spleen

183
Q

The spleen notices that the RBCs cant fit through and what occurs?

A

Hemolysis

184
Q

What are the key findings for hereditary spherocytosis?

A

Family hx, splenomegaly, spherocytes and retics on smear, hyperchromic

185
Q

What is the most common hemolytic anemia due to cell membrane defect?

A

Hereditary spherocytosis

186
Q

The diagnosis for hereditary spherocytosis is often based on what?

A

Clinical grounds

187
Q

What can diagnose hereditary spherocytosis?

A

Spherocytes on smear, familiar hemolytic anemia, abnormal osmotic fragility test or other screening test, Coombs negative

188
Q

What is the treatment for hereditary spherocytosis?

A

Supportive- transfusions as needed. Folic acid supplementation

189
Q

What can be considered for hereditary spherocytosis if severe?

A

Splenectomy, but it will not cure the disease just removes the site of hemolysis

190
Q

What is PNH?

A

Paroxysmal nocturnal hemoglobinuria

191
Q

Rare colonial hematopoietic stem cell disorder

A

PNH

192
Q

Abnormal sensitivity of RBC membranes to lysis by compliment?

A

PNH

193
Q

What is deficient in PNH?

A

Compliment regulating proteins CD55 and CD59

194
Q

CD55 and CD59 deficits permits what?

A

Unregulated formation of complement membrane attack complex on RBC membranes and intravascular hemolysis

195
Q

Patients with significant PNH live how long?

A

10-15 years, thrombosis primary cause of death

196
Q

PNH is mostly a disease of who?

A

Adults, although childhood causes have been reported

197
Q

What are some signs and symptoms for PNH?

A

Episodic hemoglobinuria, symptoms of anemia: fatigue, weakness, varies with severity, esophageal spasms, ED, abdominal pain, dyspnea, thrombosis, pulmonary HTN, end organ damage, CKD

198
Q

What is episodic hemoglobinuria?

A

Reddish brown urine, often first morning urine. Not actually bloody but hemoglobin in urine

199
Q

Where does thrombosis take place in PNH?

A

Mesenteric vein, hepatic veins, central nervous system veins, skin vessels (formation of painful nodules)

200
Q

Variation in severity of hemoglobinuria within hours in unique to what condition?

A

PNH

201
Q

What is key to diagnose PNH?

A

Flow cytometry is key: CD55 cells

202
Q

What else is used to diagnose PNH?

A

CBC, reticulocytosis, direct Coombs: negative, urine hemosiderin, serum LD, iron deficiency common, bone marrow

203
Q

Bone marrow for PNH

A

Variable morphology hypoplasia or erythropoiesis hyperplasia or both; bone marrow karoyotype may be normal or demonstrate a colonial abnormality

204
Q

Lab results for PNH

A

CBC: variable anemia, leukocytosis, thrombocytopenia
Reticulocytosis: variable
Peripheral smear: nondiagnostic, macroovalocytes and polychormasia
Direct Coombs: negative*
Urine hemosiderin: elevated
Serum LD: elevated
Iron deficiency common

205
Q

PNH diagnosis key

A

Flow cytometry of granulocytes deficiency of CD55 and CD59*

206
Q

FLAER assay

A

Fluorescein labeled proaerolysin by flow cytometry, more sensitive for PNH

207
Q

Essentials of diagnosis for PNH

A

Episodic hemoglobinuria, thrombosis, suspect in confusing cases of hemolytic anemia or pancytopenia, flow cytometry results with CD55 and CD59 deficiency

208
Q

How do you treat severe PNH with hemolysis or thrombosis?

A

Transfusions as needed, Eculizumab** Therapy, corticosteroids, allergenic stem cell transplant

209
Q

What are some other treatments of PNH?

A

Iron deficiency if indicated, should be under care of hematologist

210
Q

Eculizumab therapy mechanism?

A

Monoclonal antibody against compliment protein C5 preventing membrane attack complex
Improved quality of life

211
Q

What are the pros to Eculizumab therapy for PNH?

A

Improved quality of life, reduced hemolysis, transfusion requirement, and thrombosis

212
Q

G6PD deficiency patho

A

Hereditary enzyme defect, extravascular hemolysis (spleen), episodic hemolytic anemia

213
Q

G6PD deficiency

A

Protection from malaria, less coronary artery disease, possibly fewer cancers, greater longevity

214
Q

What is the inheritance for G6PD deficiency?

A

X-linked disorder, affected 10-15% American black males hemizygous, female carriers rarely affected

215
Q

Numerous ___ isoenzymes have been described

A

G6PD, >150

216
Q

American black population has what G6PD variant?

A

G6PD-A, have normal function

217
Q

White population has what G6PD variant?

A

G6PD-B, have normal function

218
Q

10-15% of American black population has what G6PD variant?

A

G6PD-A-, reduction in normal enzyme activity and reduction stability

219
Q

The normal enzyme activity declines rapidly after RBC> ?

A

RBC> 40days

220
Q

Mediterranean, Ashkenazi Jewish and Asian variants

A

Extremely low enzyme activity (class 2)

221
Q

When should you expect G6PD?

A

An episode of onion-immune hemolytic anemia, especially if occurring after drug ingestion, exposure to fava beans, or associated with an infection

222
Q

Signs and symptoms of G6PD deficiency

A

Patients are generally healthy and without splenomegaly

223
Q

During an episode of hemolysis, what are the lab findings for G6PD?

A

Hgb >8g/dL, increased: reticulocyte count, serum indirect bilirubin
Peripheral smear: “bite cells” “blister” Heinz bodies-special stain, decreased G6PD enzyme assay

224
Q

What do the labs look like between hemolytic episodes in G6PD?

A

Normal

225
Q

X-linked recessive disorder seen commonly in African American men?

A

G6PD Deficiency

226
Q

Bite cells and blister cells on peripheral blood smear?

A

G6PD Deficiency

227
Q

Reduced levels of G6PD between hemolytic episodes

A

G6PD Deficiency

228
Q

What should be avoided if you have G6PD deficiency?

A

Avoid known oxidant drugs, pregnant/nursing who are heterozygous should avoid drugs with oxidant potential, avoid fava beans

229
Q

What is a treatment option for G6PD deficiency?

A

Transfusion if severe episode of hemolysis or symptomatic anemia

230
Q

What are the 3 most common drugs to avoid if you have G6PD?

A

Dapsone, Nitrofurantoin, Sulfa drugs

231
Q

What are some other drugs to avoid with G6PD?

A

Methylene blue, Rasburicase, Phenazopyridine, Primaquine, Tolonium chloride, Henna compounds

232
Q

Patho behind autoimmune hemolytic anemia

A

Autoantibodies IgG bind to RBC membrane at room temperature, can be seen in SLE, CLL, or lymphomas

233
Q

What is the presentation for autoimmune hemolytic anemia?

A

Fatigue, dyspnea, angina pictorial, possible heart failure, jaundice, splenomegaly

234
Q

Laboratory findings for autoimmune hemolytic anemia

A

Increased reticulocyte, + Direct Coombs, +/- Indirect Coombs, decreased haptoglobin, increased indirect bilirubin

235
Q

What will be seen on peripheral smear for autoimmune hemolytic anemia?

A

Spherocytes, NRBCs

236
Q

What are the essentials for diagnosis of autoimmune hemolytic anemia?

A

Acquired anemia caused by IgG autoantibody, spherocytes and reticulocytosis on peripheral smear, + Coombs test

237
Q

What two types of anemia both have spherocytes and reticulocytosis?

A

Autoimmune hemolytic anemia and Hereditary spherocytosis

238
Q

What is the main difference between AIHA and hereditary spherocytosis?

A

Coombs + in AIHA

239
Q

What is the treatment for AIHA?

A

Prednisone, transfusion-may be difficult to crossmatch, splenectomy, therapeutic plasma pheresis

240
Q

When should you refer to hematologist for pts with AIHA?

A

All patients

241
Q

When should you admit someone with AIHA?

A

If symptomatic anemia or rapidly falling hemoglobin

242
Q

What is hemolytic anemia due to IgM antibodies?

A

Cold Agglutinin disease

243
Q

Coombs test + and IgM antibodies is what?

A

Cold Agglutinin Disease

244
Q

What can cold Agglutinin disease occur with?

A

Waldenstrom macroglobulinemia, lymphoma, or CLL or post mycoplasma pneumoniae or certain viral illnesses

245
Q

What is the clinical presentation for cold Agglutinin disease?

A

Symptoms related to RBC agglutination on exposure to cold, mottle or numb fingers, toes, ears, acrocyanosis, episodic low back pain, dark colored urine, hemoglobinuria on exposure to cold

246
Q

What are the essentials for diagnosis of cold Agglutinin disease?

A

Increased reticulocytes on blood smear, Coombs + for compliment only, - for antibodies, + cold Agglutinin titer

247
Q

What is the treatment for cold Agglutinin disease?

A

Avoid exposure to cold, Rituximab or immunosuppressive therapy, if transfusion use in-line blood warmer

248
Q

What is an autosomal recessive disorder with abnormal hemoglobin, chronic hemolytic anemia and effects people of Africa can descent?

A

Sickle cell disease

249
Q

HbS gene for sickle cell is carried by how many American blacks?

A

8%

250
Q

Hemoglobin S (HbS)

A

Substitution of a Valine for a Glutamic acid as the 6th amino acid of the beta-globin chain

251
Q

HbS

A

Tetramer that is poorly soluble when deoxygenated

252
Q

Genotype AA will have what?

A

Normal diagnosis, no sickle cell

253
Q

Genotype AS will have what?

A

Sickle cell trait

254
Q

Genotype SS will have what?

A

Sickle cell anemia

255
Q

When is the onset of sickle cell disease?

A

During first year of life when HbF declines and production switches from gamma globulin to beta globulin

256
Q

“Sickling”

A

HbS is unstable, it changes shape with deoxygenation and undergo hemolysis, can occlude microcirculation

257
Q

RBC lifespan in sickle cell is what?

A

10-20 days

258
Q

What can cause a sickle cell disease crisis?

A

Infection, hypoxia, dehydration, acidosis, physical/physiological stress, extreme exercise, ETOH, pregnancy, cold weather

259
Q

What are some signs and symptoms for sickle cell disease?

A

Anemia, jaundice, pigment gallstones, splenomegaly, poor healing ulcers over lower legs, life threatening severe anemia, pain over long bones or back, chest pain, stroke, priapism, retinopathy, systemic (heart, lungs, liver) occlusion

260
Q

What are the 3 types of sickle cell disease crises?

A
  1. Vaso-occlusive crisis
  2. Hematologic crisis
  3. Infections
261
Q

Vaso-occlusive crisis

A

MSCK pain, dactylitis (hand-foot syndrome), acute chest pain syndrome, stroke, priapism

262
Q

Hematologic crisis

A

Splenic sequestration, aplastic crisis, hemolytic crisis

263
Q

Infections causing sickle cell crisis

A

Pneumonia, meningitis, sepsis, osteomyelitis, UTIs/renal infections

264
Q

Exam findings for sickle cell anemia

A

Chronically ill, jaundiced, hepatomegaly, spleen not palpable, cardiomegaly, hyperdynamic precordium and systolic murmurs, non-healing ulcers, retinopathy

265
Q

What are some complications for sickle cell disease?

A

Retinopathy, pulmonary HTN, delayed puberty, infections related to hyposplenism, bone necrosis, cardiomegaly, cholelithiasis

266
Q

What will a CBC show for sickle cell anemia?

A

Degree of anemia will vary; Hct usually 20-30%, WBC and platelets can be elevated

267
Q

What will the blood smear show for sickle cell anemia?

A

irreversibly sickle cells reticulocytosis, Howell-Jolly bodies and target cells

268
Q

What other lab tests must be drawn for sickle cell anemia?

A

Hemoglobin S screening test, hemoglobin electrophoresis chemistries demonstrate hemolysis, increased indirect bilirubin

269
Q

What are the essentials of diagnosis for sickle cell anemia?

A

Recurrent pain episodes, + family hx and lifelong hx of hemolytic anemia, irreversibly sickle cells on smear, hemoglobin S* seen on Electrophoresis

270
Q

What is the supportive treatment for sickle cell anemia?

A

Folic acid 1mg PO daily, pneumococcal vaccine, avoid known precipitating factors

271
Q

What is the treatment for an acute sickle cell crisis?

A

generous analgesia for pain transfusions for aplastic or hemolytic crisis, identify precipitating factors, keep well hydrated, oxygen, Abx, VTE prophylaxis, transfusion if needed

272
Q

What are some long term therapies for sickle cell anemia?

A

Long term transfusion therapy to reduce recurrent stroke in kids, disease modulators, allergenic hematopoietic stem cell transplants

273
Q

What disease modulators can be used for sickle cell anemia?

A 
Cytotoxic agents (Hydroxyurea) increases hemoglobin F levels
Omega-3 fatty acid supplementation
274
Q

Who can get allergenic hematopoietic stem cell transplants?

A

Kids with suitable HLA-matched donor, investigational in adults

275
Q

AS genotype

A

Heterozygous, hematologically normal, screening will be +, hemoglobin electrophoresis will show 40% HgbS

276
Q

What are the clinical risks if you have the sickle cell trait?

A

Risk of sudden cardiac death and rhabdo with vigorous exercise, especially at high altitudes, increased risk of VTE, microscope and gross hematuria, hyposthenuria, possible CKD

277
Q

When should you refer someone with sickle cell?

A

All should be referred to hematology and a comprehensive sickle cell center

278
Q

What are the benefits of sickle cell anemia?

A

Resistance to malaria: sickle cells have shorter survival, eliminated in spleen as well as parasite
Membrane is porous and leaks nutrients like K which is needed for parasite

279
Q

What are the 4 types of Microcytic anemia?

A
  1. Iron deficient
  2. Thalassemia
  3. Sideroblastic anemia
  4. Anemia of chronic disease
280
Q

What is the MCV for microcytic anemia?

A

<80fL

281
Q

What are the 4 types of normocytic anemias?

A
  1. Anemia of chronic disease
  2. Aplastic anemia
  3. Acute blood loss
  4. Most hemolytic anemia
282
Q

What is the MCV for normocytic anemia?

A

80-100fL

283
Q

What are the subtypes of macrocytic anemia?

A
  1. Megaloblastic

2. Macrocytic

284
Q

What are the 2 types of megaloblastic macrocytic anemia?

A
  1. Vit b12 deficiency

2. Folic acid deficiency anemia

285
Q

What are the 3 types of macrocytic anemia?

A
  1. Reticulocytosis
  2. Liver disease
  3. Alcoholism
286
Q

What is the MCV for macrocytic anemia?

A

> 100fL

Heme (3 decks)

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