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CPHON Slides > 9 Hematology > Flashcards

9 Hematology Flashcards

1
Q

What percentage of plasma is water?

A

90%

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2
Q

What is included in plasma? (3 things)

A

clotting factors, fibrinogen and antibodies

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3
Q

lymphocytes are divided into what 2 categories?

A

B cell (antibody) and T cell (hypersensitivity)

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4
Q

What is the inheritance of sickle cell?

A

autosomal recessive. so 2 carriers have a 1 in 4 chance of an affected or unaffected child. and a 1 in 2 chance that they will have a carrier.

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5
Q

What is the inheritance of HS?

A

autosomal dominant, but only one abnormal gene is needed for the disorder to express itself so the chance is 50/50

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6
Q

Inheritance pattern of Fanconi Anemia?

A

autosomal recessive. 1/4 chance it will affect the child if both parents are carriers

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7
Q

Patient with anemia and some physical anomalies including short stature, skin pigmentation, upper limb deformities, microcephaly (and could have GI/renal and cardiac issues). Which type of anemia?

A

Fanconis

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8
Q

What is the percent chance people with Fanconi’s end up progressing to MDS or AML?

A

50%

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9
Q

Which cell lines are affected with fanconis?

A

All 3

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10
Q

What does a chromosomal breakage study diagnose?

A

Fanconis anemia

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11
Q

What is the cure for Fanconi’s anemia?

A

transplant - 66% survival with matched donors, 29% with unrelated matched

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12
Q

What is an early sign of Schwachman Diamond syndrome?

A

Pancreatic insufficiency - fatty foul smelling stools due to fatty infiltration.

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13
Q

Short stature, bone deformities, pancreatic insufficiency, FTT and bone marrow failure would likely be which type of anemia?

A

Schwachman Diamond

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14
Q

Schwachman Diamond is what type of inheritance?

A

autosomal recessive

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15
Q

Which diseases include an increased risk for MDS/AML and require regular bone marrow biopsies?

A

(Schwachman Diamond, Fanconi’s, for the BMs) dyskeratosis congenita, diamond blackfan anemia, congenital neutropenias

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16
Q

Hyperpigmentation (trunk, neck, face), nail dystrophy (ridges in the nails and distorted, weak fingernails) and leukoplakia (which patches in cheeks) can be part of which syndrome? What happens as a result of this syndrome?

A

Dyskeratosis congenita, the severe inherited form. Progressive bone marrow failure is part of this in the second decade of life usually. often it is thrombocytopenia first.

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17
Q

What is an example of pure red cell aplasia?

A

diamond blackfan. cell defect kills RBC precursers.

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18
Q

Diamond blackfan anemia is inherited how?

A

autosomal dominant.

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19
Q

50% of people with diamond blackfan have these features:

A

deformities of hands, short stature, snub nose, thick upper lip, light hair, wide set eyes. some

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20
Q

Macrocytic anemia with low or absent retic (sometimes high plt and mild neutropenia) is which disease?

A

Diamond Blackfan anemia

21
Q

What is the treatment for diamond blackfan anemia?

A

Steroids, then tapered down until possibly discontinued. supportive transfusions. only cure is transplant. many have remissions while weaning steroids-fails often.

22
Q

What to watch for with diamond blackfan patients?

A

iron overload, steroid side effects (including decreased response to puberty, impaired immunity, bone density loss, etc).

23
Q

Pallor, fatigue, heart murmur preceeded by viral illness. Low hgb and retic. What is this?

A

TEC, resolves in 4-6 weeks, etiology unknown

24
Q

What is Kostmann syndrome?

A

severe congenital neutropenia, more common with Middle Eastern descent

25
Q

Possible causes of autoimmune neutropenia?

A

post infections like Parvo, lupus, ALPS,

26
Q

Treatment for autoimmune neutropenia?

A

usually not required, can use GCSF or IVIG as needed

27
Q

What is the difference between warm agglutinins and cold agglutinins? Which is more life threatening?

A

Warm - antibodies destroy RBCs at normal body temp. Rapid and life threatening
Cold - antibodies activate below normal body temp - mild, self limiting, need warm blood if transfused.

28
Q

Acute symptoms: Low coombs, low hgb, high retic and bili, jaundice, splenomegaly. abnormally shaped RBCs. Possible diagnosis?

A

Autoimmune hemolytic anemia.

29
Q

Why do patients with automimmune hemolytic anemia have dark urine?

A

high bili and jaundice due to lysing RBCs.

30
Q

Treatment for autoimmune hemolytic anemia.

A

high dose steroids and IVIG. may need transfusions and splenectomy. Plasmapharesis may work but probably temporary.

31
Q

What is PNH?

A

an acquired hemolytic anemia. life threatening. It is a result of a mutation in the PIGA gene.

32
Q

What is a treatment option for PNH?

A

transplant. or immunosuppression like aplastic anemia. could also use Solris, a monoclonal antibody

33
Q

How is G6PD diagnosed?

A

usually on newborn screen. prolonged jaundice

34
Q

What does a pyruvate kinase deficiency cause?

A

hemolytic anemia.

35
Q

Pt with anemia and thrombocytopenia after infectious diarrhea which led to acute renal failure could have

A

HUS. most common cause of ARF in children.

36
Q

What 3 factors cause vaso-occlusive crisis in SCD?

A

ischedmia due to vessel occlusion, endothelial damage, local inflammation

37
Q

What leads to 25% of all SCD deaths?

A

ACS

38
Q

SCD - pain crisis, pneumonia, prior episode of ACS and underlying pulmonary disease are all risk factors for?

A

ACS. lungs occluded with sickled RBCs leads to rapid deterioration of respiratory function.

39
Q

Why consider neuropsych testing for SCD?

A

dleayed learning, possible silent infarcts

40
Q

How is thalassemia inherited?

A

autosomal recessive. one or more gene mutations.

41
Q

For alpha thal - what are the 4 types? (one is asymptomatic, one has mild/microcytic anemia, one has mild to severe anemia and one is lethal)

A

a thal silent carrier - asymptomatic
a thal trait - mild anemia
HbH - mild to severe anemia
Hydrops fetalis - lethal

42
Q

Alpha thal vs beta thal - which is usually a deletion and which is usually a mutation?

A

alpha thal - degrees of deletion

beta thal - degrees of mutation

43
Q

Normal amount of a chains but not enough B chains results in an excess of a chains and leads to hemolysis. Which disease?

A

beta thal

44
Q

Which beta thal is asymptomatic with mild anemia? which is moderate anemia? which is severe and transfusion dep?

A

minor (trait), intermedia, and major.

45
Q

What 2 factors are major contributers to cardiac disease and endocrinopathies in thalassemia?

A

iron overload and poor chelation.

46
Q

Pt has a normal PT and prolonged PTT, which bleeding disorder?

A

hemophilia

47
Q

Lymphoproliferative disorders, wilms tumors, lupus, heartdisease and valproic acid can lead to which disorder?

A

acquired VwB

48
Q

Patient with elevated D Dimer, low platelets and ATIII, and prolonged PT/PTT?

A

DIC

49
Q

Treatment of DIC?

A

FFP/cryo, transfuse RBC/plt if needed, ventilator/dialysis if needed, and treat underlying cause

CPHON Slides (24 decks)

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