Hematology

Question 1

HbA

Answer 1

alpha2, beta2

Question 2

HbA2

Answer 2

alpha2, delta2

Question 3

HbF

Answer 3

alpha2, gamma2

Question 4

HbH

Answer 4

beta4 - nonfunctional

Question 5

HbBarts

Answer 5

gamma4 - nonfunctional

Question 6

What shifts oxygen dissociation curve up and to the left

Answer 6

HbF - holds on to it tighter

Question 7

What shifts oxygen dissociation curve down to the right

Answer 7

acidosis, hypoxia, 2,3 DPG

Question 8

Which iron can bind oxygen, which cannot

Answer 8

Fe2+ can bind; Fe3+ cannot bind O2

Question 9

What causes Fe3+

Answer 9

antibiotics, anesthetics, aniline dyes, metoclopramide, rasburicase, chlorates, nitrates and bromates.

Question 10

What is the Rx for methemoglobin

Answer 10

methylene blue

Question 11

What is methylene blue contraindicated in?

Answer 11

G6PD

Question 12

What conditions can lead to production of methemoglobin?

Answer 12

acute gastroenteritis (stool bicarb) loss –> acidosis, bacterial overgrowth –> nitrates

Question 13

What co-exists with lead poisoning

Answer 13

Fe deficiency

Question 14

what are findings in lead poisoning

Answer 14

basophilc stippling in RBC, microcytosis only with Fe deficiencies, free erythrocyte protoporphyrin

Question 15

What is Hb at birth typically?

Answer 15

15-20 g/dl

Question 16

When does anemia nadir?

Answer 16

2-3 months (mean 10.7)

Question 17

When does nadir happen for premies?

Answer 17

earlier and lower nadirs

Question 18

Which stores is smaller: folate or B12

Answer 18

folate

Question 19

When can deficiency of folate be seen and how quickly can anemia develop?

Answer 19

1 month; anemia by 4 months

Question 20

Histology of folate, B12 deficiency

Answer 20

hypersegmented poly

Question 21

HbF and A

Answer 21

normal

Question 22

HbFAS

Answer 22

sickle cell trait

Question 23

HbFS

Answer 23

presumptive sickle cell

Question 24

HbFS(little A)

Answer 24

sickle cell - beta thalassemia low MCV

Question 25

Pattern: SC pt, sudden weakness, dyspnea, abdominal pain, low retic count, in kids <6

Answer 25

splenic sequestration

Question 26

Rx splenic sequestration

Answer 26

transfusion

Question 27

In SCD patient, TCD show high flow, what does that indicate?

Answer 27

increased stroke risk

Question 28

Pattern: SCD patient, refusal to bear weight, low-grade feber, incarsed HBs decreased HBF

Answer 28

Hand-foot syndrome - one of first symptoms

Question 29

Pattern: dactylitis

Answer 29

SCD

Question 30

Hb normal estimate

Answer 30

11 + 0.1 x age

Question 31

Lower limit of MCV estimate

Answer 31

70 + 1 x age

Question 32

Mechanis of lead toxicity

Answer 32

inhibits heme synthase from protoporphyrin

Question 33

Pattern: decreased Fe, increased ferritin, decreased EPO relative to degree of anemia

Answer 33

anemia of chronic disease

Question 34

What is the sign of adequate treatment

Answer 34

increased ferritin storage

Question 35

Peak incidence of iron deficiency

Answer 35

6 months to 3 years - premies at higher risk

Question 36

Indication for transfusion

Answer 36

``` Acute chest Sequestration Stroke Refractory priapism Aplastic crisis ```

Question 37

What is cause of aplastic crisis?

Answer 37

Parvovirus B12

Question 38

Association - polychromasia

Answer 38

reticulocytes

Question 39

Association - spherocytes

Answer 39

HS-spectrin, autoimmune antibody, Hb precipitation thal (spleen take a bite so it plumps up) Positive osmotic fragility test

Question 40

Association - howell-jolly bodies

Answer 40

small condensed nuclei | asplenia, extramed hemopoiesis, marrow replacement

Question 41

Association - Heinz bodies

Answer 41

Aggregated Hb - supravital stain | Thal, unstable Hb, RBC enzyme defects, G6PD

Question 42

Association - basophilic stippling

Answer 42

ribosomes condense | Thal, Fe deficiency, lead poisoning

Question 43

Schistocytes

Answer 43

fragmented - microangiopathic hemolytic anemia

Question 44

macrocytosis with megaloblastic

Answer 44

B12/folate deficiency

Question 45

macrocytosis without megaloblastic

Answer 45

bone marrow failure syndromes - Diamond Blackfan, Fanconi anemia, aplastic anemia, drugs, chronic liver disease, hypoTH

Question 46

Describe the basis of the genetic mutation that occurs with thalassemia alpha

Answer 46

2 copies of alpha-globin gene on chromosome 6 1 missing - silent carrier - normal 2 missing - mild, hypochromic, microcytic, mild anemia 3 missing - moderate anemia 4 missing - lethal

Question 47

Describe the basis of the genetic mutation that occurs with thalassemia beta

Answer 47

1 beta globin gene - disease is due to the extent that gene expression is decreased; expression of disease is also dependent on patient's capacity to make gamma globulin for HbF

Question 48

Beta-thal phenotype

Answer 48

silent carriers, amount of HbA2 and HbF levels are normal

Question 49

beta-thal trait

Answer 49

heterozygous for mutation; microcytic hypochromic cells with mild anemia; target cells, basophilic stippling, ineffective erythropoiesis (leading to hepatosplenomegaly)

Question 50

Pattern: mild anemia, heptaosplenomegaly, mild anemia, target cells

Answer 50

beta-thal trait

Question 51

Severe thal phenotype

Answer 51

ineffective erythropoiesis

Question 52

What is the difference between severe beta thal and alpha thal

Answer 52

alpha thal there is hemolysis | beta thal has ineffective erythropoiesis

Question 53

Thal major - heterozygous for 2 different mutations

Answer 53

very bad

Question 54

Pattern: hb 3-7g/dL, MCV 50-60, retic <1%, RBC, target cells, spherocytes, fragmented cells, massive HSM, frontal bossing, maxillary prominence (failure to pneumatize sinuses), growth retardation, thining cortices, dilated marrow cavities in long bones, skull xray looks like hair on ends

Answer 54

thal major

Question 55

Rx for severe beta-thal

Answer 55

splenectomy increases RBC survival; chelation therapy, BMT curative, 5-azacytidine to increase HbF

Question 56

Cause of hereditary spherocytosis

Answer 56

abnormal ankyrin and spectrin

Question 57

Pattern: parvovirus B19 infection can trigger

Answer 57

hereditary spherocytosis

Question 58

pattern: Pattern: neonatal jaundice, extravascular hemolysis

Answer 58

hereditary spherocytes

Question 59

Rx for hereditary spherocytes

Answer 59

splenectomy fixes hemolysis, but infection risk high

Question 60

pattern: neonatal jaundice, after oxidative challenge, progressive pallor, low grade fever, discolored urine, jaundice, plychromasia, Heinze bodies, high retic count

Answer 60

G6PD