Hematology Flashcards

1
Q

Anemia is defined as __________ or ___________

A

Low hemoglobin

Low hematocrit

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2
Q

Symptoms of anemia are a result of ____________

A

Decreased oxygen delivery to tissues

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3
Q

Core symptoms of all types of anemia

A
Fatigue
Dyspnea on exertion
Palpitations
If severe:
Syncope and chest pain
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4
Q

Types of macrocytic anemia (MCV > 100)

A
B12 Deficiency
Folate Deficiency
Alcoholism
Hypothyroidism
Liver disease
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5
Q

Types of microcytic anemia (MCV < 80)

A

Iron deficiency
Thalassemia
Anemia of chronic dz
Sideroblastic Anemia

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6
Q

Types of normocytic anemia (MCV 80-100)

A

Blood loss
Hemolysis
Anemia of chronic dz

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7
Q

How does a pt with vitamin B12 deficiency present?

A

Neurological symptoms

Peripheral neuropathy most common

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8
Q

Lab values for vitamin B12 deficiency

A

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

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9
Q

Vitamin B12 deficiency can be secondary to medication, such as:

A

Metformin
PPIs
Cimetidine

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10
Q

Treatment for vitamin B12 deficiency

A

Vitamin B12 replacement

IM preferred if severe neurological problems

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11
Q

Treatment for folate deficiency

A

Folic acid

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12
Q

A patient with iron deficiency will present with:

A

Blood loss

GI or menses

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13
Q

Lab values for iron deficiency

A
Low serum iron
Low ferritin
High iron binding capacity
High RDW
Elevated platelet count
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14
Q

Treatment for iron deficiency

A

Always look for a cause!

Ferrous sulfate

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15
Q

Complications of ferrous sulfate

A

Can turn stools black but will have negative guaiac stools

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16
Q

Thalassemia is characterized by hemoglobin having reduced __________

A

Alpha or beta chains

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17
Q

Thalassemia will have ___________ or ______________ on peripheral smeare

A

Target cells

Tear-drop shaped cells

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18
Q

Beta thalassemia is diagnosed with _____________

A

Electrophoresis

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19
Q

Treatment for beta thalassemia

A

Transfusions

Iron chelation

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20
Q

Lab values for anemia of chronic disease

A

Low serum iron, low iron binding capacity, normal ferritin

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21
Q

Sideroblastic anemia may be due to (4):

A
  1. alcohol
  2. INH (isoniazid)
  3. copper deficiency
  4. zinc toxicity
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22
Q

Most common cause of hemolysis normocytic anemia

A

sickle cell disease

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23
Q

Ultimate complications of anemia

A

Arrhythmias
MI
CHF

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24
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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25
Q

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

A

Acute lymphocytic leukemia (ALL)

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26
Q

Children with ____________ have increased rates of ALL

A

Down syndrome

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27
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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28
Q

Physical exam with ALL:

A

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

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29
Q

Diagnosis of ALL

A

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

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30
Q

Management of ALL

A
Oral chemo (>90% remission)
Stem cell transplant if relapse
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31
Q

B cell clonal malignancy

A

Chronic lymphocytic leukemia (CLL)

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32
Q

Most common leukemia in adults overall

A

chronic lymphocytic leukemia (CLL)

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33
Q

Signs/Symptoms of CLL

A

Most are asymptomatic (find leukocytosis incidentally)

Fatigue, DOE, increased infxns

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34
Q

Physical exam with CLL

A

Painful lymphadenopathy

Hepatosplenomegaly

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35
Q

Diagnosis of CLL

A
  1. Peripheral smear - lymphocytes with smudge cells (fragile B cells)
  2. Pancytopenia - thrombocytopenia, anemia
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36
Q

Management of CLL

A

Observation if indolent
Chemo if symptomatic or progressive
Allogeneic stem cell transplant is curative. Poorer prognosis.

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37
Q

Most common acute form of leukemia in adults

A

Acute myelogenous leukemia (AML)

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38
Q

Signs/Symptoms of AML

A

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

39
Q

Diagnosis of AML

A

Leukostasis (WBC > 100,000)

Bone marrow - Auer Rods

40
Q

Auer rods on bone marrow

A

AML

41
Q

Management of AML

A

Combo chemotherapy

+/- allogeneic bone marrow transplant after remission

42
Q

Tumor Lysis Syndrome

A

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

43
Q

Granulocyte proliferation seen with well differentiated WBCs

A

Chronic myelogenous leukemia (CML)

44
Q

Signs/symptoms of CML

A

Most asymptomatic until they develop blastic crisis, splenomegaly

45
Q

Diagnosis of CML

A
  1. Cytogenetics or FISH - philadelphia chromosome

2. Strikingly increased WBC counts (> 100,000)

46
Q

Philadelphia chromosome

A

CML

Philadelphia CreaM cheese

47
Q

Management of CML

A

Chemo

Allogeneic stem cell transplant in severe dz or if failed chemo

48
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)
49
Q

Thrombocytopenia sx

A
Petechiae
Bruising
Purpura
Mucocutaneous bleeding involving the skin, oral, GI, GU
Epistaxis
50
Q

Microangiopathic hemolytic anemia sx

A

Anemia
Jaundice
Fragmented RBCs/schistocytes on peripheral smear

51
Q

Primary TTP is due to

A

autoimmune (idiopathic)

52
Q

Secondary TTP may be due to:

A
Malignancy
Marrow transplant
SLE
Estrogen
Pregnancy
HIV
53
Q

What is decreased with TTP?

A

ADAMTS13

54
Q

Diagnosis of TTP

A
  1. Labs - thrombocytopenia, normal coags
  2. Hemolytic anemia - increased reticulocytes, schistocytes
    Coombs negative
    Splenomegaly
55
Q

Management of TTP

A
  1. Plasmapheresis - tx of choice - adds ADAMTS13

2. Immunosuppression - corticosteroids, cyclophosphamide

56
Q

Most common inherited bleeding disorder (autosomal dominant)

A

Von Willebrand Disease

57
Q

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

A

Von willebrand disease

58
Q

Signs/Symptoms of von willebrand disease

A
  • bleeding!
    Gingival bleeding, epistaxis, menorrhagia and skin bruising
    NSAIDs/Aspirin may precipitate an episode
59
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

60
Q

First line treatment for von willebrand disease

A

Desmopressin

Second line: von willebrand factor concentrate

61
Q

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

A

Hemophilia

62
Q

Factor VIII Deficiency

A

Hemophilia A

63
Q

Factor IX Deficiency

A

Hemophilia B or Christmas disease

64
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

65
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

66
Q

Treatment of hemophilia

A

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

67
Q

Most common cause of inherited thrombophilias

A

Factor V Leiden

68
Q

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

A

Factor V Leiden

69
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

70
Q

Lymphoma that peaks in 20s and then in 50s

A

Hodgkin’s Disease Lymphoma

71
Q

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

A

Hodgkin’s Disease Lymphoma

72
Q

Lymph node involvement with Hodgkin’s Disease lymphoma

A

Upper lymph node involvement

Neck, axilla, shoulder, chest (mediastinum)

73
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

74
Q

Associated symptoms with Hodgkin’s Disease Lymphoma

A

fever
weight loss
anorexia
night sweats

75
Q

Pel Ebstein Fever

A

Intermittent cyclical fevers x 1-2 weeks

Hodgkin’s disease lymphoma

76
Q

40% of hodgkin’s disease lymphoma is associated with

A

Epstein-Barr Virus

77
Q

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

A

Excellent

78
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

79
Q

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

A

Non Hodgkin’s Lymphoma

80
Q

Waldeyer’s Ring

A

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

81
Q

Lymph node involvement with Non-Hodgkin’s lymphoma

A

Peripheral multiple node involvement

Axillary, abdominal, pelvic, inguinal, femoral

82
Q

Extranodal spread seen with non-hodgkin’s lymphoma

A
GI MC
Skin 2nd MC
Testes
Bone marrow
GU
Liver, spleen, thyroid, kidney, spine
83
Q

Survival rate with non-hodgkin’s lymphoma is:

A

Variable

84
Q

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

A

Polycythemia Vera (Primary Erythrocytosis)

85
Q

Polycythemia Vera is caused by:

A

JAK2 mutation

86
Q

Increased hematocrit in the absence of hypoxia

A

Primary erythrocytosis

87
Q

Signs/Symptoms of polycythemia vera

A

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

88
Q

Pruritus especially after hot bath

A

Seen with polycythemia vera (primary erythrocytosis)

89
Q

Episodic burning/throbbing of hands and feet with edema

A
Erythromelalgia
Polycythemia Vera (Primary erythrocytosis)
90
Q

Physical exam with polycythemia vera (primary erythrocytosis)

A

Splenomegaly
Facial plethora (flushed face)
Engorged retinal veins

91
Q

Major Criteria for Polycythemia Vera

A
  1. Increased RBC mass, hematocrit and Hgb
  2. Bone marrow biopsy showing hypercellularity
  3. JAK2 mutation presence
92
Q

Minor Criteria for Polycythemia Vera

A

Decreased serum erythropoietin levels

93
Q

Criteria for polycythemia vera

A

All major or first 2 major + minor = polycythemia vera

94
Q

Management of polycythemia vera

A

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