Anemia Cases - Hertz - SRS Flashcards

1
Q

Interpret this CBC.

What study/ies would you like to order?

A

Moderate hypochromic microcytic anemia

Order Iron studies

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2
Q

You order iron studies on your patient and they come back with low transferrin, low serum iron and high TIBC.

Based on this, and the attached image, what do you see happening in the bone marrow sample shown?

A

Iron deficiency anemia

Bone marrow shows increased increased iron in macrophages.

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3
Q

What is the most common cause of anemia in hospitalized patients in the US?

A

Impaired red cell production associated with chronic diseases that produce systemic inflammation.

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4
Q

What is this and what probably caused it?

What type of anemia could this patient have?

A

Osteomyelitis

DM

Anemia of chronic disease

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5
Q

What caused this?

What anemia is this patient at risk for?

A

Rheumatoid arthritis

Anemia of chronic disease

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6
Q

What is this most likely?

Anemia patient is at risk for?

A

Squamous or small cell carcinoma

Anemia of chronic disease (suprise)

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7
Q

In anemia of chronic disease, what would the following levels be?

  1. Serum ferritin
  2. Serum iron
  3. Transferrin saturation (%)
  4. Hemoglobin
A
  1. Serum ferritin - normal or increased
  2. Serum iron - decreased
  3. Transferrin saturation (%) - Normal or decreased
  4. Hemoglobin - decreased
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8
Q

In iron deficiency anemia, what are the levels of the following?

  1. Serum ferritin
  2. Serum iron
  3. Transferrin saturation (%)
  4. Hemoglobin
A
  1. Serum ferritin - Decreased
  2. Serum iron - decreased
  3. Transferrin saturation (%) - decreased
  4. Hemoglobin - decreased
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9
Q

What is different in the levels of the following between anemia of chronic disease and Iron deficiency anemia?

  1. Serum ferritin
  2. Serum iron
  3. Transferrin saturation (%)
  4. Hemoglobin
A

In anemia of chronic disease the serum ferritin will be normal or increased where-as in iron deficiency anemia this is decreased.

He made a big deal out of this point. Learn it, know it, love it.

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10
Q

Several effects of inflammation contribute to the observed iron abnormalities in anemia of chronic disease. Particularly what mediatior stimulates an increase in hepatic production of hepcidin.

A

IL-6

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11
Q

In anemia of chronic disease, it can be said that the erythroid precursors are starved for iron in the midst of plenty. Explain this.

A

The IL-6 induced overproduction of hepcidin reduces transfer of iron from the storage pool to developing erythroid precursors in the bone marrow.

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12
Q

Apart from the iron deprivation, what else causes the progenitors to not proliferate adequately?

A

Inflammatory cytokines antagonize the production of EPO, leading to EPO levels that are abnormally low for the degree of anemia.

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13
Q

The RBCs in anemia of chronic disease can be normocytic normochromic, or hypochromic and microcytic as in that of iron deficiency.

How do you distinguish between anemia of iron deficiency and that of chronic disease?

A

High serum ferritin legel in anemia of chronic disease (acute phase reactant with inflammation)

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14
Q

What is the only way to correct the anemia of chronic disease?

A

Treat the underlying disease

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15
Q

What is this this cell from a patient with hereditary cytosis infected with?

What does this cause?

A

Parvo virus - a complication HS patients are at risk for.

Leads to aplastic crises

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16
Q

What does aplastic anemia refer to?

A

chronic primary hematopoietic failure and attendant pancytopenia.

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17
Q

What do most cases of “known” etiology aplastic anemia follow?

A

Exposure to chemicals and drugs

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18
Q

In some cases, aplastic anemia arises in an unpredictable idiosyncratic fashion following exposure to drugs that normally do not cause marrow suppression. What are two examples of implicated drugs?

A

Chloramphenicol and gold salts

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19
Q

What is the cause of 65% of aplastic anemias?

A

Idiopathic

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20
Q

Two major etiologies of aplastic anemia have been invoked, one extrinsic and one intrinsic. What are examples of each?

A

Extrisic - Immune-mediated

Intrinsic - abnormality of stem cells

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21
Q

In aplastic anemia stem cells may first be antigenically altered by exposure to drugs, infectious agents or other unidentified environmental insults. This provokes a cellular immune response during which activated TH1 cells produce cytokines such as Interferon-y and TNF. How does this lead to aplastic anemia?

A

IFN-Y and TNF suppress and kill hematopoietic progenitors.

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22
Q

Aplastic anemia can transform into what two neoplasms?

A

Myeloid leukemia

Myelodysplasia

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23
Q

What does this bone marrow biopsy reveal?

A

Aplastic anemia - markedly hypocellular marrow with primarily fat cells present.

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24
Q

Aplastic anemias can occur at any age and in either sex, the onset usually being insidious. Initial manifestations vary somewhat, depending on which cell line is predominantly affected. Regardless, what ultimately appears in time?

A

Pancytopenia

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25
Q

It is important to distinguish aplastic anemia from other causes of pancytopenia such as “aleukemic” leukemia and myelodysplastic syndromes.

What does the diagnosis of aplastic anemia rest upon?

A

Examination of bone marrow biopsy

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26
Q

What is the treatment of choice for aplastic anemia?

A

Bone marrow transplantation

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27
Q

What is the prognosis for a patient with aplastic anemia?

A

Variable, with treatment 5 year survival exceeds 75%.

Older patients and those without suitable donors often respond well to immunosuppressive therapy.

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28
Q

Identify the disease states shown in the attached images.

A

Left: aplastic anemia

Right: leukemia

29
Q

On physical exam, of a patient with aplastic anemia what will you feel when palpating the spleen?

A

No enlargement - splenomegally is characteristically absent.

30
Q

Attached are a couple of characteristic clinical findings of aplastic anemia. Describe what you see.

What stain is used in the image at right?

A

Left: hypochromic red cells

Right: reticulocytes (supravital stain)

31
Q

What is the “rule” in aplastic anemia?

A

Reticulocytopenia

32
Q

What cell progenitors are suppressed in pure red cell aplasia?

A

Only erythroid progenitors

33
Q

pure red cell aplasia may occur in association with neoplasms. What are two examples that are particularly associated with this process?

A

Thymoma

Large granular lymphocytic leukemia

34
Q

Apart from thymoma and large granular lymphocytic leukemia, what else is associated with development of pure red cell aplasia?

3

A
  1. Drug exposures
  2. autoimmune disorders
  3. parvovirus infection
35
Q

You obtain the attached image from the peripheral smear of a patient with pure red cell aplasia.

What caused their pure red cell aplasia?

A

Parvo virus

36
Q

Your next patient also has pure red cell aplasia, this time you obtain the findings in the image attached.

What lead to this patient’s pure red cell aplasia?

A

Large granular lymphocytic leukemia

37
Q

You have yet another patient with pure red cell aplasia. You do a peripheral blood smear and don’t really see any definitive results, so you decide to do an MRI and obtain the attached image.

What is causing this patients pure red cell aplasia?

A

Thymoma

38
Q

Your patient is a 72 yo male with pancytopenia and a history of prostate cancer. The peripheral blood smear is attached.

What do the cells shown most often indicate?

A

Dacryocytes in this patient’s smear most likely indicate metastatic cancer.

39
Q

What is myelophthistic anemia?

A

A form of marrow failure in which space occupying lesions replace n.l. marrow elements.

40
Q

What is the most common cause of myelophthisic anemia?

A

Metastatic cancer, most often carcinoma arising in the…

  1. breast
  2. lung
  3. prostate
41
Q

Apart from metastatic cancer, what other disease process can produce myelophthisic anemia?

A

Any infiltrative process (e.g. granulomatous disease) involving the marrow can produce myelophthisic anemia.

42
Q

Based on the attached image, what is the most likely cause of this 47 yo woman’s myelophthisic anemia?

A

Breast cancer metastases to the marrow

43
Q

The diseases that cause myelophthisic anemia lead to marrow distortion and fibrosis which disturbs the mechanisms that regulate the egress of red cells and granulocytes from the marrow. This leads to the finding shown here. What is the term that described the image shown?

A

Leukoerythroblastosis - abnormal release of nucleated erythroid precursors and immature granulocytic forms into peripheral blood.

44
Q

In addition to leukoerythroblastosis, dacryocytes may be seen in myelophthisic anemia. Why do the RBCs take on this shape?

A

Believed to be d/t deformity from their tortuous escape from the fibrotic marrow.

45
Q

What are three “other” forms of marrow failure?

A
  1. Chronic renal failure
  2. hepatocellular liver disease
  3. endocrine disorders
46
Q

What endocrine disorder is especially associated with marrow failure?

A

Hypothyroidism - Hashimoto’s disease for example.

47
Q

What type of anemia is hashimoto’s associated with?

A

Mild normochromic, normocytic anemia

48
Q

A 55 yo woman comes in for her annual physical and gets blood work done. The attached CBC is her result.

What is going on?

A

Polycythemia

49
Q

What does this peripheral blood smear reveal?

A

Polycythemia - sometimes referred to as “ketchup blood”

50
Q

Polycythemia denotes an abnormally high red cell count, usually with a corresponding increase in hemoglobin level. It may be relative, or absolute.

When is it relative?

When is it absolute?

A

Relative - when there hemoconcentration d/t decreased plasma volume.

Absolute - when there is an increase in the total red cell mass

51
Q

What are some examples of causes of relative polycythemia?

A

Dehydrateion d/t…

  1. prolonged emesis
  2. diarrhea
  3. excessive use of diuretics
  4. Exercise
52
Q

When is absolute polycythemia considered to be primary?

A

When it results from an intrinsic abnormality of hematopoietic precursors.

53
Q

When is absolute polycythemia considered to be secondary?

A

When the red cell progenitors are responding to an increase in the levels of erythropoietin.

54
Q

What are two examples of primary absolute polycythemia?

What are EPO levels in this case?

A
  1. Polycythemia vera
  2. Inherited erythropoietin receptor mutations (rare)

EPO levels are low.

55
Q

What are EPO levels like in secondary absolute polycythemia?

A

High

56
Q

What are the 4 main categories of causes of secondary absolute polycythemia?

A

Compensatory

Paraneoplastic

Hemoglobin mutants with high O2 affinity

Inherited defects that stabilize HIF-1alpha

57
Q

What are three examples of compensatory secondary absolute polycythemia?

A
  1. Lung disease
  2. High-altitude living
  3. cyanotic heart disease
58
Q

What are three examples of tumors that cause secondary absolute polycythemia via paraneoplastic syndrome?

A

EPO secreting tumors such as…

  1. Renal cell carcinoma
  2. hepatocellular carcinoma
  3. cerebellar hemangioblastoma
59
Q

What are two examples of inherited defects that stabilize HIF-1alpha and cause secondary absolute polycythemia?

A
  1. Chuvash polycythemia (homozygous VHL mutations)
  2. Prolyl hydroxylase mutations
60
Q

What is the msot common cause of primary polycythemia?

A

Polycythemia vera

61
Q

What do the mutations involved in polycythemia vera lead to?

A

EPO independent growth of red cell progenitors

62
Q

How do we define an “elevated hematocrit value”?

A

Greater than the 97.5th percentile value of the reference range for normal individuals when accounting for…

  1. Altitude of residence
  2. Age
  3. Sex
63
Q

What should elevated hematocrit really be called?

A

Erythrocytosis - implying an increase in red blood cell count, hemoglobin and hematocrit

64
Q

Erythrocytosis refers to an increase in erythroid values without?

A

An accompanying increase in the leukocyte or platelet count.

65
Q

What does polycythemia technically imply?

A

An increase in all three myeloid cell lines in peripheral blood.

  1. erythrocytosis
  2. leukocytosis
  3. thrombocytosis
66
Q

What is the most common cause of erythrocytosis?

A

Cigarrete smoking

67
Q

A hematocrit greater than 50% is associated with what clinical consequences?

A
  1. Increased blood viscocity
  2. increased cardiovascular consequences
  3. Increased risk of thrombosis and hemorrhage
68
Q

What hematocrit levels indicate the need for phlebotomy to decrease the RBC mass?

A

Hematocrit greater than or equal to 54