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Hematology Flashcards

1
Q

HbA

A

alpha2, beta2

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2
Q

HbA2

A

alpha2, delta2

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3
Q

HbF

A

alpha2, gamma2

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4
Q

HbH

A

beta4 - nonfunctional

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5
Q

HbBarts

A

gamma4 - nonfunctional

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6
Q

What shifts oxygen dissociation curve up and to the left

A

HbF - holds on to it tighter

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7
Q

What shifts oxygen dissociation curve down to the right

A

acidosis, hypoxia, 2,3 DPG

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8
Q

Which iron can bind oxygen, which cannot

A

Fe2+ can bind; Fe3+ cannot bind O2

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9
Q

What causes Fe3+

A

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

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10
Q

What is the Rx for methemoglobin

A

methylene blue

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11
Q

What is methylene blue contraindicated in?

A

G6PD

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12
Q

What conditions can lead to production of methemoglobin?

A

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

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13
Q

What co-exists with lead poisoning

A

Fe deficiency

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14
Q

what are findings in lead poisoning

A

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

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15
Q

What is Hb at birth typically?

A

15-20 g/dl

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16
Q

When does anemia nadir?

A

2-3 months (mean 10.7)

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17
Q

When does nadir happen for premies?

A

earlier and lower nadirs

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18
Q

Which stores is smaller: folate or B12

A

folate

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19
Q

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

A

1 month; anemia by 4 months

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20
Q

Histology of folate, B12 deficiency

A

hypersegmented poly

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21
Q

HbF and A

A

normal

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22
Q

HbFAS

A

sickle cell trait

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23
Q

HbFS

A

presumptive sickle cell

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24
Q

HbFS(little A)

A

sickle cell - beta thalassemia low MCV

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25
Q

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

A

splenic sequestration

26
Q

Rx splenic sequestration

A

transfusion

27
Q

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

A

increased stroke risk

28
Q

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

A

Hand-foot syndrome - one of first symptoms

29
Q

Pattern: dactylitis

30
Q

Hb normal estimate

A

11 + 0.1 x age

31
Q

Lower limit of MCV estimate

A

70 + 1 x age

32
Q

Mechanis of lead toxicity

A

inhibits heme synthase from protoporphyrin

33
Q

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

A

anemia of chronic disease

34
Q

What is the sign of adequate treatment

A

increased ferritin storage

35
Q

Peak incidence of iron deficiency

A

6 months to 3 years - premies at higher risk

36
Q

Indication for transfusion

A 
Acute chest
Sequestration
Stroke
Refractory priapism
Aplastic crisis
37
Q

What is cause of aplastic crisis?

A

Parvovirus B12

38
Q

Association - polychromasia

A

reticulocytes

39
Q

Association - spherocytes

A

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

40
Q

Association - howell-jolly bodies

A

small condensed nuclei

asplenia, extramed hemopoiesis, marrow replacement

41
Q

Association - Heinz bodies

A

Aggregated Hb - supravital stain

Thal, unstable Hb, RBC enzyme defects, G6PD

42
Q

Association - basophilic stippling

A

ribosomes condense

Thal, Fe deficiency, lead poisoning

43
Q

Schistocytes

A

fragmented - microangiopathic hemolytic anemia

44
Q

macrocytosis with megaloblastic

A

B12/folate deficiency

45
Q

macrocytosis without megaloblastic

A

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

46
Q

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

A

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

47
Q

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

A

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

48
Q

Beta-thal phenotype

A

silent carriers, amount of HbA2 and HbF levels are normal

49
Q

beta-thal trait

A

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

50
Q

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

A

beta-thal trait

51
Q

Severe thal phenotype

A

ineffective erythropoiesis

52
Q

What is the difference between severe beta thal and alpha thal

A

alpha thal there is hemolysis

beta thal has ineffective erythropoiesis

53
Q

Thal major - heterozygous for 2 different mutations

54
Q

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

A

thal major

55
Q

Rx for severe beta-thal

A

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

56
Q

Cause of hereditary spherocytosis

A

abnormal ankyrin and spectrin

57
Q

Pattern: parvovirus B19 infection can trigger

A

hereditary spherocytosis

58
Q

pattern: Pattern: neonatal jaundice, extravascular hemolysis

A

hereditary spherocytes

59
Q

Rx for hereditary spherocytes

A

splenectomy fixes hemolysis, but infection risk high

60
Q

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

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