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Emergency EOR > Hematology > Flashcards

Hematology Flashcards

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1
Q

DDX for easy bruising

A

Cushing’s syndrome
von Willebrand disease
Hemophilia

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2
Q

Aplastic anemia crisis occurs in sickle cell disease or G6PD deficiency if infected with:

A

Parvovirus B19

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3
Q

Most common presentation of sickle cell disease at 6-9months

A

Dactylitis (sausage fingers)

Delayed growth and development

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4
Q

Skeletal H shaped vertebrae

A

Avascular necrosis of bones - sickle cell disease

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5
Q

Skin ulcers, especially on tibia

A

Sickle cell disease

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6
Q

Pain occlusive crises in sickle cell disease can be triggered by:

A 
Cold weather
Hypoxia
Infection
Dehydration
EtOH
Pregnancy
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7
Q

Diagnosis of sickle cell disease

A
  1. CBC with peripheral smear - target cells, sickled erythrocytes, Howell-Jolly bodies
  2. Low hemoglobin, low hematocrit
  3. Hemoglobin Electrophoresis - looking for HgbS
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8
Q

Howell-Jolly bodies on peripheral smear

A

Sickle cell disease

Indicates functional asplenia

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9
Q

Management of sickle cell disease

A
  1. IV hydration and oxygenation
  2. Pain control - avoid meperidine
  3. Hydroxyurea
  4. Folic acid
  5. Children should be immunized
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10
Q

Most common inherited bleeding disorder (autosomal dominant)

A

von Willebrand disease

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11
Q

Characterized as a defect or deficiency in a factor that helps platelets stick to sites of endothelial injury

A

von Willebrand disease

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12
Q

Signs/symptoms of von willebrand disease

A

bleeding
Gingival bleeding, epistaxis, menorrhagia and skin bruising
NSAIDs/aspirin may precipitate an episode

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13
Q

Diagnosis of von willebrand disease

A 
Normal platelet count, normal PT
Von willebrand factor antigen- decreased
Von willebrand factor activity - decreased
Factor VIII activity - decreased
If all abnormal, perform RIPA
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14
Q

First line treatment for von willebrand disease

A

Desmopressin

Second line: von willebrand factor concentrate

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15
Q

Group of inherited (x-linked recessive [male]) bleeding disorders

A

Hemophilia

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16
Q

Factor VIII Deficiency

A

Hemophilia A

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17
Q

Factor IX deficiency

A

Hemophilia B or christmas disease

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18
Q

Signs/symptoms of hemophilia

A

Pt who is bleeding into joints, muscles, or GI tract

First episode can occur anywhere from first few months of life up until age 4

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19
Q

Diagnosis of hemophilia

A

Normal platelet count, normal PT, prolonged aPTT
When pts plasma combined with normal plasma, aPTT will correct itself
confirmation done by ordering specific assay

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20
Q

Treatment of hemophilia

A

Hemophilia A - Desmopressin
Hemophilia B - specific clotting factor
Factor XI deficiency - no prophylactic therapy needed

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21
Q

Most common cause of inherited thrombophilias

A

Factor V Leiden

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22
Q

Mutant form of coagulation factor that is insensitive to activated protein C (anticoagulant)

A

Factor V Leiden

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23
Q

Screening in asymptomatic pts should only be done if there are multiple ______________with DVT formation under age of _______

A

First degree relatives
50
Otherwise, routine screening not recommended

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24
Q

Pentad of thrombotic thrombocytopenic purpura (TTP)

A
  1. Thrombocytopenia
  2. Microangiopathic hemolytic anemia
  3. Kidney failure/uremia
  4. Neurologic sx - HA, CVA, AMS
  5. Fever (rare)
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25
Q

Thrombocytopenia sx

A 
Petechiae
Bruising
Purpura
Mucocutaneous bleeding involving the skin, oral, GI, GU
Epistaxis
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26
Q

Microangiopathic hemolytic anemia sx:

A

Anemia
Jaundice
Fragmented RBCs/schistocytes on peripheral smear

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27
Q

Primary TTP is due to

A

Autoimmune (idiopathic)

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28
Q

Secondary TTP may be due to

A 
Malignancy
Marrow transplant
SLE
Estrogen
Pregnancy
HIV
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29
Q

What is decreased with TTP?

A

ADAMTS13

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30
Q

Diagnosis of TTP

A
  1. Labs - thrombocytopenia, normal coags
  2. Hemolytic anemia - increased reticulocytes, schistocytes
    Coombs negative
    Splenomegaly
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31
Q

Management of TTP

A
  1. Plasmapheresis - tx of choice - adds ADAMTS13

2. Immunosuppression - corticosteroids, cyclophosphamide

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32
Q

Malignancy of lymphoid stem cells in bone marrow, migrating to the lymph nodes, spleen, liver and other organs

A

Acute Lymphocytic Leukemia (ALL)

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33
Q

Most common childhood malignancy, peaking between ages 3-7 y/o

A

Acute lymphocytic leukemia (ALL)

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34
Q

Children with ___________ have increased rates of ALL

A

Down’s syndrome

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35
Q

signs/symptoms of ALL

A
  1. Pancytopenia symptoms: fever, fatigue, lethargy, bone pain
  2. CNS symptoms: HA, neck stiffness, visual changes, vomiting
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36
Q

Physical exam with ALL:

A

Pallor, fatigue, petechiae, bruising, hepatosplenomegaly, lymphadenopathy, +/- mediastinal mass

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37
Q

Diagnosis of ALL

A

Bone marrow - hypercellular with > 20% blasts, anemia, thrombocytopenia

38
Q

Management of ALL

A 
Oral chemo (>90% remission)
Stem cell transplant if relapse
39
Q

Most common acute form of leukemia in adults

A

Acute myelogenous leukemia (AML)

40
Q

Signs/symptoms of AML

A

Pancytopenia - anemia, thrombocytopenia, neutropenia
Splenomegaly
Gingival hyperplasia
Bone pain

41
Q

Diagnosis of AML

A

Leukostasis (WBC > 100,000)

Bone marrow - Auer rods

42
Q

Auer rods on bone marrow

A

AML

43
Q

Management of AML

A

Combo chemotherapy

+/- allogeneic bone marrow transplant after remission

44
Q

Tumor Lysis Syndrome

A

Seen with AML
Occurs after chemo induction
Treatment: allopurinol, IV fluids

45
Q

Anemia is defined as ____________ or ____________

A

Low hemoglobin

Low hematocrit

46
Q

Symptoms of anemia are a result of ______________

A

Decreased oxygen delivery to tissues

47
Q

Core symptoms of all types of anemia

A

Fatigue
Dyspnea on exertion
Palpitations
If severe: syncope and chest pain

48
Q

Types of macrocytic anemia (MCV > 100)

A 
B12 deficiency
Folate deficiency
Alcoholism
Hypothyroidism
Liver disease
49
Q

Types of microcytic anemia (MCV < 80)

A

Iron deficiency
Thalassemia
Anemia of chronic dz
Sideroblastic anemia

50
Q

Types of normocytic anemia (MCV 80-100)

A

Blood loss
Hemolysis
Anemia of chronic dz

51
Q

How does a pt with vitamin B12 deficiency present?

A

Neurological symptoms

Peripheral neuropathy most common

52
Q

Lab values for vitamin B12 deficiency

A

Low B12 level
High MMA
May also see: elevated iron, indirect bilirubin, and LDH

53
Q

Vitamin B12 deficiency can be secondary to medication, such as

A

Metformin
PPIs
Cimetidine

54
Q

Treatment for vitamin B12 deficiency

A

Vitamin B12 replacement

IM preferred if severe neurological problems

55
Q

Treatment for folate deficiency

A

Folic acid

56
Q

A patient with iron deficiency will present with

A

Blood loss

GI or menses

57
Q

Lab values for iron deficiency

A 
Low serum iron
Low ferritin
High iron binding capacity
High RDW
Elevated platelet count
58
Q

Treatment for iron deficiency

A

Always look for cause

Ferrous sulfate

59
Q

Complications of ferrous sulfate

A

Can turn stools black but will have negative guaiac stools

60
Q

Thalassemia is characterized by hemoglobin having reduced ____________

A

Alpha or beta chains

61
Q

Thalassemia will have ___________ or ___________ on peripheral smear

A

Target cells

Tear-drop shaped cells

62
Q

Beta thalassemia is diagnosed with _____________

A

Electrophoresis

63
Q

Treatment for beta thalassemia

A

Transfusions

Iron chelation

64
Q

Lab values for anemia of chronic disease

A

Low serum iron
Low iron binding capacity
Normal ferritin

65
Q

Sideroblastic anemia may be due to (4):

A
  1. alcohol
  2. INH (isoniazid)
  3. copper deficiency
  4. zinc toxicity
66
Q

Most common causes of hemolysis normocytic anemia

A

Sickle cell disease

67
Q

Ultimate complications of anemia

A

Arrhythmias
MI
CHF

68
Q

Lymphoma that peaks in 20s and then in 50s

A

Hodgkin’s Disease Lymphoma

69
Q

Reed sternberg cells pathognomonic - B cell proliferation with bilobed or multilobular nucleus “owl eye”

A

Hodgkin’s Disease Lymphoma

70
Q

Lymph node involvement with Hodgkin’s Disease lymphoma

A

Upper lymph node involvement

Neck, axilla, shoulder, chest (mediastinum)

71
Q

With Hodgkin’s disease lymphoma, sometimes you will see pts with painful lymph nodes with _____________

A

EtOH ingestion

Although usually pts present with painless lymph nodes

72
Q

Associated symptoms with Hodgkin’s Disease Lymphoma

A

Fever
Weight Loss
Anorexia
Night sweats

73
Q

Pel Ebstein Fever

A

Intermittent cyclical fevers x 1-2 weeks

Hodgkin’s disease lymphoma

74
Q

40% of hodgkin’s disease lymphoma is associated with

A

Epstein-Barr Virus

75
Q

Hodgkin’s disease lymphoma has an _______ 5 year cure rate

A

Excellent

76
Q

Lymphoma that is most commonly seen after 50 y/o and has an increased risk with immunosuppression (HIV, viral infxn)

A

Non hodgkin’s lymphoma

77
Q

Lymphoma with diffuse large B cell that is more common (more aggressive)

A

Non-Hodgkin’s Lymphoma

78
Q

Waldeyer’s Ring

A

Tonsils, base of tongue and nasopharynx that are involved in lymph node spread with Non-Hodgkins’ lymphoma

79
Q

Lymph node involvement with Non-Hodgkin’s lymphoma

A

Peripheral multiple node involvement

Axillary, abdominal, pelvic, inguinal, femoral

80
Q

Extranodal spread seen with non-hodgkin’s lymphoma

A 
GI MC
Skin 2nd MC
Testes
Bone marrow
GU
Liver, spleen, thyroid, kidney spine
81
Q

Survival rate with non-hodgkin’s lymphoma

A

Variable

82
Q

Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines (RBCs, WBCs, platelets)

A

Polycythemia Vera (primary erythrocytosis)

83
Q

Polycythemia vera is caused by:

A

JAK2 mutation

84
Q

Increased hematocrit in the absence of hypoxia

A

Primary erythrocytosis

85
Q

Signs/symptoms of polycythemia vera

A

Symptoms due to increased RBC mass
Thrombosis or hyperviscosity
HA, dizziness, tinnitus, blurred vision, pruritus, fatigue, thrombosis

86
Q

Pruritus especially after hot bath

A

Seen with polycythemia vera (primary erythrocytosis)

87
Q

Episodic burning/throbbing of hands and feet with edema

A 
Erythromelalgia
Polycythemia Vera (primary erythrocytosis)
88
Q

Physical exam with polycythemia vera (primary erythrocytosis)

A

Splenomegaly
Facial plethora (flushed face)
Engorged retinal veins

89
Q

Major criteria for polycythemia vera

A
  1. Increased RBC mass, hematocrit and Hgb
  2. Bone marrow biopsy showing hypercellularity
  3. JAK2 mutation presence
90
Q

Minor criteria for polycythemia vera

A

Decreased serum erythropoietin levels

91
Q

Criteria for polycythemia vera

A

All major or first 2 major + minor = polycythemia vera

92
Q

Management of polycythemia vera

A

Phlebotomy management of choice
Done until hematocrit < 45% to reduce high risk of venous thrombosis
Low dose aspirin

Emergency EOR (12 decks)

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