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Lecture 3&4 Flashcards

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1
Q

What is anemia?

A

Women hemoglobin <12 and men <13

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2
Q

What are some of the ways we distinguish the different anemias?

A

Smears, size of cells

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3
Q

Which anemias are production issues?

A

Anemia of chronic disease

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4
Q

What is the most common type of anemia world wide?

A

Iron deficient

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5
Q

Macrocytic anemia is an MCV greater than what?

A

100

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6
Q

What are the mechanisms of macrocytic anemia?

A

Abnorms in DNA metabolism, shift to immature cells, bone marrow disorder, lipid abnormalities

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7
Q

Megaloblastic macrocytic anemia has an MCV of what?

A

> 115 fl

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8
Q

What are the common causes of megaloblastic macrocytic anemia?

A

Vitamin B12 deficiency, folic acid deficiency

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9
Q

What are common causes of macrocytic anemia?

A

Reticulocytosis, alcoholism, liver disease, hypothyroidism, medication effect, aplastic anemia

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10
Q

90% of alcoholics have an MCV of what?

A

100-110 fl

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11
Q

If alcoholics quit drinking, what will improve?

A

Macrocytic anemia will resolve after 2-4 months

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12
Q

What can be seen in liver cirrhosis that shows macrocytic anemia?

A

Target cells will be seen

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13
Q

Patients with hypothyroidism will have macrocytic anemia, which is more pronounced when?

A

During thyroid crisis

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14
Q

What types of meds can cause macrocytic anemia?

A

Combination antiretroviral therapy for HIV, Hydroxyurea for sickle cell disease

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15
Q

Aplastic anemia

A

The bone marrow decides its done making RBCs

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16
Q

“Aplastic anemia”

A

Pancytopenia: bone marrow stops making everything

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17
Q

What type of age distribution does aplastic anemia have?

A

Biphasic: 10-25 years and >60 years

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18
Q

What causes aplastic anemia?

A

Injury to pluripotent stem cells

Impairs proliferation and differentiation, induces T-cell mediated autoimmune response

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19
Q

What are some acquired causes of aplastic anemia?

A

Chemo and radiation, toxins, viral, drugs, immune disorder, pregnancy

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20
Q

What autoimmune disease can cause aplastic anemia?

A

SLE

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21
Q

What is Fanconi anemia?

A

Defect in DNA repair pathway

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22
Q

What is the clinical presentation of aplastic anemia?

A

Weakness and fatigue, cardiopulmonary compromise, progressive anemia

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23
Q

Cardiopulmonary compromise in aplastic anemia

A

Not delivering enough oxygen, will be tachycardia and have an elevated respiratory rate as a response

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24
Q

Other clinical presentations of aplastic anemia

A

Mucosal bleeding, skin bleeding, petechiae, menorrhea in women, infections

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25
What types of infections are commonly seen with aplastic anemia?
Bacterial, sepsis, pneumonia, UTI, invasive fungal infections: common cause of death
26
PE findings of aplastic anemia
Pallor and petechiae most common findings, purpura
27
What should NOT be seen on PE for aplastic anemia?
Hepatosplenomegaly, lymphadenopathy, bone tenderness
28
What will a CBC show for aplastic anemia?
Pancytopenia, anemia can be severe
29
What will be seen on peripheral smear for aplastic anemia?
RBCs normocytic, can be macrocytic. Decreased or absent polychromatophilic RBCs, cellular elements: reduced in #, abnormal cells NOT present
30
What will the reticulocyte index/count be so aplastic anemia?
Decreased
31
What is the diagnostic criteria for moderate aplastic anemia?
Bone marrow cellularity <30%, absence of severe pancytopenia, depression of atleast 2 of 3 blood elements below normal
32
What is needed for diagnostic criteria of aplastic anemia?
Bone marrow biopsy!
33
What is the diagnostic criteria for severe aplastic anemia?
Bone marrow biopsy showing <25% of normal cellularity OR <59% cellularity with <30% cells are hematopoietic AND 2 of following: Absolute reticulocyte count <40,000 Absolute neutrophil count <500 Platelet count <20,000
34
Very sever aplastic anemia criteria
Criteria for SAA are met, absolute neutrophil count is <200
35
Aplastic anemia essentials for diagnosis
Pancytopenia, no abnormal hematopoietic cells in circulation or on bone marrow, hypocellular bone marrow
36
What is the treatment for aplastic anemia?
Treat underlying cause, management of cytopenias, infection treatment and prevention (if caused by drug, remove the drug)
37
Aplastic anemia therapy is stratified based on what?
Age and disease severity: will be more aggressive with treatment in kids and young adults
38
What is the treatment for mild-moderate aplastic anemia?
Supportive care, EPO growth factors, myeloid growth factors, transfusions, Abx or antifungals for infection
39
What is the TOC for severe aplastic anemia?
Allergenic hematopoietic stem cell transplant (HCT) TOC in children under 20 with HLA matched sibling/donor Preferred in adults 20-50 with HLA matched donor
40
What are other options of treatment for severe aplastic anemia?
Immunosuppressive therapy (IST) for adults >50 with no HLA matched donor and considered in adults 20-50 with severe comorbidities
41
What factors affect the prognosis of AA?
Age, severity of pancytopenia, response to initial therapy
42
SAA survival rates as high as 80-90% depend on what?
Availability of HCT, improved IST and improved supportive care
43
Untreated AA has a 1 year mortality rate of what?
70%
44
Severe aplastic anemia untreated prognosis
Rapidly fatal illness
45
What is the prognosis for AA if you get an allergenic bone marrow transplant?
``` With a sibling donor: <20 YO: 80% survival rate 20-50YO: 65-70% survival rate With HLA matched unrelated donor: Survival rates drop by 10-15% ```
46
Prognosis for AA with equine ATC-cyclosporine immunosuppressive treatment
Response with 70% pts Up to 1/3 relapse 1/4 may develop clinical hematologic abnorms
47
What is the most common cause of congenital aplastic anemia?
Fanconi anemia
48
Fanconi anemia
Automakers recessive, defect in DNA repair pathway, several congenital abnorms, progressive bone marrow failure, increased incidence of malignancies
49
What is the management for Fanconi anemia?
Supportive modalities: | Androgens, hematopoietic growth factors
50
What is the only treatment option that can restore normal hematopoiesis in Fanconi anemia?
Allergenic hematopoietic cell transplant (HCT)
51
What are the megaloblastic anemias?
Vital B12 and folate deficiencies
52
Megaloblastic anemia
MCV >110-115
53
Macrocytic anemias
MCV >100 fl
54
A lack of what leads to abnormal myelin?
Lack of Methionine
55
Lack of methionine and abnormal myelin causes what?
Lack of vitamin B12
56
What is found in megaloblastic anemia?
Macro-ovalocytic RBCs and segmented neutrophils*
57
What will the bone marrow show for megaloblastic anemia?
Erythropoiesis hyperplasia and a megaloblastic morphology
58
What are the most common causes of megaloblastic anemia?
Faulty preparation of foods, folate deficiency in pregnancy
59
There are decreasing rates of megaloblastic anemia, why?
Current folate administration during pregnancy and vitamin supplementation in the elderly
60
Clinical hx and symptoms pointing to vitamin B12 and folate deficiency
Unexplained neurologic si/sxs like dementia, weakness, sensory ataxia, paresthesia High risk population: elderly, alcoholics, pts with malnutrition, bariatric surg pts
61
What lab findings can point to vit B12 and folate Deficiency?
Macro-ovalocytes, MCV >100 with or without anemia, hypersegmented neutrophils, pancytopenia of uncertain cause
62
Hypersegmented neutrophils
>5% of neutrophils with >5 lobes OR | >1% of neutrophils with >6 lobes
63
Vitamin B12 is found in what?
Animal products like meat fish and dairy
64
What is the daily dietary absorption of vitamin B12?
5mcg absorption, liver stores about 2-5mg
65
Daily utilization of vitamin B12
3-5mcg
66
What is the storage requirement for vitamin B12?
3 years
67
What is required for the absorption of B12?
Intrinsic factor
68
What does intrinsic factor do
Proceed by parietal cells in stomach, required for absorption of VitB12
69
Where is the vitamin B12 and IF complex absorption take place?
Terminal ileum
70
Vitamin B12 is a cofactor for two reactions in the body
1. Nuclei acid metabolism | 2. Myelin synthesis
71
Vitamin B12 is critical in what?
DNA synthesis and regulation, specifically DNA synthesis of hematopoietic cells
72
What are some causes of Vitamin B12 deficiency?
Dietary deficiency, inadequate intrinsic factor, pancreatic insufficiency, ileal disease, competition for vitB12 in gut, medications that block absorption, transcobalamin 2 deficiency
73
What is pernicious anemia?
Autoimmune disease where antibodies destroy gastric parietal cells, causing atrophic gastritis and bind/neutralize intrinsic factor
74
Pernicious anemia makes you higher risk for what?
GI/gastric cancers
75
What are some signs and symptoms of vitB12 deficiency?
Symptoms relative to severity of anemia, glossitis, vague GI disturbances, neurologic syndrome
76
What neurologic syndrome will be seen with vitB12 deficiency?
Peripheral nerves affected 1st: distal/peripheral paresthesia, difficulty with balance and proprioception, altered cerebral function
77
PE findings of vitB12 deficiency
Pale, mildly icteric or sallow, atrophic glossitis | Neuro exam: decreased vibration, position sensation and memory disturbances
78
What will a CBC show for vitB12 deficiency?
Severe macrocytic anemia, Hct as low as 10-15%, MCV 110-140, megaloblastic picture, pancytopenia
79
What other lab findings will show vitB12 deficiency?
Low reticulocyte index, serum B12 level low (<170)
80
What will bone marrow biopsy show for vitB12 deficiency?
Megaloblastic morphology, erythroid hyperplasia
81
What is the Schilling test?
Inject with medicinal B12, test the urine and blood levels to see if they can absorb it. Used to test fo pernicious anemia
82
What are some lab findings for pernicious anemia?
Serum gastrin: elevated Pepsinogen: low Ratio or pepsinogen 1 to pepsinogen 2: low
83
What are the essentials of diagnosis for vitB12 deficiency?
Macrocytic anemia, megaloblastic blood smear (macro-ovalocytes and hypersegmented neutrophils) and low serum B12 level
84
What is the parenteral therapy treatment for vitB12 deficiency?
100mcg VitB12 SC or IM daily for 1 week, then weekly for 1mo, then monthly for life
85
What is the oral therapy option for vitB12 deficiency?
Methylcoabalamin 1mg daily: sublingual or oral indefinitely
86
What else can be given for vitB12 deficiency if you have folic acid deficiency too?
Folic Acid 1mg PO daily, treat for 1-4 mos of vitB12 replacement

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