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Heme - Wier/Pallera > Bone Marrow Failure Syndromes > Flashcards

Bone Marrow Failure Syndromes Flashcards

1
Q

What is the most common cause of aplastic anemia?

A

Idiopathic causes

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2
Q

What is a viral cause of Aplastic Anemia?

A

Parvovirus

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3
Q

If you suspect aplastic anemia what should you be looking for in the bone marrow?

A

*Look for Hypercellular vs. Hypocellular

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4
Q

What are some conditions that cause bone marrow failure with hypercellular bone marrow?

A
  • Bone Marrow Infiltration via Malignancy
  • B12 or Folate Deficiency (Megaloblastic)
  • Storage Disorders
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5
Q

What are some conditions that cause bone marrow failure with hypocellular bone marrow?
• Congenital
• Acquired

A

Congenital
• Fanconi’s Anemia

Acquired 
• Idiopathic
• Myelodysplastic Syndrome
• Drugs/Chemicals
• Radiation
• Viruses
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6
Q

What 2 things Define Aplastic Anemia?

• who gets it?

A
  • Peripheral PANcytopenia
  • Hypocellular Bone Marrow

*Can occur in any age group and in both genders

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7
Q

What is the primary mechanism by which cells are killed in aplastic anemia?
*What cell line is killed?

A

Immune Mediated Destruction of Hematopoietic Stem Cells

**THIS IS WHY YOU GET PANCYTOPENIA

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8
Q

What are some infectious causes of Aplastic Anemia?

A
  • Infectious Mononucleosis
  • Hepatitis
  • Parovirus
  • Cytomegalovirus
  • Miliary Tuberculosis
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9
Q

T or F: Paroxysmal Nocturnal Hemoglobinuria is a potential cause of Aplastic Anemia.

A

True

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10
Q

What drugs are known to causes aplastic anemia?

A
  • Chloramphenicol

- Quinacrine

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11
Q

What lab findings would you expect to see on someone with aplastic anemia?

  • explain each of these findings.
  • what is the hallmark finding?
A

RELATIVE lymphocytosis:
• Lymphocytes live much longer than neutrophils

Normochromic, Normocytic RBCs (mild to moderate anisocytosiis and poikilocytosis possible)
• cells ARE NOT neoplastic, they are just getting destroyed

LOW Reticulocytes:
• Hallmark Finding

HYPOcellular Bone Marrow
• more than 70% fat in marrow

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12
Q

What are 3 common treatments of Aplastic Anemias?

A
  • Immunosuppression
  • Stem Cell Transplant
  • Transfusion
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13
Q

What should be your sequence of treatment for aplastic anemias?

A
  1. Withdrawal Offending Agent (drug, chemical, etc)
  2. Supportive Care (Antibiotics for neutropenia, Transfusion)
  3. Immunosuppressive Therapies
  4. Hematopoietic Cell Transplantation (HSCT)
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14
Q

Why has is been hypothesized that Aplastic Anemia is almost certainly an immune mediated phenomenon?

A

Because Immunosuppressive therapy is so effective at treating it

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15
Q

What defines Pure Red Cell Aplasia?

A

*Selective Decreases in erythroid precursor cells in the bone marrow while WBCs and Platelets are unaffected

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16
Q

What are some causes of Pure Red Cell Aplasia?

*are these acquired or congenital?

A

Acquired

Causes:
• Viral (parovirus) or bacterial infections
• Patients with Hemolytic Anemias may halt erythorpoeisis
• Patients with Thyoma - T cell mediated responses against red cells bone marrow EPO or erythroblasts are sometimes produced

17
Q

How do you treat a Red Cell Aplasia?

A
  • Supportive Care

* Immunosupressives

18
Q

What is the difference between a myelodysplastic syndrome and an aplastic anemia?

A

Presence or absence of neoplastic cells in the bone marrow

19
Q

What are Myelodysplastic Syndromes?

• How do they tend to terminate?

A

Primary Neoplastic Stem Cell Disorders that tend to Terminate in Acute Leukemia

20
Q

Do quantitative or qualitative abnormalities causes myelodysplastic syndromes?

A

QUALITATIVE
• Most of these have defects in differentiation (that’s why they are dysplastic syndromes and not proliferative syndromes)

21
Q

What are some findings that are characteristic of a peripheral smear in a myelodysplastic syndrome?

A
  • Nucleated RBCs
  • Oval Macrocytes
  • HYPOsegmented neutrophils, pseudo-Pelger-Heut PMNs
  • HYPOgranulated neutrophils
  • Hyperchromatin clumping
  • Giant Platelets
22
Q

What finding is characteristic of bone marrow in someone with myelodysplastic changes?

A

Ringed Sideroblasts

23
Q

What cell type are myelodysplasias thought to arise from?

A

Multi-Potent Stem Cells

24
Q

What do myelodysplasias turn into when more than 20% of peripheral or bone marrow blasts are seen in a patient?

A

Acute Myelogenous Leukemia (AML)

25
Q

What treatments are often given for MDS?

• Why are these treatments given?

A

Supportive Care:
• EPO

Chemotherapy - HYPOMETHYLATING AGENTS:
• Azacytidine
• Decitabine
**Allow Differentiation of Cells

26
Q

What is lenalidomide used to treat?

A

Myelodysplastic Syndrome with del(5q)

27
Q

In what categories of Myeloproliferative Disorders would you consider doing a stem transplant in.

A
  • Younger Patients (under 60-75 years)
  • More Severly affected Patients
  • Potential for cure with therapy
28
Q

What two conditions are about the only one where you will see Auto SCT (stem cell transplant performed)?

A
  • Lymphomas

* Multiple Myelomas

29
Q

What are the advantages of doing allogeneic vs. Autologous SCT?

A
  • Can be used when patients bone marrow fails

* Donor cells can attack remaining cancer cells in recipient

30
Q

What conditions could not be treated with Autologous SCT and why?

A

Aplastic Anemia - SCT won’t work because you’re body is having an immuno response to itself so the cells you are re-emplanting are the root of the problem

Myelodysplatic Syndrome - no good cells are left to harvest

Heme - Wier/Pallera (12 decks)

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