Hematology #1 Flashcards

1
Q

MCC of anemia worldwide

A

Iron deficiency anemia

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2
Q

What is the most common cause of iron deficiency anemia in the US
What is the most common cause of iron deficiency anemia in the world

A
  • Chronic blood loss: menstruation, Colon cancer, Hookworms

- Diet (worldwide)

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3
Q

Risk factors for iron deficiency anemia

A
  • Increased metabolic requirements: children, pregnant, lactating women
  • Cow milk ingestion in young children: infants given cow’s milk < 1 year of age
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4
Q

Symptoms of iron deficiency anemia

A
  • Fatigue, weakness, exercise intolerance, dyspnea
  • Pasophagia (craving for ice)
  • Pica (craving for non-food substances)
  • Koilonychia (spooning of nails)
  • Angular cheilitis (inflammation of both corners of mouth)
  • Glossitis (smooth tongue)
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5
Q

What do iron studies in iron deficiency anemia show?

A
  • Decreased ferritin
  • Increased TIBC (transferrin)
  • Decreased transferrin saturation
  • Decreased serum iron
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6
Q

What gives a definitive diagnosis for iron deficiency anemia?

A

Bone marrow biopsy: absent iron stores

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7
Q

What is ferritin?

A

Ferritin stores iron in the blood

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8
Q

What is transferrin?

A

Transporters of iron in the blood (ferry)

-TIBC is the binding capacity of transferrin, so if they are increased, there is low iron in the blood

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9
Q

Treatment for iron deficiency anemia

A

-Iron replacement

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10
Q

What are some tips you should give patients when telling them how to take their iron replacement?

A

-Take iron with vitamin C (ascorbic acid), with water or orange juice and take on an empty stomach

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11
Q

What is the main cause of lead poisoning anemia in the US

A

-ingestion or inhalation of environmental lead (paint chips or lead dust)

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12
Q

Symptoms of lead poisoning anemia

A
  • Neurologic symptoms: ataxia, fatigue, learning disabilities, wrist or foot drop
  • Intermittent abdominal pain, vomiting, loss of appetite
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13
Q

Diagnostic for lead poisoning anemia

A

-Serum lead level > 10 mcg/dL on venous sampling most accurate

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14
Q

What does a peripheral smear for lead poisoning anemia?

A

Microcytic hypochromic anemia with basophilic stippling

-Ringed sideroblasts in the bone marrow

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15
Q

Treatment for lead poisoning anemia

A
  • Removal of the source of lead (most important treatment)

- If 45-69 mcg/dL: Succimer (oral) or Calcium disodium (if oral no tolerated)

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16
Q

What is thalassemia?

A

Decreased production of globin chains

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17
Q

When should you consider thalassemia?

A

If microcytic anemia with normal serum Fe or no response to Fe treatment

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18
Q

Explain the absorption of B12

A

B12 is released by acidity of the stomach and combines with intrinsic factor where it is absorbed mainly in distal ileum

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19
Q

B12 deficiency causes ________. It is needed to convert ______ to methionine for DNA synthesis.

A
  • Abnormal synthesis of DNA

- Homocysteine

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20
Q

Etiologies of B12 deficiency anemia

A
  • Pernicious anemia (lack of intrinsic factor)
  • Crohn Disease
  • Chronic alcohol use
  • H2 blockers and PPIs
  • Metformin
  • Vegan diet
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21
Q

What symptoms differ between B12 deficiency and folate deficiency anemia?

A
  • In B12 deficiency, there are neurologic abnormalities.
  • Symmetric paresthesias (MC initial symptom)
  • Decreased DTRs
22
Q

Labs for B12 deficiency

A
  • Macrocytic anemia (Increased MCV)
  • Increased homocysteine
  • Increased methylmalonic acid (distinguishes B12 from folate)
23
Q

For symptomatic B12 deficiency anemia or neuro findings, what should be given?

A

IM B12

-In adults, IM cyanocobalamin injection weekly until deficiency corrected, then monthly

24
Q

MCC of folate deficiency anemia

A

-Inadequate intake: alcoholics, unbalanced diet

25
Q

What do labs for folate deficiency show?

A
  • Increased MCV
  • Increased homocysteine
  • Normal methylmalonic acid!!!!
26
Q

MC initial presentation of sickle cell disease

A

Dactylitis (global swelling of finger or toe, sausage-like appearance)

27
Q

What is seen on a peripheral smear for sickle cell disease?

A
  • Sickled erthrocytes
  • Decreased hemoglobin and hematocrit
  • Howell-Jolly bodies
28
Q

Although DNA analysis is always the definitive diagnostic for sickle cell disease, what is seen on hemoglobin electrophoresis?

A

Sickle cell disease: HbS, little to no HbA, increased HbF

Sickle cell trait: HbS, decreased HbA

29
Q

What is the first step in management of pain crisis with sickle cell disease?

A

IV hydration and oxygen (reverses and prevents sickling)

30
Q

What else is needed for RBC production and DNA synthesis in a sickle cell patient?

A

Folic acid supplementation

31
Q

Hydroxyurea is given to sickle cell patients to reduce episodes. What is the MOA of Hydroxurea?

A

-Increases production of HbF (which does not sickle and has a higher affinity for oxygen), reduces RBC sickling

32
Q

What type of osteomyelitis is usually associated with sickle cell disease?

A

Salmonella Osteomyeltitis

33
Q

_______ is given as early as 2-3 months of age until at least 5 years old to prevent infectious complications

A

Prophylactic Penicillin

34
Q

Thrombotic Thrombocytopenic Purpura (TTP) is due to

A

ADAMTS13 deficiency

35
Q

What is the role of ADAMTS13?

A

Cleaves von Willebrand factor

36
Q

Therefore, a deficiency in ADAMTS13 leads to

A

Large vWF multimers that cause small vessel thrombosis

37
Q

Pentad of symptoms for TTP

A
  • Thrombocytopenia: mucocutaneous bleeding
  • Microangiopathic anemia: splenomegaly
  • Neuro symptoms: headache, confusion, seizures
  • Fever
  • Kidney failure or uremia
38
Q

Initial treatment of choice for TTP

A

-Plasmapheresis

39
Q

Triad of symptoms with Hemolytic Uremic Syndrome (HUS)

A
  • Thrombocytopenia
  • Hemolytic Anemia
  • Renal dysfunction (uremia)
40
Q

Risk factors for HUS

A

-Predominantly seen in children with a recent history of gastroenteritis

41
Q

What do labs show for HUS?

A
  • Thrombocytopenia with normal coagulation studies
  • Hemolysis: schistocytes (helmet cells)
  • Increased BUN and Creatinine
42
Q

Treatment for HUS

A
  • Supportive therapy (fluid and electrolyte replacement, dialysis)
  • Plasmapheresis
  • NO ABX or ANTI-MOTILITY AGENTS
43
Q

hemophilia A is a deficiency of

A

Factor VIII

44
Q

Hemophilia A is an ______ disorder that occurs almost exclusively in _____. Because it is a deficiency of Factor VIII, it leads to failure of ______

A

X-linked recessive
Males
hematoma formation

45
Q

Hemophilia B is an ______ disorder. It occurs almost exclusively in _____. It is a lack of factor ____ and therefore, can lead to a failure of hematoma formation.

A

X-linked recessive
Males
Factor IX (9)

46
Q

Symptoms of Hemophilia A and Hemophilia B

A
  • Hemarthrosis: delayed bleeding or swelling weight-bearing joints
  • Excessive hemorrhage in response to trauma and surgery (tooth extraction, epistaxis)
47
Q

What is aPTT?

A

Tells how long it takes for your blood to make a clot

-Tests response to anticoagulant therapies

48
Q

What is PT?

A

Tells how long it takes for your blood to form a clot

49
Q

Treatment for Hemophilia A

A
  • Factor VIII infusion: first-line therapy

- Desmopressin (prior to procedures to prevent bleeding)

50
Q

Treatment for Hemophilia B

A
  • Factor IX infusion (first line therapy)

- Desmopressin is NOT useful in this type