Bone Marrow Disorders Flashcards

1
Q

Pure red cell aplasia (PRCA)

A

Severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence of erythroid absence.
The bone marrow ceases to produce RBC’s

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2
Q

PRCA causes

A
Autoimmune disease
Thymoma
Viral infxns
Large granular lymphocyte leukemia
Idiopathic
congenital
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3
Q

PRCA dx

A

Very low retic count and anemia when the following are present:

  • normocytic, normochromic anemia
  • Absolute retic < 10,000
  • Normal WBC and platelet count
  • Normal marrow, except no erythroid precursors
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4
Q

PRCA Tx

A

Red cell transfusions
Removal of cause
Usually relapses

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5
Q

Myelophthisic Anemia (MA)

A

Severe anemia caused by the displacement of hematopoetic bone marrow into peripheral blood.

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6
Q

MA Causes

A

Chronic myeloproliferative disease
Leukemia
Lymphoma
Metastatic carcinoma lymphopma

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7
Q

MA Dx

A

Tear drop RBC’s
Immature granulocytes
Treat underlying cause/cancer

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8
Q

Myelodysplastic Syndrome (MDS)

A

Heterogenous group of stem cell disorders characterized by dysplastic and ineffective blood cell production.
Dysplasia of one or more cell lines
AKA pre-leukemia

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9
Q

MDS patients have a reduction in which blood cells?

A

Normal RBC’s
Platelets
Granulocytes

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10
Q

MDS leads to?

A

Anemia
Bleeding
Increased risk for infxn

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11
Q

Who is affected by MDS?

A

Elderly (60 - 80)

Double the incidence of AML

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12
Q

Transformation of MDS to acute leukemia occurs in ___% of patients.

A

40%

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13
Q

Manifestations of MDS

A
Anemia (macro, normo)
Oval macrocytes
Neutropenia
Thrombocytopenia
Dysplastic features
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14
Q

Blasts account for ___ than 20% in MDS.

A

Less than 20%. If greater, consider Acute Myeloid Leukemia

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15
Q

MDS Tx

A

Bone marrow transplant. It is risky the older you get.

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16
Q

Aplastic anemia (AA)

A

Severe anemia characterized by pancytopenia accompanied by hypocellular bone marow

17
Q

Aplastic causes

A

Direct stem cell injury (meds)
Autoimmune
SLE (rare)

18
Q

How many cases of aplastic anemia are idiopathic?

A

70%

19
Q

AA symptoms

A
Pallor
Mucousal bleeding
ecchymoses
infxns
gingivitis
20
Q

AA lab findings

A
Pancytopenia
normo, normo anemia
Low retic
Empty fatty bone marrow
Few hematopoetic cells
21
Q

AA Tx

A

Bone marrow transplant
Immunosuppression
Transfusions if needed
Withdraw etiological agent if found

22
Q

Fanconi Anemia (FA)

A

Genetic disease, 1 in 350,000 births.
Bone marrow failure by 40
Development of AML
Usually diagnoses by 8 yrs

23
Q

Is FA the most common cause of inherited bone marrow failure?

A

Yes.

24
Q

FA Dx

A

Chromosome fragility test (MMC, DEB)

25
Q

FA Tx

A

Transfusions
Androgens
Growth factors
Bone marrow transplant

26
Q

Allogenic

A

Comes from another person

30 - 50% will die

27
Q

Syngenic

A

Comes from an identical twin

28
Q

Autologous

A

Comes from the patient

<5% will die

29
Q

MUD

A

Matched unrelated donors

30
Q

MRD

A

Matched related donors

25% chance of being a match

31
Q

CD34+

A

Identification for stem cells