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High Yield Boards > Hematology > Flashcards

Hematology Flashcards

1
Q

Where do hematologic stem cells originate from?

A

Mesoderm

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2
Q

Timeline of fetal hematopoiesis

A

Initially-secondary yolk sac
5/6 weeks to 22 weeks gestations : fetal liver
After 22 weeks: bone marrow

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3
Q

Fetal hemoglobin is made of

A

Two alpha chains and two gamma chains

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4
Q

Hemoglobin A is made of

A

Two alpha chains and two beta chains

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5
Q

Hemoglobin A2 is made of

A

Two alpha chains and two delta chains

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6
Q

Hemoglobin Barts is made of

A

Four gamma chains

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7
Q

Hemoglobin H is made of

A

Four beta chains

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8
Q

Alpha globin genes: how many and where located

A

Four genes on chromosome 16

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9
Q

Beta globin genes: how many and what chromosome

A

Two genes on chromosome 11

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10
Q

Mutation in sickle cell disease

A

Valine for glutamic acid at position six (on chromosome 11 in beta globulin gene)

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11
Q

Cause of hemoglobin E

A

Abnormal transcription message in the beta-globulin gene results in decreased production of beta chains; 26 GLU —> LYS

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12
Q

How much does 1% fetal hemoglobin represent on a Kleinauer-Betke

A

50 mL of fetal blood

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13
Q

How much RhoGam do you give

A

Number of vials = mL of fetal blood/15
(1 vial = 300 mcg)

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14
Q

How does Apt test work

A

Add NaOH to sample. Fetal hgb is resistant to alkaline denaturation so will be pink. Adult hemoglobin will be yellow-brown

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15
Q

Test for Fanconi Anemia

A

Test chromosomes with mitomycin C —> increased chromosomal breaks

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16
Q

How to calculate Mentzer index?

A

MCV/#RBC; >13.5 consistent with iron deficiency, <11.5 consistent with thalassemia major

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17
Q

Cause of hereditary spherocytosis

A

Defect in membrane proteins (spectrin, ankyrin, band 3, protein 4.2)

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18
Q

Test for hereditary spherocytosis

A

Osmotic fragility test

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19
Q

Inheritance of G6PD deficiency

A

X-linked recessive

20
Q

What does glucose-6-phosphate dehydrogenase do?

A

Converts glucose-6-phosphate to Penrose phosphate in the hexose monophosphate shunt pathway (needed to make glucose); requires NADP (which goes to NADPH and is needed to maintain the antioxidants catalase and glutathione)

21
Q

Inheritance of pyruvate kinase deficiency

A

Autosomal recessive

22
Q

Calculation of blood volume exchanged for partial exchange transfusion

A

Blood volume exchanged= ((observed hamtocrit-desired hematocrit)/observed hematocrit)* infants blood volume (mL)

23
Q

State of normal iron vs with methemoglobin

A

Normal is reduced or ferrous state (2+); methemoglobin is oxidized or ferric state (3+)

24
Q

Inheritance pattern of TAR syndrome

A

Autosomal recessive

25
Q

Inheritance pattern of amegakaryocyte thrombocytopenia

A

Suspected x-linked transmission; male:female = 2:3

26
Q

Neonatal clotting factors compared to adult levels

A

Contact (11, 12, prekallekrein, and HMW kininogen) < 70%
Vitamin K dependent (2, 7, 9, 10) <70% of adult levels
5, 8, 13, vWF, fibrinogen > 70%
ATIII, protein C, protein S: 30%
Thrombomodulin: 3x greater

27
Q

Most common hemophilia and its inheritance

A

Hemophilia A (factor VIII deficiency); c-linked recessive

28
Q

Inheritance pattern of hemophilia B

A

X-linked recessive (factor IX deficiency)

29
Q

Inheritance pattern of factor XI deficiency

A

Autosomal recessive (also associated with Noonan syndrome) No degree of correlation between level of deficiency and symptoms unlike hemophilia A and B)

30
Q

Inheritance of factor 13 deficiency

A

Autosomal recessive; has normal routine clotting studies

31
Q

Calculation for the volume of red blood cells to transfuse in a neonate with acute hemorrhage

A

Desired increase in hematocrit x 1.6 x weight

32
Q

Normal coagulation profile and infants with vitamin K deficient bleeding

A

Normal platelet count, increased PT, normal PTT (PTT can become increased with prolonged vitamin K deficiency)

33
Q

Typical blood volume for:
Term infant
Preterm infant
SGA infant

A

85 mL/kg
100 mL/kg
105 mL/kg

34
Q

Neonate to present with HLH before a month of age frequently having a mutation of the:

A

Perforin gene in chromosome 10q22.1

35
Q

Areas of the brain, most frequently affected by hyperbilirubinemia

A

Globus pallidus and subthalamic nucleus

36
Q

MCHC / MCV in spherocytosis

A

> 0.36

37
Q

What is result if newborn screen shows: FA

A

Normal, no further action

38
Q

What is result if newborn screen shows: FAS or FAC

A

Sickle cell carrier or Hemoglobin C carrier, family testing, and counseling

39
Q

What is result if newborn screen shows: FS

A

Sickle cell anemia, sickle Beta0 thalassemia; start penicillin prophylaxis, refer to hematology, family testing and counseling

40
Q

What is result if newborn screen shows: FSC

A

Sickle Cell Disease; start penicillin ppx, refer to heme, family testing and counseling

41
Q

What is result if newborn screen shows: FSA

A

Sickle Beta+ Thalassemia; start penicillin ppx, heme referral, family testing and counseling

42
Q

How is the Kleihauer Bette test done

A

Blood is treated with citric acid phosphate buffer, fetal cell stay red

43
Q

What can give a false positive result on the Kleihauer-Betke

A

Anything that increases maternal hemoglobin F such as thalassemia, minor, sickle cell, or hereditary persistence of fetal hemoglobin

44
Q

Anemia, short stature, triphalangeal thumbs

A

Diamond Blackfan anemia

45
Q

Features of tar syndrome

A

Thrombocytopenia (from absent megakaryocytes), absent radius but normal thumbs, 30-35% cardiac (TOF or ASD), facial capillary hemangioma, eosinophilia and leukemias reactions

46
Q

Clinical features of amegakaryocytic thrombocytopenia

A

Severe isolated thrombocytopenia, no other abnormalities, 50% develop aplastic anemia, risk of developing leukemia

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