Hematology Flashcards

1
Q

Hct 25 - 30%

A

Dyspnea (worse on exertion ), fatigue

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2
Q

Hct 20 - 25%

A

Lightheadedness, angina

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3
Q

Hct < 20%

A

Syncope, chest pain

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4
Q

Causes of cardiac ischemia

A
  • anemia
  • hypoxia
  • coronary artery disease
  • carbon monoxide poisoning
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5
Q

Causes of microcytosis

A

LITS

  • Lead poisoning
  • Iron deficiency
  • Thalassemia
  • Sideroblastic anemia
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6
Q

Microcytosis

A
  • low MCV

- low reticulocyte count

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7
Q

Causes of macrocytic anemia

A
  • B12 and folate deficiency
  • Sideroblastic anemia
  • Alcoholism
  • Liver disease or hypothyroidism
  • Medications (e.g. zidovudine or phenytoin)
  • Myelodysplastic syndrome
  • Antimetabolite rx: azathioprine, 6-MP, or hydroxyurea
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8
Q

Under normal circumstances, situations that raise reticulocyte count

A
  • Blood loss

- Hemolysis

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9
Q

Normocytic anemia

A
  • acute blood loss

- hemolysis

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10
Q

Treatment of severe anemia

A
  • Packed red blood cells
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11
Q

When do you transfuse a patient:

A
  1. If patient is symptomatic

2. Low hct in an elderly pr or one w/ heart disease.

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12
Q

Symptomatic from anemia

A
  • SOB
  • Lighthead, confused, and sometimes syncope
  • Hypotension and tachycardia
  • Chest pain
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13
Q

Packed Red Blood Cells

A
  • concentrated form of bloos
  • whole blood w/ 150ml plasma removed
  • Hct is 70 - 80%
  • 1 unit of PBRCs raise Hct by about 3 points per unit
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14
Q

Fresh Frozen Plasma

A
  • replaces clotting factors in pts with elevated PTT, aPTT, or INR
  • used as replacement w/ plasmapheresis
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15
Q

Blood products for IgA deficient donor

A

IgA deficient donor FFP

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16
Q

Cryoprecicipate

A
  • used to replace fibrinogen
  • some utility in DIC
  • provides high amts of clotting factors in smaller plasma volume
  • High factor VIII and VWF
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17
Q

Microcytosis

A
  • MCV lower than noral

- usually below 80fL

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18
Q

Iron deficiency

A
  • caused by blood loss
  • Fe needs for 1 - 2mg /day
  • menstruating women need 2 - 3 mg /day
  • pregnant women need 5 - 6 mg/day
  • Fe absorbed in duodenum
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19
Q

Chronic disease

A
  • caused by cancer or chronic infxn
  • Fe is locked in storage or trapped in macrophages or in ferritin
  • hemoglobin synthesis can’t move forward
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20
Q

Anemia in renal failure

A

Deficiency of erythropoiestin

- MCV is initially normal then decreases

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21
Q

Sideroblastic anemia

A
  • can be macrocytic as well associated w/ myelodysplasia, preleukemic syndomre
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22
Q

Common causes of sideroblastic anemia

A
  • Alcohol suppressive effect on marrow (MCC)
  • Lead poisoning
  • Isoniazid
  • Vit B6 deficiency
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23
Q

Thalassemia

A
  • extremely common cause of microcytosis

- most patients are assymptomatic

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24
Q

Pt with anemia and c/o blood loss (GI Bleeding). Likely dx?

A

Iron deficiency

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25
Q

Mensturating woman c/o anemia. Likely dx?

A

Iron deficiency

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26
Q

Pt with cancer or chronic infxn is anemic. Likely dx?

A

Anemia of Chronic dix

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27
Q

Anemic Pt with rheumatoid arthritis. Likely dx?

A

Anemia of chronic disease

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28
Q

Alcoholic pt has anemia. Likely dx?

A

Sideroblastic anemia

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29
Q

Asymptomatic pt with anemia. Likely dx?

A

Thalassemia

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30
Q

Most common way to diagnosis microcytic anemia.

A

Peripheral smea

- target cells are most common w/ thalassemia

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31
Q

Pt has low ferritin. Likely dx?

A

Iron deficiency

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32
Q

Pt has high iron. Likely dx?

A

Sideroblastic anemia

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33
Q

Pt has normal iron studies. Likely dx?

A

Thalassemia

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34
Q

Iron deficiency

A
  • low ferritin is extremely specific for Fe deficiency

- increased TIBC b/c of lots of unbound sites on receptors

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35
Q

Chronic disease

A
  • serum is low in circulation b/c Fe is trapped in storage
  • Stored Fe (ferritin) elevated or normal
  • Circulating Fe is decreased
  • Low TIBC
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36
Q

Sideroblastic anemia

A
  • only microcytic anemia with elevated Fe level
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37
Q

Thalassemia

A
  • genetic disease w/ normal Fe
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38
Q

Unique lab features of Fe deficiency

A
  • increased RDW b/c new cells are more FE deficient and smaller
  • as body runs out of Fe, newer cells have less hemoglobin
  • elevated platelet count
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39
Q

Unique lab fx of sideroblastic anemia

A

Prussian blue staining for ringed sideroblastic anemia is msot accutate test
- basophilic stippling can occur in any cause of sideroblastic anemia

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40
Q

Unique lab features: thalassemia

A

Hemoglobin electrophoresis is most accurate diagnostic test

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41
Q

Alpha thalassemia

A

3 genes deleted shows moderate anemia with hemoglobin H, whihc are beta-4 tetrads
- increased reticulocytes

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42
Q

Hemoglobin Bart

A
  • four genes deleted
  • gamma 4 tetrads
    = CHF causes death in utero
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43
Q

Beta thalassemia

A
  • increased hemoglobin F and A2
44
Q

Beta thalassemia intermedia

A
  • normal hemoglobin F

- no transfusion dependence

45
Q

Iron deficiency tx

A
  • replace Fe with oral ferrous sulfate

- occasionally pts w/ IM Fe

46
Q

Tx of anemia of chronic disease

A
  • correct underlying disease

- onlt anemia associated w/ ESRD responds to erythropoietin

47
Q

Tx of sideroblastic anemia

A

Correct underlying cause

- respond to Vitamin B6 or pyroxidine replacement

48
Q

Tx of thalassemia

A
  • Trait can’t be treated
  • Beta thalassemia major managed w/ lifelong chronic transfusion
  • Iron overload is managed by deferasirox or deferipone
49
Q

Megaloblastic anemia

A
  • presence of hypersegmented neutrophils

- B12 and folate deficiency and antimetabolite medications cause hypersegmeneted neutrophils

50
Q

Vitamin B12 deficiency: Causes

A
  • Pernicious anemia
  • PANCREATIC INSUFFICIENCY
  • Dietary deficiency
  • Crohn dx or any disease damaging terminal ileum
  • Blind loop syndrome (gastrectomy or gastric bypass)
  • Diphyllobothrium or HIV
51
Q

Folate deficiency: causes

A
  • Dietary dfeficiency (goat’s milk has no folate and provides limited Fe)
  • Psoriasis and skin loss or turnover
  • Drugs: phenytoin and sulfa
52
Q

Celiac disease causes what nutritional deficiencies?

A

Vitamin B12
Folate
Iron deficiency

53
Q

Clinical findings which differentiate folate and Vit B12 deficiencies

A

B12 have neurological abnormalities

  • peripheral neuropathy is most common
  • dementia is least common
  • posterior column damage to vir
54
Q

Causes of neurological findings in Vit B12 deficiency

A

Posterior column damage to position and vibratory sensation or “subacute combined degeneration
of cord
- look for ataxia

55
Q

Dx tests associated with folate and Vit B12 deficiency

A
  • megaloblastic anemia
  • increased LDH and increased indirect bilirubin leveles
  • decreased reticulocyte count
  • hypercellular bone marrow
  • macroovalocytes
  • increased homocysteine levels
56
Q

Lab test differences btwn folate and vitamin B12 deficiencies

A

Both folate and Vit B12 deficiencies have increased homocysteine
ONLY B12 have increased methylmanonic acid (MMA)

57
Q

Confirmation of parietal anemia

A
  • cause of macrocytic anemia

Confirmed with anti-instrinsic factor and anti-parietal cell antibodies

58
Q

Why are reticulocyte counts so low in setting of macrocytic anemia

A

Red cells are destroyed as they leave the marrow, so the reticulocyte is low

59
Q

Why does pancreatic insufficiency important for B12 deficiency

A

Pancreatic enzymes needed for absorption of Vitamin B12

60
Q

What is a complication of B12 and folate replacement?

A

Hypokalemia

- Reintroduction of B12 and folate causes cells to be produced so rapidly so that marrow packages serum K quickly

61
Q

Obstructive Sleep Aonea

A
  • recurrent transient obstruction of upper airwy dur to pharyngeal collapse during sleep
  • usually patients are overweight
62
Q

Obstructive Sleep Apnea: Sx

A
  • excessive daytime sleepiness
  • snoring
  • morning headaches
  • impotence
  • arterial hypertension
63
Q

Discuss OSA and erythropoeitin

A
  • OSA causes short term hypoxemia which is sensed by kidneys and stimulaes increased erythropoetin production
  • increased epo can cause polycythemia
64
Q

TTP

A
  • patients with hemolytic anemia and thrombocytpenia
  • altered mental status, renal failure and fever are common
  • defect in ADAMTS13
65
Q

Best test for TTP

A

** peripheral blood smear ( can see

66
Q

TTP: Hx and PE

A
  • low platelet count
  • microangiopathic hemolytic anemia
  • neuro changes (delirium, seizure, stroke)
  • impaired renal fxn (incr. BUN:Cr)
  • fever
    • be suspicious if 3/5 are present
67
Q

3 causes of microangiopathic hemolytic anemia

A
  • HUS
  • TTP
  • DIC
68
Q

Management of severe hypercalcemia (Ca > 14)

A

Short term

  • normal salne hydraton plus calctionin
  • avoid loop diuretics unless volume overload

Long term
- Bisphosophates (zoledronic acid)

69
Q

Management of asymptomatic or mild hypercalcemia (ca < 12)

A
  • No immediate treatment required

- avoid thiazide diuretic and lithium use

70
Q

B-thalassemia

A
  • usually of Med descent
  • asymptomatic w/ mild anemia
  • disproportionately high RBC count
  • low MCV
  • target cells on smear
71
Q

B-thalaseemia on electrophoresis

A
  • elevated hemoglobin A2 on electrophoresis
72
Q

Tx of B-thalaseemia

A
  • Reassurance w/ no specific therapy required
73
Q

A-thalassemis

A
  • pts missing 2/4 alpha globin chains
  • asymptomatic w/ mild anemia
  • MCV < 75
  • target cells on peripheral semar
  • normal hemoglobin electrophoresis
74
Q

Heparin Induced Thrombocytopenia

A
  • heparin induced released of PF4 from platelets form heparin-PF4 complexes
  • IgG antibodies against heparin-PF4 complexes causes platelet activation, endothelial cell activation
  • increased PTT due to thrombin consumpton
75
Q

Pt has thrombocytopenia and hypercoagulation w/in days of initiating anticoagulation therapy. Likely dx?

A

HIT due to unfractionated heparin or low weight molecular heparin

76
Q

Hairy cell leukemia

A
  • B-lymphocyte derived chronic leukemia
  • lymphocytes have fine, hair-like irregular projections
  • bone marrow may become fibrotic thus may lead to dry tap
  • TRAP (tartrate resistant acid phosphotase) stain
77
Q

Tx of hairy cell leukemia

A
  • Cladribine (purine analog)

can be toxic to bone marrow and adverse effect include neurological and kidney damage

78
Q

Hereditary spherocytosis

A
  • usally autosomal dominant
  • triad: hemolytic anemia, jaundice, and splenomegaly
  • increased risk for bilrubin gallstones & infxn from parvovirus B19
79
Q

Hereditary spherocytosis: Lab findings

A
  • reticulocytye count from 5- 20%
  • normal to low MCV
  • increased MCHC indicating membrane loss & dehyrdration
  • spherocytes on peripheral smear
  • Negative Coombs test
80
Q

Heme manifestations of SLE

A

Pancytopenia

  • Anemia: due to chronic dx, renal insuffuciency, and AHA
  • Leukopenia: autoimmune destruction
    thrombocytopenia: immune mediated
81
Q

Acute hemolytic transfusion reaction

A

Rxn from transfusion of mismatched blood (ABO)

  • starts WITHIN HOUR of transfusion
  • pts: have fever, chills, hemoglobinuria, FLANK PAIN and discomfort and site
82
Q

Dx of acute hemolytic transfusion rxn

A
  • positive direct Coombs test
  • pink plasma (plasma free hemoglobin > 25)
  • Hemoglobinuria
  • Repeat type and cross-match shows missmatch
83
Q

Management of acute hemolytic transfusion reaction

A
  • Immediate cessation of transfusion while mantainig IV access and supportive care
84
Q

Delayed hemolytic transfusion

A
  • from anamanestic antibody response to a red blood antigen to which pt was previously sensitized
  • causes low-grade hemolysis WITHIN 2-10 days
85
Q

IgA deficiency

A

rapid onset of shock, angioedema/urticaria & respiratory distress

  • occurs WITHIN SECONDS-MINUTES of tranfusion
  • caused by recipient of anti-A IgG antibodies
86
Q

Mgmt of anaphylaxis due to IgA deficiency

A
  • Epinepherine with circulatory and respiratory support
87
Q

Giant cell tumor of bone

A
  • benign and locally aggressive skeletal tumor that presents w/ pain, swelling, and decreased range of motion
  • “Soap-bubble” appearance on X-rays
  • usually in epiphyseal regions of long bones and involves distal femur and proximal tibia around knee joint
88
Q

Osgood Schlatter disease

A
  • overuse injury caused by repetitive strain
  • seen in young children and teens who have undergone rapid growth spurt
  • X-ray shows avulsion apophysis of tibial tubercle
89
Q

Esophageal cancer

A
  • squamous cell most common worldwide

- adenocarcinoma most popular in US, Europe and Australa

90
Q

Risk factors for esophageal squamous cell carcinoma

A

Alcohol and tobacc

91
Q

Risk factors for adenocarcinoma

A
  • Barrett’s esophagus (columnar metaplasia distal esophagus)
92
Q

Hx/PE: for esophageal cancer

A
  • progressive dysphagia initially to solid then liquids

- weight loss, odynophagia, GERD, GI bleeding and vomiting

93
Q

Dx of esophageal cancer

A

Barrium swallow followed by EGD w/ biopsy

94
Q

Tx of esophageal cancer

A

Chemoradiation and surgical resection is first line treatment
- has poor prognosis

95
Q

Location of SCC of esophagus

A

in upper and middle 1/3 of esophagus

96
Q

Location of adenocarcinoma of esophagus

A

in lower 3rd

97
Q

Lynch Syndrome / HNPCC

A
  1. > 3 relatives w/ colorectal (1 must be 1st deg relative)
  2. involvement in > 2 generations
  3. at least one case diagnosed before 50
  4. Exclusion of FAP
98
Q

Lynch Syndrome I vs Lynch Syndrome II

A

Lynch Syndrome I - hereditary site specific colon cancer

Lynch Syndrome II - incr risk for extracoloni

99
Q

Vitamin B 12

A

-Macrocytic, megaloblastic anemia)
Neuro sx (optic neuropathy, subacute combined degenearation, parasthesias)
- Glossitis

100
Q

Leading cause of B12 deficiency

A

Pernicious anemia

  • antibodies to IF needed for b12 absorption
  • chronic gastritis for decr. production of IF
101
Q

Long term complications of pernicious anemia

A

Gatric cancer

102
Q

ITP

A

IgG are formed against patient’s platelets

- most common immunologic disorder in women of childbearing age

103
Q

Hx/PE: ITP

A

pts generally feel well but present w/

- easy bruising, petechiae, hematuria, hematemesis or melena

104
Q

ITP associated w/ which conditions

A
  • Lymphoma
  • Leukemia
  • SLE
  • HIV
  • HCV
105
Q

Acute ITP

A

abrupt onset of hemorrhagic complications following viral ilness
- usually in children 2-6 y.o

106
Q

Chronic ITP

A

insiduous onset unrelated to infext

  • often affects adults 20 - 40
  • females 3x more likely affected than males
107
Q

Dx of ITP

A
  • DIAGNOSIS OF EXCLUSION

- can be confirmed w/ hx and pe, CBC, and peripheral blood smear