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
Most common childhood malignancy, peaking between ages 3-7 y/o
Acute lymphocytic leukemia (ALL)
26
Children with ____________ have increased rates of ALL
Down syndrome
27
Signs/Symptoms of ALL:
1. Pancytopenia symptoms: fever, fatigue, lethargy. Bone pain 2. CNS symptoms: HA, neck stiffness, visual changes, vomiting
28
Physical exam with ALL:
Pallor, fatigue, petechiae, bruising, hepatosplenomegaly, lymphadenopathy, +/- mediastinal mass
29
Diagnosis of ALL
Bone marrow - hypercellular with > 20% blasts, anemia, thrombocytopenia
30
Management of ALL
``` Oral chemo (>90% remission) Stem cell transplant if relapse ```
31
B cell clonal malignancy
Chronic lymphocytic leukemia (CLL)
32
Most common leukemia in adults overall
chronic lymphocytic leukemia (CLL)
33
Signs/Symptoms of CLL
Most are asymptomatic (find leukocytosis incidentally) | Fatigue, DOE, increased infxns
34
Physical exam with CLL
Painful lymphadenopathy | Hepatosplenomegaly
35
Diagnosis of CLL
1. Peripheral smear - lymphocytes with smudge cells (fragile B cells) 2. Pancytopenia - thrombocytopenia, anemia
36
Management of CLL
Observation if indolent Chemo if symptomatic or progressive Allogeneic stem cell transplant is curative. Poorer prognosis.
37
Most common acute form of leukemia in adults
Acute myelogenous leukemia (AML)
38
Signs/Symptoms of AML
Pancytopenia - anemia, thrombocytopenia, neutropenia Splenomegaly Gingival hyperplasia Bone pain
39
Diagnosis of AML
Leukostasis (WBC > 100,000) | Bone marrow - Auer Rods
40
Auer rods on bone marrow
AML
41
Management of AML
Combo chemotherapy | +/- allogeneic bone marrow transplant after remission
42
Tumor Lysis Syndrome
Seen with AML Occurs after chemo induction Treatment: allopurinol, IV fluids
43
Granulocyte proliferation seen with well differentiated WBCs
Chronic myelogenous leukemia (CML)
44
Signs/symptoms of CML
Most asymptomatic until they develop blastic crisis, splenomegaly
45
Diagnosis of CML
1. Cytogenetics or FISH - philadelphia chromosome | 2. Strikingly increased WBC counts (> 100,000)
46
Philadelphia chromosome
CML | Philadelphia CreaM cheese
47
Management of CML
Chemo | Allogeneic stem cell transplant in severe dz or if failed chemo
48
Pentad of thrombotic thrombocytopenic purpura (TTP)
1. Thrombocytopenia 2. Microangiopathic hemolytic anemia 3. Kidney failure/uremia 4. Neurologic sx - HA, CVA, AMS 5. Fever (rare)
49
Thrombocytopenia sx
``` Petechiae Bruising Purpura Mucocutaneous bleeding involving the skin, oral, GI, GU Epistaxis ```
50
Microangiopathic hemolytic anemia sx
Anemia Jaundice Fragmented RBCs/schistocytes on peripheral smear
51
Primary TTP is due to
autoimmune (idiopathic)
52
Secondary TTP may be due to:
``` Malignancy Marrow transplant SLE Estrogen Pregnancy HIV ```
53
What is decreased with TTP?
ADAMTS13
54
Diagnosis of TTP
1. Labs - thrombocytopenia, normal coags 2. Hemolytic anemia - increased reticulocytes, schistocytes Coombs negative Splenomegaly
55
Management of TTP
1. Plasmapheresis - tx of choice - adds ADAMTS13 | 2. Immunosuppression - corticosteroids, cyclophosphamide
56
Most common inherited bleeding disorder (autosomal dominant)
Von Willebrand Disease
57
Characterized as a defect or deficiency in a factor that helps platelets stick to sites of endothelial injury
Von willebrand disease
58
Signs/Symptoms of von willebrand disease
- bleeding! Gingival bleeding, epistaxis, menorrhagia and skin bruising NSAIDs/Aspirin may precipitate an episode
59
Diagnosis of von willebrand disease
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
First line treatment for von willebrand disease
Desmopressin | Second line: von willebrand factor concentrate
61
Group of inherited (x-linked recessive [male]) bleeding disorders
Hemophilia
62
Factor VIII Deficiency
Hemophilia A
63
Factor IX Deficiency
Hemophilia B or Christmas disease
64
Signs/Symptoms of hemophilia
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
Diagnosis of hemophilia
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
Treatment of hemophilia
Hemophilia A - Desmopressin Hemophilia B - specific clotting factor Factor XI Deficiency - no prophylactic therapy needed
67
Most common cause of inherited thrombophilias
Factor V Leiden
68
Mutant form of coagulation factor that is insensitive to activated protein C (anticoagulant)
Factor V Leiden
69
Screening in asymptomatic pts should only be done if there are multiple ______________ with DVT formation under age of _______
First degree relatives 50 Otherwise, routine screening not recommended
70
Lymphoma that peaks in 20s and then in 50s
Hodgkin's Disease Lymphoma
71
Reed sternberg cells pathognomonic - B cell proliferation with bilobed or multilobular nucleus "owl eye"
Hodgkin's Disease Lymphoma
72
Lymph node involvement with Hodgkin's Disease lymphoma
Upper lymph node involvement | Neck, axilla, shoulder, chest (mediastinum)
73
With Hodgkin's disease lymphoma, sometimes you will see pts with painful lymph nodes with _______________
EtOH ingestion | Although usually pts present with painless lymph nodes
74
Associated symptoms with Hodgkin's Disease Lymphoma
fever weight loss anorexia night sweats
75
Pel Ebstein Fever
Intermittent cyclical fevers x 1-2 weeks | Hodgkin's disease lymphoma
76
40% of hodgkin's disease lymphoma is associated with
Epstein-Barr Virus
77
Hodgkin's disease lymphoma has an __________ 5 year cure rate
Excellent
78
Lymphoma that is most commonly seen after 50 y/o, and has an increased risk with immunosuppression (HIV, viral infxn)
Non Hodgkin's Lymphoma
79
Lymphoma with diffuse large B cell that is more common (more aggressive)
Non Hodgkin's Lymphoma
80
Waldeyer's Ring
Tonsils, base of tongue and nasopharynx that are involved in lymph node spread with Non-Hodgkin's lymphoma
81
Lymph node involvement with Non-Hodgkin's lymphoma
Peripheral multiple node involvement | Axillary, abdominal, pelvic, inguinal, femoral
82
Extranodal spread seen with non-hodgkin's lymphoma
``` GI MC Skin 2nd MC Testes Bone marrow GU Liver, spleen, thyroid, kidney, spine ```
83
Survival rate with non-hodgkin's lymphoma is:
Variable
84
Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines (RBCs, WBCs, platelets)
Polycythemia Vera (Primary Erythrocytosis)
85
Polycythemia Vera is caused by:
JAK2 mutation
86
Increased hematocrit in the absence of hypoxia
Primary erythrocytosis
87
Signs/Symptoms of polycythemia vera
Symptoms due to increased RBC mass Thrombosis or hyperviscosity Headache, dizziness, tinnitus, blurred vision, pruritus, fatigue, thrombosis
88
Pruritus especially after hot bath
Seen with polycythemia vera (primary erythrocytosis)
89
Episodic burning/throbbing of hands and feet with edema
``` Erythromelalgia Polycythemia Vera (Primary erythrocytosis) ```
90
Physical exam with polycythemia vera (primary erythrocytosis)
Splenomegaly Facial plethora (flushed face) Engorged retinal veins
91
Major Criteria for Polycythemia Vera
1. Increased RBC mass, hematocrit and Hgb 2. Bone marrow biopsy showing hypercellularity 3. JAK2 mutation presence
92
Minor Criteria for Polycythemia Vera
Decreased serum erythropoietin levels
93
Criteria for polycythemia vera
All major or first 2 major + minor = polycythemia vera
94
Management of polycythemia vera
Phlebotomy management of choice Done until hematocrit < 45% to reduce high risk of venous thrombosis Low dose aspirin