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Clin Med - Hematology > Bone Marrow Failure > Flashcards

Bone Marrow Failure Flashcards

1
Q

What is unilineage bone marrow failure

A

one cytopenia: anemia (RBC) OR neutropenia (WBC) OR thrombocytopenia (platelets)

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2
Q

What is multilineage bone marrow failure

A

multiple cytopenias: anemia (RBC) AND neutropenia (WBC) AND thrombocytopenia (platelets) AKA pancytopenia

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3
Q

define pancytopenia

A

all three cell lines (RBCs, WBCs, and platelets) are decreased

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4
Q

What are RBCs, WBC, and platelets made from

A

hematopoietic stem cells in the bone marrow

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5
Q

Define hematopoiesis

A

process of making blood cells

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6
Q

Define erythropoiesis

A

making erythrocytes

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7
Q

Define myelopoiesis

A

making neutrophils

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8
Q

Define thrombopoiesis

A

making platelets

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9
Q

Define bone marrow failure (BMF)

A

refers to peripheral blood cytopenias resulting from decreased or absent blood cell production in the bone marrow

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10
Q

Is bone marrow failure (BMF) acquired or inherited (genetics)

A

both VERY important to distinguish between the two

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11
Q

Classifications of bone marrow failure (BMF)

A

severe: bone marrow with less than or equal to 25% cellularity AND meets at lest two criteria of cytopenias (ANC less than 500, platelets less than 20K, or ARC less than 40K) moderate mild

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12
Q

ANC ARC

A

absolute neutrophil count absolute retic count

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13
Q

Clinical manifestations of bone marrow failure (BMF)

A

as per their cytopenias ie) symptomatic anemia or bleeding or…etc)

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14
Q

Diagnosis of bone marrow failure (BMF)

A

examination of bone marrow so do a bone marrow biopsy unexplained inc or dec in blood cell counts unexplained inc or dec in abnormal cells

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15
Q

Difference between BMF and Aplastic Anemia (AA)

A

see picture

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16
Q

Diff Dx for hematopoietic stem cell (HSC) injury

A

aplastic anemia or bone marrow failure: idiopathic immune mediated due to drugs, toxin, viruses inherited BMF syndorme (fanconi anemia or dyskeratosis congenita)

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17
Q

Acquired AA/BMF

A

idiopathic (80% of acquired AA/BMF) post-hepatitis drugs toxins infection

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18
Q

Congenital AA/BMF

A

Falconi Anemia Dyskeratosis Congenita Diamond Blackfan Anemia

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19
Q

Clinical presentation of AA/BMF

A

sign and symptoms of: anemia (fatigue, pallor) thrombocytopenia (bruising, bleeding) leukopenia/neutropenia (fever, signs/symptoms of severe infection)

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20
Q

Diagnostic tests

A

CBC with diff bone marrow aspirate and biopsy

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21
Q

Other diagnostic tests for acquired AA/BMF

A

viral studies (parvovirus B19) liver panel vit B12 ANA profile (antinuclear antibody: screens for Lupus) PNH (paroxysmal nocturnal hemoglobinuria) by flow cytometry

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22
Q

Other diagnostic tests for congenital AA/BMF

A

elevated Hgb F elevated MCV chromosome breakage analysis* telomere length testing* genetic panel

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23
Q

Treatment for AA/BMF

A

depends on underlying etiology

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24
Q

pathophysiology of acquired idiopathic AA

A

not sure, but maybe immune mediated destruction of hematopoietic stem cells (HSC)

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25
Q

diagnostic test for acquired idiopathic AA

A

no confirmatory test rule out genetic/congential causes

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26
Q

is acquired idiopathic AA life threatening

A

yes, requires urgent evaluations and care as patients are at high risk for serious bacterial infection

27
Q

treatment of acquired idiopathic AA

A

Best/1st choice: hematopoietic stem cell (HSC) transplant 2nd choice: immunosuppressive therapy (combo of antithymocyte globulin (ATG) and cyclosporine (CSA): unclear why works but they are lymphocytotoxic so they may decrease immune mediated destruction of HSC) (about 1/3 relapse) also: supportive care with blood transfusions util definitive therapy

28
Q

treatment of acquired post-hepatits AA

A

similar to acquired idiopathic AA

29
Q

treatment of acquired AA due to infection

A

similar to acquired idiopathic AA

30
Q

treatment of acquired AA due to drugs

A

remove offending agent

31
Q

treatment of acquired AA due to toxins

A

remove offending agent

32
Q

another name for congenital/inherited/genetic AA

A

inherited bone marrow failure syndrome (IBMFS)

33
Q

the three IBMFS

A

Falcon Anemia Dyskeratosis Congentia Diamond Blackfan Anemai

34
Q

why is it important to rule out IBMFS before looking at acquired AA causes for patients with AA prior to initiation of therapy (another way to ask this question: why is it important to distinguish if AA is acquired or congenital

A

IBMFS will not respond to immunosuppressive therapy (have exposed them to drugs that could have nasty side effects and have delayed definitive Dx) other family members may be affected (this is important info if matched related bone marrow transplant is planned)

35
Q

Common phenotypes of IBMFS

A

poor growth endocrinopathies anatomical abnormalities of the limbs/digits (i.e. thumbs) anatomical abnormalities of genitourinary tract anatomical abnormalities of heart

36
Q

Persons with IBMFS are predisposed to develop what

A

leukemia or other cancers

37
Q

diagnosis of IBMFS

A

genetic testing

38
Q

treatment of IBMFS

A

HSC transplant is curative but not always indicated for IBMFS

39
Q

define falconi anemia (FA)

A

mostly autosomal recessive disorder characterized by PROGRESSIVE BMF leading to pancytopenia (often starts with isolated thrombocytopenia and/or macrocytosis)

40
Q

common congenital abnormalities of falconi anemia (FA)

A

abnormal skin pigment (cafe au lait spot), short stature, skeletal abnormalities (microophthalmia: small eyes), upper limb abnormalities (thumbs), and reproductive organ abnormalities (25% may have no anatomical abnormalities)

41
Q

when are hematologic abnormalities noticed in Falconi Anemia (FA)

A

median age 7

42
Q

diagnostic test for Fanconi Anemia (FA)

A

chromosome breakage: increased chromosome breakage when exposed to DNA cross linking agents

43
Q

treatment of Fanconi Anemia (FA)

A

HSC transplant supportive blood transfusions until HSCT

44
Q

prognosis of Fanconi Anemia (FA)

A

800-1000x higher risk of developing cancer inc risk of myelodysplastic syndrome (MDS) and progression to acute myeloid leukemia (AML) requires continued monitoring for development of endocrinopathies, cancer surveillance, and anatomic abnormalities

45
Q

characteristics of Dyskeratosis Congenita

A

BMF (may present early on with mild cytopenia, most often thrombocytopenia) high incidence of cancer lung and liver fibrosis

46
Q

what are those with Dyskeratosis Congenita at risk for

A

MDS leukemia head/neck cancer

47
Q

what percent of those with Dyskeratosis Congentia develop BMF by age 30

A

90%

48
Q

Clinical presentation of Dyskeratosis Congentia

A

leukoplakia (oral) hypo/hyper-pigmentation (reticulate) nail dystrophy can show all together as classic mucocutaneous triad

49
Q

Common family Hx of Dyskeratosis Congentia

A

MDS early graying of hair nail abnormalities lung fibrosis

50
Q

Pathogenesis of Dyskeratosis Congenita

A

mutations in genes encoding for factors implicated in telomere function

51
Q

diagnostic test for Dyskeratosis Congenita

A

test for telomere length (will be very short for age)

52
Q

define Diamond Blackfan Anemia (DBA)

A

genetically and clinically heterogeneous due to mutations in the ribosomal subunit, bone marrow will have paucity of erythroid precursors but normal myeloid precursors and megakaryocytic

53
Q

Presentation of Diamond Blackfan Anemia (DBA)

A

isolated macroytic anemia within first year of life, generally rest of CBC looks normal

54
Q

characteristics of Diamond Blackfan Anemia (DBA)

A

skeletal abnormalities (thumb) renal issues craniofacial or cardiac abnormalities short stature

55
Q

diagnosis of Diamond Blackfan Anemia

A

RBC have inc activity of adenosine deaminase (eADA)

56
Q

what are patients with Diamond Blackfan Anemia at risk for

A

malignancies

57
Q

treatment of Diamond Blackfan Anemia

A

supportive care with RBC transfusions anemia may respond to steroid therapy HSC transplant if indicated

58
Q

what is the only curative option for congenital/inherited BMF

A

HSC transplant

59
Q

what are your options to treat acquired BMF

A

HSC transplant immune suppressive therapy

60
Q

HLA matching best source

A

MRD (matched related donor) usually sibling

61
Q

other sources for HLA matching

A

MUD (matched unrelated donor) haploidentical (50% match of related donor so usually parent: not the best outcomes: in emergency situations)

62
Q

complications of HSC transplant

A

graft rejection (original disease returns because recipients immune system attacked and killed donated stem cells) graft vs host disease (when donor stem cells attack the recipients native tissues) above increased with mismatching between donor and recipient

63
Q

complications of HSC transplant if use autologous source (from donor)

A

no complications except that there is a risk of same disease returning

Clin Med - Hematology (9 decks)

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