Anemias Flashcards

(100 cards)

1
Q

The who defines anemia for females as

A

Hct<4,000,000

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2
Q

The who defines anemia for woman as

A

Hct<4,000,000

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3
Q

Macrophages supply what?

A

supply Fe++ for Hgb production

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4
Q

what is the function of fibroblasts in maintaining homeostatic balance

A

support bone marrow integrity

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5
Q

what is the function of adipocytes in maintaining homeostasis?

A

store energy as fat

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6
Q

What is the function of osteoblasts and osteoclasts in maintaining homeostasis

A

support the boney trabeculae

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7
Q

what regulates erythropoiesis?

A

erythropoietin

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8
Q

where is erythropoietin secreted from

A

the kidney

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9
Q

what is the function of erythropoietin

A

governs day to day RBC production

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10
Q

what is the main stimulus for EPO

A

O2 availability

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11
Q

How should EPO levels change in relation to an anemia

A

they should increase in proportion to the severity of the anemia

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12
Q

what substrates are needed for RBC production

A

Iron,B12,Folate

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13
Q

what is the normal lifespan of an RBC

A

120days

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14
Q

what does hypoxia do to RBC’s

A

Increases RBC’s

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15
Q

Which types of people have relatively lower RBS mass?

A

African americans and older adults

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16
Q

there is an increased mortality with Hgb of what?

A

Hgb of <12g/dL

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17
Q

What would the plasma and erythrocytes look like in an athlete?

A

increase in both plasma and erythrocytes

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18
Q

what are examples of volume and anemia concept

A

Acute bleeding, When normal volume is restored, dehydration and pregnancy

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19
Q

what would a reticulocyte with an index<2.5 be classified as?

A

hypoproliferative

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20
Q

what would a reticulocyte index >2.5 be classified as?

A

hyperproliferative

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21
Q

what is a reticulocyte?

A

an immature RBS that is stained with methylene blue and reveals residual Ribosomal RNA as beads of tiny deep blue precipitates

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22
Q

what happens to the retic count with blood loss?

A

the marrow should be able to increase production by 3 to 4 times normal over a 10day period

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23
Q

Anemia symptoms

A

DOE, Fatigue, Palpitations, H/A, Tinnitus, Chest pain, Esophageal rings, Nail spooning, Picas, Neuropathies

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24
Q

Clinical signs of Anemia

A

Tachycardia, Orthostatic hypotension, Systolic ejection murmur, Pallor

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25
GI signs of anemia
Jaundice, Positive stool guaiac, splenomeagly, hepatomegaly, Petechiae, glossitis, Koilonychia, neurologic, musculo-skeletal
26
what is the definition of microcytic and hypochromic anemia
MCV<80mm3
27
what are causes of microcytic anemia (TICS)
Thalassemia major/minor Iron deficiency Chronic dz/Inflammatory dz Sideroblastic anemia and Pb poisoning
28
Iron deficiency anemia accounts for what % of anemias
50% of anemias worldwide
29
serum irons is measured how?
Iron bound to transferrin in the serum | norm 40-140uG/dL
30
total iron binding capacity
total transferrin in the serum | norm 200-400uG/dL
31
Fe/TIBC
is the % saturation of iron binding capacity | norm should be 25-50%
32
Serum ferritin
is a rough estimate of iron stored in the reticuloendothelial system norm. 30-250ng/ml
33
what are causes of Iron deficiency anemia
``` GI bleeding Excessive Menstruation Malnutrition and Diet Insufficiency Malabsorption Increased demand (pregnancy&growth spurts) Blood donation,blood loss in dialysis ```
34
what are specific clinical manifestations of Iron Deficiency Anemia
Angular Cheilosis PICA Koilonychia Plummer-Vinson Syndrome
35
How do you treat Iron Deficiency Anemia
6wks to correct anemia 6 months to replete bone marrow stores Ferrous Sulfate 325mg 3x/day, avoid milk/dairy products/calcium simultaneously -Can be given IV or IM
36
When should you give IV or IM iron treatment
``` intolerant to oral preparation If more rapid correction of Fe deficiency anemia is neded Patients can not absorb oral Iron Chronic blood loss Pt on Dialysis ```
37
what are causes for treatment failure
Unidentified blood loss non-sdherence Incorrect diagnosis GI malabsorption
38
What would you want to educate your patient on with Fe++ side effects
Constipation, black stools, nausea, bloating, abdominal pain and diarrhea -do not take with tetracyclines/fluoroquinolones and antacids/PPI's and be careful with calcium supplements Vitamin C
39
Where is alpha-thalassemia most prevalent?
Increase in malaria regions: Southeast Asia & China
40
where is beta-thalassemia most prevalent?
in mediterranean populations | Southern Italy, Greece and North Africa
41
What is the definition of Thalassemia
genetic d/o characterized by inadequate production of either alpha or beta globin chain of hemoglobin. There is an ineffective erythropoiesis and increase hemolysis
42
what happens when there is a defective alpha or beta globin chain production?
an imbalance of globin chains The unaffected chain takes over-accumulates in the RBC interfering with normal maturation of the RBC Leads to free radical production that induces hemolysis and anemia
43
what are heinz bodies
are precipitants of excess alpha chains in beta-thalassemia are precipitants of excess beta chains in alpha thalassemia -they impair DNA synthesis and decrease RBC production -they damage RBC membrane and increase RBC destruction
44
what are the clinical features of thalassemia
positive family history history of life-long hypochromic microcytic anemia not responsive to Fe++ From silent carrier statues to profound anemia -depends on genetic defects
45
Beta-thalassemia minor contains how many gene mutations?
one gene mutation | results in mild anemia
46
beta-thalassemia major contains how many gene mutations?
two gene mutations results in severe transfusion dependent anemia-Iron overload If transfusions is delayed growth retardation ensues in children
47
What are complications of beta-thalassemia major (cooley's anemia)
``` growth retardation Severe anemia abnormal facial structure pathologic fractures and osteopenia hepatosplenomegaly jaundice and bilirubin gall stones high out put CHF short life span <30yrs ```
48
what are the laboratory findings for thalassemia
``` Hg electrophoresis (confirms diagnosis) Inc Increase in fetal hemoglobin decrease in Hgb beta thal but increase in Hgb A2 HgbH in severe alpha thalassemia ```
49
what will thalassemia look like in a peripheral smear
small, pale RBC target cells nucleated erythroblasts basophilic stippling
50
other laboratory findings in thalassemia
``` high serum iron and ferritin Hgb level 3-6g/dl Marked microcytosis Heinz bodies in beta-thalassemia increase retic count with hemolysis ```
51
what is the treatment for thalassemia?
transfusion + deferoxamine (to prevent iron overload) Splenectomy Experiment allogeneic hemtaopoietic stem cell transplantation in severe cases avoid iron containing supplements
52
Transfusion complications
``` Iron overload -heart failure -liver failure pancreatic failure w/DM & malabsorption -Other endocrine abnormalities -skin pigmentation Tx iron overload with chelation ```
53
Thalassemia patient education
genetic counseling testing of parents prenatal diagnosis for severe forms of thalassemia
54
what is the definition of normochromic normocytic anemia
MCV 80-100
55
what are the causes of a normochromic normocytic anemia
``` anemia of chronic disease Early Fe deficiency Myelodysplasia or marrow failure Acute blood loss Anemia associated with renal failure ```
56
what is the pathophysiology of chronic/inflammatory disease
``` impaire iron utilization and metabolism -retic prod is low norm or incr. ferritin norm Fe/TIBC low transferrin and low serum iron Iron remain sequestered in RE system Controlled by hepcidin which is released by liver and controls Fe absorption ```
57
what is the most important hormone regulating the Fe release from cells
Hepcidin. | As hepcidin levels increase there is a decrease in iron availability.
58
what decreases the response to erythropoietin
IL-1, IL-6 & TNF
59
What is the treatment for anemia of chronic disease
treatment of the underlying condition is the most important Fe++ supplement may or may not help There is a decrease response to EPO
60
Anemia due to chronic renal failure is common and severe due to
EPO deficiency Uremic toxins interfere with RBC production and survival Hemodialysis Responds well to EPO and Iron
61
What are intrinsic causes of hemolytic anemia (RBC defects)
G6PD deficiency Sickle cel Hereditary spherocytosis
62
what are the extrinsic causes?
``` immune hemolytic anemia Drug induced hemolytic anemia TTP hemolytic uremic syndrome DIC mechanical hemolysis d/t cardiac valve dysfunction hyperslpenism ```
63
what are general clinical features with hemolytic anemias
may be associated with jaundice | may have symptoms consistent with tissue hypoxia, similar to other anemia related syndrome: pallor, SOB, Tachycardia
64
what is sickle cell anemia
point mutation of the beta-globin chain of Hb-A molecule- with replacement of glutamine with valine-Hbs short arm of chromosome 11 mutation
65
what is the pathophys of sickle cell anemia
HbS become sickled when doxygenated- producing structural cellular damage sickle cell HgB can return to its normal configuration when reoxygentated
66
what is sickle cells disease defined as in terms of genetics
homozygous hemoglobin S
67
what is sickle cell trait defined as
heterozygous for Hgb A and HgbS | little tendency to sickle unless there is severe hypoxia usually asymptomatic
68
sickle cell demographics
affects .1-.2% african american about 10% of african american carry sickle cell positive fam. hx
69
symptoms of sickle cell anemia
due to deformed RBC's causing hemolysis, splenic sequestration and microvascular occlusion
70
what happens during painful crisis with sickle cell anemia?
``` bone infarctions osteomyelitis part w/salmonella pneumococcal and other infx due to autoslpenectomy acute chest syndrome- pulmonary infarcts, hypoxia, fever slpenic sequestration crisis arthropathy renal papillary necrosis priapism-impotence retinal hemorrhage ```
71
what are complications of sickle cell?
infections d/t splenic infarction causes overwhelming infection by encapsulated organmism (strep, hemo,kleb,sal) parvo B19 causing hemolysis aplastic crisis due to viral infx. transient arrest in Erythropoiesis with marked decreased in retic
72
how is sickle cell diagnosed
``` Hg electrophoresis -will detect HbS Smear -sickled cells, hemolysis, reduced RBC count -reticulocytes -nucleated RBC -Target Cells -Howell jolly bodies d/t autosplenectomy ```
73
what is the treatment for sickle cell anemia
prevent sickle cell crisis by monitoring condition that demand O2 from RBC's -infection -exercise -climate extremes -dehydration during periods of physiologic/emotional distress When patient with Hgb AS or SS presents with paint and distress this is a sickle cell crisis until proven otherwise -Consider BM transplant -hydroxyurea given PO chronically, this increases Hbf which decreases Hbs and painful crisis and acute chest syndrome WBC and Retics which may worsen symptoms
74
how to treat sickle cell crisis
IV hydration, Supplemental O2, Analgesics-morphine, transfusion for Hct<30% R/O occult infx, MI or PE
75
what are supportive cares treatments for sickle cell
folic acid pneumococcal and H flu vac. Penicillin prophylaxis Opthalmological monitoring against retinopathy Orthopedic care careful monitoring of renal,pulm and cardiac function paint management
76
what is the definition of autoimmune hemolytic anemias
acquired antibody mediated toward RBC membrane antigens, mostly non-specific but occasionally against Rh Types IgM or IgG
77
what is warn AIHA
IgG mediated more common than cold AIHA IgG binds to RBC at >37C results in extravascular hemolysis- especially in spleen and liver
78
what are the causes of warm AIHA
primary or Idopathic | Secondary to lymphoid malignancies, collagen vascular dz, viral infx or drugs
79
cold AIHA
IgM mediated IgM binds to RBC in temp <37C produces complement fixation and activation leading to intravascular hemolysis within blood vessels
80
what causes cold AIHA
Idiopathic lymphoproliferative dz mycoplasma pneumonia Infectious mono EBV
81
what are clinical signs of autoimmune hemolytic anemia
jaundice splenomegaly fatigue pallor
82
what are the lab findings like for AIHA
``` sine qua non: positive direct coombs test elevated retic count blood smear shows immature RBC free serum hemoglobin and hemoglobinuria high unconjugated bilirubin elevated LDH low or absent haptoglobin ```
83
Tx of AIHA
``` treat underlying disease -Lymphoma, Mycoplasma pneumonia, EBV Immunosuppressive meds RBC transfusions Folate splenectomy-to reduce hemolysis and destruction/sequestration ```
84
What is a macrocytic anemia
MCV>100
85
what is the cause of a B12 macrocytic anemia
most common cause if of B12 is impaired GI absorption due to lack of intrinsic factor produced by gastric parietal cells
86
what is a pernicious anemia
is a lack of intrinsic factor due to auto-antibody destruction of gastric parietal cells
87
what are causes of B12deficiency
``` Bariatric Surgery Gastrectomy Poor diet-alcoholic Vegetarians Terminal ileal resection Crohns celiac competing organism -tape worm, GI or fungal growth ```
88
what is the clinical presentation of B12 deficiency?
can take >3yrs to show up Macrocytic hypoproliferative anemia Weakness, faintness, pallor of skin and mucous membranes Dyspnea after slight exertion Atrophic glossitis and stomatitis Neuropathy-distinguishes between B12 and Folate Important causes of dementia
89
what are the lab findings in B12 deficiency
blood smear shows marked macrocytosis >100mm low serum B12<250pg/ml high methylmalonic acid (high only in B12) high homocysteine (elevated in both B12 and folate) macro ovalocytes and hypersegmented PMN low retic count elevated LDH and Indirect bilirubin
90
what are the co-factors for DNA synthesis
B12 and folate
91
what happens with there is a deficiency in erythropoiesis
Ineffective RBC production defective and fragile RBC Asynchronous maturation between cytoplasm
92
What is the importance of B12 in terms of neurons
B12 maintains neuronal myelin integrity | deficiency predisposes to myelin breakdown with resulting neurological symptoms=peripheral neuropathy
93
what is the treatment for B12 deficiency anemia
1mg B12 (cyanocobalamin) IM/PO x7days then q wkx 4-8 wks (IM for pernicious anemia) If tx is delayed >6mths neurological prob may not respond to tx Folate can reverse hematologic abnormalities of B12 but not neurologic symptoms IT IS ESSENTIAL TO GET B12 & FOLATE in MACROCYTIC ANEMIA
94
what is the pathophys for folate deficiency
body stores sufficient 2-3 months | decrease folic acid causes impaired RBC maturation and early destruction
95
what are the causes for folate deficiency
``` inadequate folate intake alcoholics anorectic persons elderly diet low in meat, fruit, and vegetables(leafy green veggies) ```
96
what are reasons for increased requirement of folate
``` pregnancy chronic hemolytic anemias malignancy dialysis infants and children defective absorption Celiac sprue ```
97
meds that can cause folate deficiency
methotrexate trimethoprim anticonvulsants
98
what are clinical presentations of folate deficiency
symptoms from dietary deficiency can be seen in a few months-quicker than B12 atrophic glossitis and angular chelosis anemia symptoms
99
what are the lab findings for folate deficiency
macro-ovalocytes hypersegmented PMNs B12 levels are normal low levels of serum folate
100
what is the Tx for folate
1-5mg/day folic acid PO avoid alcohol and folic acid antagonist treat malabsorption rule out B12 deficiency