Hematologic Flashcards

1
Q

What are symptoms of iron deficiency?

A

Pica, pagophagia
Koilonychia
Angular chelitis
Plummer-vinson (dysphagia + esophageal webs + atrophic gastritis + Fe deficiency)

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2
Q

How do you diagnose iron deficiency?

A

Fe < 150
CBC (microcytic, hypochromic):
Low MCV, MCHC, Fe, ferritin, & transferrin
Elevated RDW & TIBC

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3
Q

What are causes of iron deficiency?

A

MC = Blood loss (menstruation, occult - colon CA, hookworms)

Diet deficiency: pregnancy, breastfed infants

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4
Q

What are features of thalassemia?

A

Decreased production of globin chains

Microcytic w/ normal or increased Fe

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5
Q

What are the 4 types of alpha thalassemia? How many alleles are affected in each type?

A
  1. Silent carrier (1/4 alleles): asx
  2. Minor (2/4): mild microcytic anemia
  3. Intermedia aka Hgb H (3/4): chronic anemia, pallor, hepatosplenomegaly,frontal & maxilla bony overgrowth, gallstones, iron overload. Presents similar to beta-thal*
  4. Hydrops fetalis (4/4): stillbirth, Hgb Barts* (gamma tetramers)
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6
Q

How do you dx alpha thalassemia?

A

CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe

Peripheral smear:
Target cells*, teardrop cells, basophilic stippling
Heinz bodies in Hgb H

Hgb electrophoresis

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7
Q

How do you treat mild alpha thalassemia?

A

No tx needed

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8
Q

How do you treat moderate alpha thalassemia?

A

Folate (if retic count is high)
Avoid oxidative stress (s/a sulfa)
Avoid iron supplementation

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9
Q

How do you treat severe alpha thalassemia?

A

Weekly blood transfusions, vit C, folate

Iron chelating agents: IV Deferoxamine, PO deferasirox

Splenectomy (if refractory)

Allogeneic bone marrow transplant = definitive

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10
Q

Who is alpha thalassemia MC in? Who is beta thalassemia MC in?

A

Alpha: SE Asian

Beta: Mediterranean*, Africans

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11
Q

What are the types of beta thalassemia? How many alleles are affected in each type?

A
  1. Trait aka minor (1/2 alleles): asx or mild-mod anemia
  2. Intermedia: anemia, hepatosplenomegaly, bony disease
  3. Major aka Cooley’s anemia (2/2 alleles): become symptomatic at 6 mos, frontal bossing & maxillary overgrowth, hepatosplenomegaly, severe hemolytic anemia, gallstones, iron overload
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12
Q

How do you diagnose beta thalassemia?

A

CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe

Peripheral smear: target cells*, teardrop cells, basophilic stippling, nucleated RBCs

Hgb electrophoresis: HgbF increased, HgbA2 increased, HgbA decreased

Skull bossing on xray (“hair on end”)

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13
Q

How do you treat beta thal trait (minor)?

A

Offer genetic counseling

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14
Q

How do you treat beta thal major?

A

Periodic blood transfusions, vit C, folate
Avoid excess Fe
Iron chelating agents: IV deferoxamine, PO deferasirox
Splenectomy if refractory
Allogenic bone transplant = definitive

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15
Q

What vitamin increases the absorption of Fe absorption?

A

Vit C!

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16
Q

Where is B12 absorbed?

A

Terminal ileum

17
Q

What causes B12 deficiency?

A
  1. Pernicious anemia: autoimmune destruction/loss of gastric parietal cells which secrete intrinsic factor
  2. Vegans
  3. Malabsorption: alcoholism , celiac, Crohn’s
  4. Decreased IF production (acid-reducing drugs, bypass surgery)
18
Q

What are clinical manifestations of B12 deficiency?

A

Neurological sx: paresthesias, gait abnormalities, memory loss, dementia

GI sx

19
Q

How do you diagnose B12 deficiency?

A

Peripheral smear: Increased MCV > 115
Increased homocysteine, increased methlmalonic acid
Pernicious anemia

20
Q

What is acute lymphocytic leukemia?

A

Malignancy of lymphoid stem cells in bone marrow –> lymph nodes, spleen, liver

21
Q

Who is ALL MC in?

A

Childhood, peak at 3-7yo

Down syndrome

22
Q

What are the clinical manifestations of ALL?

A

Pancytopenia sx: fever MC, fatigue, lethargy, bone pain

CNS sx: HA, stiff neck, visual changes, vomiting

23
Q

What does ALL look like on PE?

A

Pallor, fatigue
Petechiae, bruising
Hepatosplenomegaly, lymphadenopathy **

24
Q

How do you diagnose ALL?

A

Bone marrow:

  • hypercellular w/ > 20% blasts
  • WBC 5-100,000
  • anemia, thrombocytopenia
25
Q

How do you treat ALL?

A
Oral chemo (hydroxyurea)
Highly responsive to combo chemo
26
Q

What is chronic lymphocytic leukemia?

A

B cell clonal malignancy

27
Q

Who is CLL MC in?

A

Adults!
> 50 yo
Males
Caucasians

28
Q

What are clinical manifestations of CLL?

A

Most asx
Fatigue MC, dyspnea on exertion
Increased infections
Lymphadenopathy, hepatosplenomegaly

29
Q

How do you diagnose CLL?

A

Smear: well-differentiated lymphocytes w/ scattered “smudge cells”, lymphocytosis > 20,000

Pancytopenia: thrombocytopenia, anemia

30
Q

How do you treat CLL?

A

Obs
Chemo (fludarabine)
Allogeneic stem cell transplant = cure

31
Q

What is chronic myelogenous leukemia?

A

Well differentiated WBCs

Granulocyte proliferation