Heme Flashcards

1
Q

In general what are the 3 most common symptoms of anemia?

A

Fatigue
Headache
Exertional dyspnea

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2
Q

What is hyperkinetic circulation and in what disease is it seen?

A

Large pulse volume & tachycardia

Seen in chronic anemia

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3
Q

A beefy red tongue and koilonychias is usually seen in:

a) acute anemia
b) chronic anemia
c) pronounced anemia
d) nutritional deficiency anemia

A

c) Pronounced anemia

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4
Q

A smooth tongue and other mucosal changes are usually seen in:

a) acute anemia
b) chronic anemia
c) pronounced anemia
d) nutritional deficiency anemia

A

d) nutritional deficiency anemia

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5
Q

What is the most common cause of anemia in the world?

A

Iron deficiency anemia

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6
Q

Pica is the hallmark for what disease?

A

Iron deficiency anemia

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7
Q

What labs need to be done for anemia?

A

1) Hemoglobin
2) Hematocrit
3) RBC indicies (MCV, MCH, MCHC)
4) RBC distribution width
5) Peripheral smear

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8
Q

Describe the following labs for Iron Deficiency anemia:

1) Hemoglobin
2) Hematocrit
3) Peripheral smear
4) Plasma ferritin
5) TIC

A

1) Low
2) Low
3) Hypochomic microcytic RBC, anisocytosis, poikilocytosis
4) <30
5) high

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9
Q

Tx for Iron deficiency anemia

A

Ferrous sulfate PO or IV

take with orange juice to enhance absorption

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10
Q

What is the genetic issue in alpha thalassemia?

A

gene deletion

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11
Q

What is the genetic issue in beta thalassemia?

A

point mutation

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12
Q

Describe the following labs for Thalassemia syndrome:

1) Serum iron
2) Ferritin
3) Peripheral smear

A

1) Normal or high
2) Normal or high
3) Hypochromic Microcytic (<80)

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13
Q

If a infant has a thalassemia disorder that is found at 1 month what type is it?

A

Alpha

Beta–begins at 4-6 months

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14
Q

If growth retardation, severe anemia, osteopneina, pathological fractures are seen at 5 months of age what would someone suspect?

A

Beta Thalassemia (cooley anemia)

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15
Q

What is the treatment for Thalassemias?

A

Mild–no iron
Hemoglobin H–follic acid supplement
Beta–transfusions & Deferoxamine, (allogenic bone marrow transplantation, splenectomy)

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16
Q

Prussian blue staining bone marrow is seen in:

A

Sideroblastic anemia

shows ringed sideroblasts

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17
Q

Sideroblastic anemia is caused by:

A

Myelodysplasia
Chronic alcoholism
Lead Poisoning

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18
Q

What lab is necessary for the evaluation of sideroblastic anemia?

What does a peripheral smear show?

A

Bone marrow evaluation

peripheral smear: normal & hypochromic cells

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19
Q

Tx for Sideroblastic anemia

A

Treat underlying cause

Transfusion

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20
Q

What are the 3 big types of Hypochromic Microcytic anemia? What is the MCV?

A

MCV<80

1) Iron deficiency anemia
2) Thalassemia syndromes (alpha & beta)
3) Sideroblastic anemia

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21
Q

What is the MCV for Normochromic Normocytic anemia?

What are the 2 main causes?

A

MCV= 80-100

1) organ failure (renal, endocrine, thyroid, liver)
2) Impaired marrow function

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22
Q

What is the most common cause for normochormic normocytic anemias?

A

T-cell mediated autoimmune suppressionof hematopoiesis

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23
Q

Pancytopenia is the hallmark for what disease?

A

Aplastic anemia

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24
Q

What is the treatment for the following:

1) anemia of chronic disease
2) anemia of RF, cancer, inflammatory disorders
3) Aplastic anemia

A

1) tx underlying disease
2) Erythropoietin
3) transfusion of RBC & pllatelets
(if severe bone marrow or immunosuppression)

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25
``` What disease would you consider with the following symptoms? Fatigue, tachycardia, anorexia, pallor Brittle nails Cheilosis Smooth tongue Formation of esophageal webs ```
Severe Iron Deficiency Anemia
26
What disease would you consider with the following? Alcoholic with tachycardia, pale, faint works in a lead factory
Sideroblastic anemia
27
What disease would you consider with the following symptoms? weakness, fatigue, vulnerability to infection, pallor, purpura, petichiae, hepatospenomegaly, lymphadenopathy, bone tenderness
Aplastic anemia
28
Macrocytic anemia 1) What is the MCV? 2) Name the 2 types
1) >100 | 2) Folic acid deficiency & vitamin B12 deficiency
29
An alcoholic person with anorexia has glossitis and vague GI symptoms?
Folic acid deficiency anemia
30
Labs for an anemic person shows: - macro-ovalocytes** - pathognomonic hypersecgmented polymorphoneclear cells - Howel Jolly bodies What other labs would you want to order?
Folic acid deficiency anemia order folic acid levels <150
31
Tx for folic acid deficiency anemia
1st: PO follic acid | also avoid alcohol & follic acid metabolism antagonists--MTX
32
Which type of anemia can cause neurologic damage? Some s/s include: stocking-glove paresthesia, clumsiness, dementia, ataxia, loss of position, fine touch and vibratory sensation
Vitamin B12 deficiency
33
What is the most common cause of Vit B12 deficiency?
Pernicious namia | atrophic gastritis-->incr risk of carcinoma
34
Where does Vit B12 absorption occur?
terminal illeum
35
Elevated reticulocyte count is hallmark for:
Hemolytic anemia
36
Labs for an anemic person shows: - Elevated MCV - anisocytosis, poikilocytosis - macro-ovalocytosis - hypersegmented neutrophils - low retic count What other labs would you want to order?
Vitamin B12 deficiency order vit B12--low
37
A person has a virus infection showing jaundice, gallstones, pallor, and symptoms of decreased oxygen. They have an elevated retic count, and elevated unconjugated bilirubin. What do you suspect? How do you treat?
Hemolytic anemia tx: depends on underlying disorder
38
A black male looks really sick after going to Denver to play Football. 1) What do you suspect? 2) what is happening to his vascular system? his organs? 3) Acidosis or Alkalosis? 4) at risk an infection of what type of organism?
1) Sickle cell anemia 2) vascular occlusion with organ swelling 3) Acidosis (hypoxemia) 4) encapsulated organisms
39
Labs shows: - Hemoglobin S in red cells (electrophoresis) - target cells - nucleated RBC - Howell-Jolly bodies - High retic count, WBC, & indirect bilirubin What other labs would you want to order?
Sickle cell anemia order peripheral smear that reveals sickled cells
40
Tx for sickle cell anemia
1) analgesics 2) fluids 3) oxygen 4) pneumococcal vaccine 5) folate supplementation 6) genetic counseling
41
X-linked recessive disorder in black & mediterranean males. Pt usually present as healthy
G6PD deficiency
42
Labs show: - high retic count - high indirect bilirubin - bit cells - Heinz bodies What other labs would you want to order?
G6PD deficiency order G6PD (low)
43
Tx for G6PD deficiency
Episodes are self-limited | Avoid oxidative stress
44
JAK2 mutation is diagnostic for:
Polycythemia vera
45
What are secondary causes of polycethemia vera?
1) chronic hypoxia 2) cigarette smoking 3) renal tumors
46
A 60 yo male smoker presents with the following symptoms: - splenomegally - normal arterial oxygen saturation - burning pain - red extremities - generalized pruritus after bathing 1) What is the condition? 2) What are you concerned of happening to his patient that leads to increased morbidity and mortality?
1) Polycythemia vera | 2) Thrombosis
47
What is the treatment for polycythemia vera?
#1 Phlebotomy Then: - Hydroxyurea: myelosuppresive therapy - Low dose aspirin
48
``` Risk factors for this disorder include: -family hx -ionizing radiation exposure -benzene certain alkylating agents ```
Leukemia
49
What is the most common type of Acute leukemia?
ALL
50
What type of acute leukemia is seen primarily in adulthood?
AML
51
s/s include: - gingival bleeding - epistaxis - menorrhagia - (DIC less common)
Acute leukemia
52
A child with gingival bleeding, epistaxis, and an abrupt onset of fever and joint pain. What would you consider is the issue?
Acute leukemia (ALL)
53
An older adult has slow progressive onset with lethargy, anorexia, dyspnea, gingival bleeding and auer rods. What would you consider is the issue?
Acute leukemia (AML) Mediastinal mass on CXR = ALL auer rods = AML
54
Pancytopenia with circulating blasts is hallmark for:
ALL
55
Mediastinal mass on CXR = | Auer rods =
Mediastinal mass on CXR = ALL | auer rods = AML
56
Tx for Acute leukemia
``` induction chemo consolidation therapy Allopurionl diuretics allogenic bone marrow transplantation ```
57
What is the most prevalent of all leukemias?
CLL
58
Men 60 yo with clonal malignancy of B lymphocytes
CLL
59
Leukemia seen in young to middle aged adults
CML
60
Three phases of CML
1. chronic 2. accelerated 3. acute
61
s/s develop gradually and include: - fatigue - anorexia - weight loss - low grade fever - excessive sweating - abd fullness - splenomegaly - blurred vision - respiratory distress - priapism
CML
62
s/s include: - recurrent infection - splenomegaly - lymphadenopathy
CLL
63
Hallmark: - isolated lymphocytosis - leukocytosis >20,000 cells/mL
CLL
64
Hallmark: - leukocytosis - median WBC count 150,000 cells/mL - Philadelphia chromosome
CML
65
Tx for CML
imatinib mesylate desatinib or milotinib allogenic bone marrow transplant
66
Tx for CLL
palliative once disease is advanced
67
A 20 yo male with painless cervical, supraclavicular and mediastinal lympahdenopathy. Has pain in node after drinking alcohol. On exam has enlarged lymphoid tissue, spleen, and liver. On labs can see Reed Sternberg cells.
Hodgkin's disease
68
Tx for Hodgkin's disease
``` chemo radiation Adriamycin Bleomycin vinblastine dcacrabazine ```
69
A 30 yo with diffuse, unexplained, painless, persistent lympahdenopathy (upper and lower body). What is a useful prognostic marker
Non-hodgkin lymphoma (can also be isolated lymphadenopathy) serum LDH is useful prognosis
70
Tx for Non-Hodgkin lymphoma
``` Radiation Rituximab Allogeneic transplant Chemo autologous stem cell in high grade ```
71
Dx Hodkin's vs Non-Hodgkin lymphoma
biopsy lymph node | Reed Sternberg cell in Hodkin's
72
A 65 yo pt with osteoporosis, lytic lesions, hypercalcemia comes in for the 4th infection in the last 2 months. Pt has anemia, proteinuria, elevated ertythrocyte sedimentation rate. What do they have?
Multiple myeloma
73
Monoclonal spike on serum protein electrophoresis is hallmark for
Multiple myeloma
74
Tx for Multiple Myeloma
Lenalidomide Dexamethasone Doxorubicin
75
Drugs associated with bleeding disorders include:
``` NSAIDS ASA certain antibiotics Anticoagulants Thiazides Gold Heparin ```
76
What are common causes of secondary thrombocytopenic purpura? (2)
SLE | CLL
77
A child comes in with petechia, purpura, and hemorrhagic bullae on the skin and mucous membranes. They have a decreased number of platelets in their blood. What do you suspect?
ITP | in children ITP is usually self limited autoimmune disorder
78
Tx for acute ITP
-resolves spontaneously or - cotocosteroids - splenectomy
79
Chronic ITP
``` high dose prednisone IV immunoglobulin danazol immunosupppressivess stem cell transplant ```
80
In what disease can someone have mild anemia and peripheral smear showing megathrombocytes?
Thrombocytopenia
81
A 30 yo previously healthy female has purpura, petechia, pallor, abd pain, microangiopathic hemolytic anemia, and abnormal neurologc signs. She has severe thrombocytopenia, red cell fragmentation, and metallopreatase enzyme is low. High LDL and indirect bili What does she have?
TTP (thrombocytopenic purpura) *fatal (also associated w/ HIV)
82
Tx for TTP
*fatal* Emergency large volume plasmapheresis Prednisone Antiplatelet agent
83
A 6 yo has an E. coli infection and has CVA tenderness. Labs show severe thrombocytopenia, red cell fragmentation, and metallopreatase enzyme is low. High LDL and inderect bili What does the pt have?
HUS (hemolytic uremia syndrome)
84
TX for HUS
Conservative management | fluids and electrolytes
85
``` A cancer patient presents with bleeding and high fever due to sepsis, and in shock. Labs show: -hypofibrinogenemia -prolonged PT -schistocytes ```
DIC (Disseminated intravascular coagulopathy)
86
Tx for DIC
Propt/aggressive tx for underlying cause Component blood transfusion
87
Autosomal dominant congenital bleeding disorder. | Bleeding time is prolonged
Von Willenbrand disease
88
Tx for Von Willenbrand disease
Desmopressin | Factor 8
89
X-linked recessive disorder affecting factor 8. | PTT is prolonged
Hemophilia A
90
X-linked recessive disorder affecting factor 9 | PTT is prolonged
Hemophilia B
91
Tx for Hemophilia A & B
Desmopressin | + infusion of factor 8 (A), 9 (B)
92
Tx for Factor XI (11)
fresh frozen plasma
93
Most common acquired coagulopathies
Vit K dependent factor deficiencies
94
Which factors are Vit K dependent?
2, 7, 9, 10
95
Tx for vit K dependent factor deficiencies
Tx underlying cause PO or IV vit K fresh frozen plasma Prevenstion: diet w/ high leafy vegetables & tx malabsorption
96
s/s for vit k dependent factor deficiencies
postoperative* not eating well received broad spectrum abx soft tissue bleeding
97
Thrombosis = Effective clotting | Acronym for things that cause thrombosis.
5Ps HHAD CAUSED CLOTTTS ``` Pregnancy/post partum Prothrombin mutation Protein S or protein C def Polycethemia vera Porxysmal nocturnal hemoglobinuria Smoking ``` ``` Heparin therapy Hyperhomocysteinemia Antithrombin III def Dysfibrinogennemia CHF Antiphosphilipid syn Uremia Surgery Estrogen DM CA Leiden factor 5 mutation Obesity Trauma/Travel Thyroid Thalassemia/sickle cell Sepsis ```
98
What is prolonged in thrombotic disorders?
PTT
99
Russell's viper venom time is specific to detect:
Lupus anticoagulant
100
Tx for thrombotic disorders/ hypercoagulable conditions
Low molecular weight heparin | Prednisone--Lupus anticoagulant