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Heme > Lecture 3&4 > Flashcards

Lecture 3&4 Flashcards

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
Q

What types of infections are commonly seen with aplastic anemia?

A

Bacterial, sepsis, pneumonia, UTI, invasive fungal infections: common cause of death

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26
Q

PE findings of aplastic anemia

A

Pallor and petechiae most common findings, purpura

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27
Q

What should NOT be seen on PE for aplastic anemia?

A

Hepatosplenomegaly, lymphadenopathy, bone tenderness

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28
Q

What will a CBC show for aplastic anemia?

A

Pancytopenia, anemia can be severe

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29
Q

What will be seen on peripheral smear for aplastic anemia?

A

RBCs normocytic, can be macrocytic. Decreased or absent polychromatophilic RBCs, cellular elements: reduced in #, abnormal cells NOT present

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30
Q

What will the reticulocyte index/count be so aplastic anemia?

A

Decreased

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31
Q

What is the diagnostic criteria for moderate aplastic anemia?

A

Bone marrow cellularity <30%, absence of severe pancytopenia, depression of atleast 2 of 3 blood elements below normal

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32
Q

What is needed for diagnostic criteria of aplastic anemia?

A

Bone marrow biopsy!

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33
Q

What is the diagnostic criteria for severe aplastic anemia?

A

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

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34
Q

Very sever aplastic anemia criteria

A

Criteria for SAA are met, absolute neutrophil count is <200

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35
Q

Aplastic anemia essentials for diagnosis

A

Pancytopenia, no abnormal hematopoietic cells in circulation or on bone marrow, hypocellular bone marrow

36
Q

What is the treatment for aplastic anemia?

A

Treat underlying cause, management of cytopenias, infection treatment and prevention (if caused by drug, remove the drug)

37
Q

Aplastic anemia therapy is stratified based on what?

A

Age and disease severity: will be more aggressive with treatment in kids and young adults

38
Q

What is the treatment for mild-moderate aplastic anemia?

A

Supportive care, EPO growth factors, myeloid growth factors, transfusions, Abx or antifungals for infection

39
Q

What is the TOC for severe aplastic anemia?

A

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
Q

What are other options of treatment for severe aplastic anemia?

A

Immunosuppressive therapy (IST) for adults >50 with no HLA matched donor and considered in adults 20-50 with severe comorbidities

41
Q

What factors affect the prognosis of AA?

A

Age, severity of pancytopenia, response to initial therapy

42
Q

SAA survival rates as high as 80-90% depend on what?

A

Availability of HCT, improved IST and improved supportive care

43
Q

Untreated AA has a 1 year mortality rate of what?

A

70%

44
Q

Severe aplastic anemia untreated prognosis

A

Rapidly fatal illness

45
Q

What is the prognosis for AA if you get an allergenic bone marrow transplant?

A 
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
Q

Prognosis for AA with equine ATC-cyclosporine immunosuppressive treatment

A

Response with 70% pts
Up to 1/3 relapse
1/4 may develop clinical hematologic abnorms

47
Q

What is the most common cause of congenital aplastic anemia?

A

Fanconi anemia

48
Q

Fanconi anemia

A

Automakers recessive, defect in DNA repair pathway, several congenital abnorms, progressive bone marrow failure, increased incidence of malignancies

49
Q

What is the management for Fanconi anemia?

A

Supportive modalities:

Androgens, hematopoietic growth factors

50
Q

What is the only treatment option that can restore normal hematopoiesis in Fanconi anemia?

A

Allergenic hematopoietic cell transplant (HCT)

51
Q

What are the megaloblastic anemias?

A

Vital B12 and folate deficiencies

52
Q

Megaloblastic anemia

A

MCV >110-115

53
Q

Macrocytic anemias

A

MCV >100 fl

54
Q

A lack of what leads to abnormal myelin?

A

Lack of Methionine

55
Q

Lack of methionine and abnormal myelin causes what?

A

Lack of vitamin B12

56
Q

What is found in megaloblastic anemia?

A

Macro-ovalocytic RBCs and segmented neutrophils*

57
Q

What will the bone marrow show for megaloblastic anemia?

A

Erythropoiesis hyperplasia and a megaloblastic morphology

58
Q

What are the most common causes of megaloblastic anemia?

A

Faulty preparation of foods, folate deficiency in pregnancy

59
Q

There are decreasing rates of megaloblastic anemia, why?

A

Current folate administration during pregnancy and vitamin supplementation in the elderly

60
Q

Clinical hx and symptoms pointing to vitamin B12 and folate deficiency

A

Unexplained neurologic si/sxs like dementia, weakness, sensory ataxia, paresthesia
High risk population: elderly, alcoholics, pts with malnutrition, bariatric surg pts

61
Q

What lab findings can point to vit B12 and folate Deficiency?

A

Macro-ovalocytes, MCV >100 with or without anemia, hypersegmented neutrophils, pancytopenia of uncertain cause

62
Q

Hypersegmented neutrophils

A

> 5% of neutrophils with >5 lobes OR

>1% of neutrophils with >6 lobes

63
Q

Vitamin B12 is found in what?

A

Animal products like meat fish and dairy

64
Q

What is the daily dietary absorption of vitamin B12?

A

5mcg absorption, liver stores about 2-5mg

65
Q

Daily utilization of vitamin B12

A

3-5mcg

66
Q

What is the storage requirement for vitamin B12?

A

3 years

67
Q

What is required for the absorption of B12?

A

Intrinsic factor

68
Q

What does intrinsic factor do

A

Proceed by parietal cells in stomach, required for absorption of VitB12

69
Q

Where is the vitamin B12 and IF complex absorption take place?

A

Terminal ileum

70
Q

Vitamin B12 is a cofactor for two reactions in the body

A
  1. Nuclei acid metabolism

2. Myelin synthesis

71
Q

Vitamin B12 is critical in what?

A

DNA synthesis and regulation, specifically DNA synthesis of hematopoietic cells

72
Q

What are some causes of Vitamin B12 deficiency?

A

Dietary deficiency, inadequate intrinsic factor, pancreatic insufficiency, ileal disease, competition for vitB12 in gut, medications that block absorption, transcobalamin 2 deficiency

73
Q

What is pernicious anemia?

A

Autoimmune disease where antibodies destroy gastric parietal cells, causing atrophic gastritis and bind/neutralize intrinsic factor

74
Q

Pernicious anemia makes you higher risk for what?

A

GI/gastric cancers

75
Q

What are some signs and symptoms of vitB12 deficiency?

A

Symptoms relative to severity of anemia, glossitis, vague GI disturbances, neurologic syndrome

76
Q

What neurologic syndrome will be seen with vitB12 deficiency?

A

Peripheral nerves affected 1st: distal/peripheral paresthesia, difficulty with balance and proprioception, altered cerebral function

77
Q

PE findings of vitB12 deficiency

A

Pale, mildly icteric or sallow, atrophic glossitis

Neuro exam: decreased vibration, position sensation and memory disturbances

78
Q

What will a CBC show for vitB12 deficiency?

A

Severe macrocytic anemia, Hct as low as 10-15%, MCV 110-140, megaloblastic picture, pancytopenia

79
Q

What other lab findings will show vitB12 deficiency?

A

Low reticulocyte index, serum B12 level low (<170)

80
Q

What will bone marrow biopsy show for vitB12 deficiency?

A

Megaloblastic morphology, erythroid hyperplasia

81
Q

What is the Schilling test?

A

Inject with medicinal B12, test the urine and blood levels to see if they can absorb it. Used to test fo pernicious anemia

82
Q

What are some lab findings for pernicious anemia?

A

Serum gastrin: elevated
Pepsinogen: low
Ratio or pepsinogen 1 to pepsinogen 2: low

83
Q

What are the essentials of diagnosis for vitB12 deficiency?

A

Macrocytic anemia, megaloblastic blood smear (macro-ovalocytes and hypersegmented neutrophils) and low serum B12 level

84
Q

What is the parenteral therapy treatment for vitB12 deficiency?

A

100mcg VitB12 SC or IM daily for 1 week, then weekly for 1mo, then monthly for life

85
Q

What is the oral therapy option for vitB12 deficiency?

A

Methylcoabalamin 1mg daily: sublingual or oral indefinitely

86
Q

What else can be given for vitB12 deficiency if you have folic acid deficiency too?

A

Folic Acid 1mg PO daily, treat for 1-4 mos of vitB12 replacement

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