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Hematology Flashcards

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
Thrombocytopenia sx
``` Petechiae Bruising Purpura Mucocutaneous bleeding involving the skin, oral, GI, GU Epistaxis ```
26
Microangiopathic hemolytic anemia sx:
Anemia Jaundice Fragmented RBCs/schistocytes on peripheral smear
27
Primary TTP is due to
Autoimmune (idiopathic)
28
Secondary TTP may be due to
``` Malignancy Marrow transplant SLE Estrogen Pregnancy HIV ```
29
What is decreased with TTP?
ADAMTS13
30
Diagnosis of TTP
1. Labs - thrombocytopenia, normal coags 2. Hemolytic anemia - increased reticulocytes, schistocytes Coombs negative Splenomegaly
31
Management of TTP
1. Plasmapheresis - tx of choice - adds ADAMTS13 | 2. Immunosuppression - corticosteroids, cyclophosphamide
32
Malignancy of lymphoid stem cells in bone marrow, migrating to the lymph nodes, spleen, liver and other organs
Acute Lymphocytic Leukemia (ALL)
33
Most common childhood malignancy, peaking between ages 3-7 y/o
Acute lymphocytic leukemia (ALL)
34
Children with ___________ have increased rates of ALL
Down's syndrome
35
signs/symptoms of ALL
1. Pancytopenia symptoms: fever, fatigue, lethargy, bone pain 2. CNS symptoms: HA, neck stiffness, visual changes, vomiting
36
Physical exam with ALL:
Pallor, fatigue, petechiae, bruising, hepatosplenomegaly, lymphadenopathy, +/- mediastinal mass
37
Diagnosis of ALL
Bone marrow - hypercellular with > 20% blasts, anemia, thrombocytopenia
38
Management of ALL
``` Oral chemo (>90% remission) Stem cell transplant if relapse ```
39
Most common acute form of leukemia in adults
Acute myelogenous leukemia (AML)
40
Signs/symptoms of AML
Pancytopenia - anemia, thrombocytopenia, neutropenia Splenomegaly Gingival hyperplasia Bone pain
41
Diagnosis of AML
Leukostasis (WBC > 100,000) | Bone marrow - Auer rods
42
Auer rods on bone marrow
AML
43
Management of AML
Combo chemotherapy | +/- allogeneic bone marrow transplant after remission
44
Tumor Lysis Syndrome
Seen with AML Occurs after chemo induction Treatment: allopurinol, IV fluids
45
Anemia is defined as ____________ or ____________
Low hemoglobin | Low hematocrit
46
Symptoms of anemia are a result of ______________
Decreased oxygen delivery to tissues
47
Core symptoms of all types of anemia
Fatigue Dyspnea on exertion Palpitations If severe: syncope and chest pain
48
Types of macrocytic anemia (MCV > 100)
``` B12 deficiency Folate deficiency Alcoholism Hypothyroidism Liver disease ```
49
Types of microcytic anemia (MCV < 80)
Iron deficiency Thalassemia Anemia of chronic dz Sideroblastic anemia
50
Types of normocytic anemia (MCV 80-100)
Blood loss Hemolysis Anemia of chronic dz
51
How does a pt with vitamin B12 deficiency present?
Neurological symptoms | Peripheral neuropathy most common
52
Lab values for vitamin B12 deficiency
Low B12 level High MMA May also see: elevated iron, indirect bilirubin, and LDH
53
Vitamin B12 deficiency can be secondary to medication, such as
Metformin PPIs Cimetidine
54
Treatment for vitamin B12 deficiency
Vitamin B12 replacement | IM preferred if severe neurological problems
55
Treatment for folate deficiency
Folic acid
56
A patient with iron deficiency will present with
Blood loss | GI or menses
57
Lab values for iron deficiency
``` Low serum iron Low ferritin High iron binding capacity High RDW Elevated platelet count ```
58
Treatment for iron deficiency
Always look for cause | Ferrous sulfate
59
Complications of ferrous sulfate
Can turn stools black but will have negative guaiac stools
60
Thalassemia is characterized by hemoglobin having reduced ____________
Alpha or beta chains
61
Thalassemia will have ___________ or ___________ on peripheral smear
Target cells | Tear-drop shaped cells
62
Beta thalassemia is diagnosed with _____________
Electrophoresis
63
Treatment for beta thalassemia
Transfusions | Iron chelation
64
Lab values for anemia of chronic disease
Low serum iron Low iron binding capacity Normal ferritin
65
Sideroblastic anemia may be due to (4):
1. alcohol 2. INH (isoniazid) 3. copper deficiency 4. zinc toxicity
66
Most common causes of hemolysis normocytic anemia
Sickle cell disease
67
Ultimate complications of anemia
Arrhythmias MI CHF
68
Lymphoma that peaks in 20s and then in 50s
Hodgkin's Disease Lymphoma
69
Reed sternberg cells pathognomonic - B cell proliferation with bilobed or multilobular nucleus "owl eye"
Hodgkin's Disease Lymphoma
70
Lymph node involvement with Hodgkin's Disease lymphoma
Upper lymph node involvement | Neck, axilla, shoulder, chest (mediastinum)
71
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
72
Associated symptoms with Hodgkin's Disease Lymphoma
Fever Weight Loss Anorexia Night sweats
73
Pel Ebstein Fever
Intermittent cyclical fevers x 1-2 weeks | Hodgkin's disease lymphoma
74
40% of hodgkin's disease lymphoma is associated with
Epstein-Barr Virus
75
Hodgkin's disease lymphoma has an _______ 5 year cure rate
Excellent
76
Lymphoma that is most commonly seen after 50 y/o and has an increased risk with immunosuppression (HIV, viral infxn)
Non hodgkin's lymphoma
77
Lymphoma with diffuse large B cell that is more common (more aggressive)
Non-Hodgkin's Lymphoma
78
Waldeyer's Ring
Tonsils, base of tongue and nasopharynx that are involved in lymph node spread with Non-Hodgkins' lymphoma
79
Lymph node involvement with Non-Hodgkin's lymphoma
Peripheral multiple node involvement | Axillary, abdominal, pelvic, inguinal, femoral
80
Extranodal spread seen with non-hodgkin's lymphoma
``` GI MC Skin 2nd MC Testes Bone marrow GU Liver, spleen, thyroid, kidney spine ```
81
Survival rate with non-hodgkin's lymphoma
Variable
82
Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines (RBCs, WBCs, platelets)
Polycythemia Vera (primary erythrocytosis)
83
Polycythemia vera is caused by:
JAK2 mutation
84
Increased hematocrit in the absence of hypoxia
Primary erythrocytosis
85
Signs/symptoms of polycythemia vera
Symptoms due to increased RBC mass Thrombosis or hyperviscosity HA, dizziness, tinnitus, blurred vision, pruritus, fatigue, thrombosis
86
Pruritus especially after hot bath
Seen with polycythemia vera (primary erythrocytosis)
87
Episodic burning/throbbing of hands and feet with edema
``` Erythromelalgia Polycythemia Vera (primary erythrocytosis) ```
88
Physical exam with polycythemia vera (primary erythrocytosis)
Splenomegaly Facial plethora (flushed face) Engorged retinal veins
89
Major criteria for polycythemia vera
1. Increased RBC mass, hematocrit and Hgb 2. Bone marrow biopsy showing hypercellularity 3. JAK2 mutation presence
90
Minor criteria for polycythemia vera
Decreased serum erythropoietin levels
91
Criteria for polycythemia vera
All major or first 2 major + minor = polycythemia vera
92
Management of polycythemia vera
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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