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Erez's HEM TEST notes 2024 > Benign hematology facts > Flashcards

Benign hematology facts Flashcards

1
Q

Definition of Transfusion independence in thalassemia

A

less than 6 PC in 6 months
no PC in previous 2 months

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2
Q

Hb in Thalassemia intermedia

A

7-10 g%

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3
Q

Beta thalassemia intermedia genotype

A

Compound heterozygotes

Homozygotic to non severe mutations (b0/b+)
# Beta heterozygote with alpha gene excess
# Hemoglobin E/β-thalassemia

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4
Q

Hereditary spherocytosis inheritance

A

AD 66%

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5
Q

Disease more common in NTDT

A

PHTN including RV dys
VTE
Silent cerebral ischemia
Cholelithiaisis
Leg ulcer
Splenomegaly

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6
Q

Luspa for NTDT

A

BEYOND study
improves Hb levels after 3 months
Decreases ferritin

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7
Q

Indications for iron chelation in NTDT

A

Ferritin level > 800 ng/mL
Liver iron concentration > 5 mg/g

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8
Q

Hydrea in NTDT

A

Improves Hb (1 g%)
May aid with PHTN/Extramedullary hematopoiesis

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9
Q

Reasons to start transfusions in NTDT

A

PHTN
Extramedullary hematopoiesis
Worsening anemia

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10
Q

Deferasirox in NTDT

A

PO
THALASSA trial
Improved iron overload in NTDT
Approved also for TDT
SE: GI, rash, LFT, increased Cre, loss of hearing, cataract
New formulation JADENU with less SE. Not approved in Israel

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11
Q

Dose related drug neutropenia vs. agranulocytosis

A

Mild neutropenia is dose dependent- usually asymptomatic
Agranulocytosis is immune mediated and usually pts are febrile

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12
Q

Mortality rates in agranulocytosis

A

7%

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13
Q

Ferrinject in HF pts with IDA

A

HEART-FID trial
Phase III
vs placebo
Did not show benefit in composite outcome
Results might have been affected by covid

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14
Q

Hereditary spherocytosis gene

A

ANK1
Band3
Spectrin
Protein 4.2

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15
Q

Hereditary spherocytosis prevalence

A

1/1000

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16
Q

HS special hemolytic complications

A

Neonatal jaundice
Megaloblastic crisis- due to folate def during pregnancy

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17
Q

Iron overload parameters in HS

A

Ferritin >500 mg/l
Saturation >60%
Liver iron concentration >4

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18
Q

Renal complications of HS

A

Distal renal tubular acidosis
in Band3 mutated pts

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19
Q

HS diagnosis

A

Screen-
# EMA- reduced binding of EMA to band 3 (more sensitive and specific)
# Osmotic fragility

Confirm- Genetic testing

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20
Q

Contraindication for splenectomy in hereditary hemolytic anemias

A

Dehydrated hereditary stomatocytosis
Due to high thrombotic risk

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21
Q

Stomatocytosis

A

Light wide transverse band in the middle of erythrocytes (stoma)
PIEZO1 in dehydrated

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22
Q

Acquired stomatocytosis

A

Alcoholism
Hepatobiliary disease
Malignant neoplasms
CV disorders

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23
Q

Rapidly progressive hemolytic
anemia in alcoholism

A

Zieve syndrome
Association with end-stage alcoholic cirrhosis
Acanthocytes are present

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24
Q

McLeod phenotype

A

Acanthocytes due to reduced kell antigen
X linked
Causes hemolysis

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25
Q

Rh deficiency (null) syndrome

A

Full or partial lack of Rh
causing mild hemolysis

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26
Q

Diagnostic test for congenital enzymopathies and skeletal RBC abnormalities

A

Targeted NGS

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27
Q

Coombs negative AIHA %

A

10%

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28
Q

Secondary warm AIHA etiologies
(5 causes)

A
  1. LY (CLL)
  2. AI (SLE)
  3. Non lymphoid cancers (ovarian tumors)
  4. Inflammatory diseases
    (UC)
  5. Drugs (Cephalosporins, NSAIDs, Fludarabine)
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29
Q

Secondary CAD etiologies

A
  1. Postinfectious (Mycoplasma pneumoniae/EBV)
  2. LY
  3. AI
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30
Q

PCH etiology

A

Primary
Viral (pediatric)
Syphilis

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31
Q

IgM target in CAD

A

I Antigen (mycoplasma)
i Antigen (EBV)- does not exist in adults

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32
Q

IgG target in PCH

A

P Antigen

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33
Q

Rituximab benefit in warm AIHA

A

Increases response rates
75% vs 30% in 1 year

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34
Q

Agents with activity as 2nd/3rd line in warm AIHA

A

MMF
Azathioprine
CY
CsA
Danazol
Bortezomib

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35
Q

Tapering doses in AIHA

A

Start tapering after 2-3 weeks
20 mg at 2 months
Discontinuation not before 6 months

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36
Q

Agents with activity in CAD

A

Bendamustine
Bortezomib
Ibrutunib
Sutimlimab

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37
Q

Drug induced TMA agents

A

Plavix
Mitomycin
Gemcitabine
Tacrolimus
Sirolimus
Quinine
Emicizumab
High doses of FEIBA

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38
Q

Tx of Shiga toxin associated TMA

A

Eculizumab seems promising

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39
Q

Agents causing direct damage to RBCs- resulting in hemolysis

A

Copper/Lead
Dapsone/Sedural/Ribavirin
Snake/Spider bites
Burns

40
Q

HbA2 levels diagnostic of b-thalasemia

A

> 3.5%

41
Q

TD beta thalassemia genotype

A

b0/b0
Severe HbE/b-thalassemia syndromes

42
Q

NTDT syndromes

A

b-thalassemia intermedia
Moderate HbE/b-thalassemia syndromes

43
Q

a-thalassemia silent carrier genotype

A

-a/aa

44
Q

a-thalassemia trait genotype

A

–/aa
-a/-a

45
Q

HbH genotype

A

–/-a
b4 tetramer

46
Q

TDT alpha thalassemia genotype

A

Severe types of HbH

47
Q

NTDT alpha thalassemia genotype

A

Mild-moderate types of HbH

48
Q

Hb electrophoresis in alpha thalassemia

A

Trait and silent carrier- normal
NTDT and TDT- 5-40% of HbH

49
Q

Chelation goal in TDT

A

Ferritin of <1000 ng/mL
LIC of <5
Cardiac T2* of >20 ms

50
Q

Deferoxamine

A

Tx of TDT thalassemia
SC or IV infusion
Continous infusion
SE: FTT, loss of hearing, retinopathy, yersinia infections, OP

51
Q

Deferiprone

A

Tx of TDT thalassemia
PO
SE: agranulocytosis!, GI, LFT, arthralgia, zinc deficiency

52
Q

Luspatercept for TDT

A

BELEIVE
Reduction in transfusion of 33%
Increased VTE

53
Q

SCD genotype

A

HbSS, HbSb0- severe (causing anemia)
HbSC, HbSb+-mild

54
Q

Sickle cell trait complications

A

Splenic infarction at high altitude
Hematuria
Renal papillary necrosis
Pyelonephritis during pregnancy

Medullary carcinoma of kidney
VTE

SHARP MV

pointy like the cells
dead

55
Q

Effect of a-thalassemia on SCD

A

Reduces hemolysis and risk of VTE

56
Q

Haplotype of S in SCD with worst prognosis

A

Central African Republic (CAR)

worsens when cold

57
Q

Differentiating HbSS and HbSB0

A

MCV

58
Q

Acute chest syndrome Sx

A

Similar to pneumonia

59
Q

Risk factors of developing acute chest syndrome

A

Young age, male
Low HbF
High WBC
High steady state HbS

60
Q

Leading cause of death in SCD

A

Acute chest syndrome

61
Q

Exchange transfusion indication in SCD with acute chest syndrome

A

Hypoxemia despite oxygen
Widespread infiltrates,
Rapid clinical deterioration.

62
Q

Preventive measures for acute chest syndrome in SCD

A

HU
Chronic RBC transfusions

63
Q

Silent cerebral infarcts in SCD %

A

30% children
50% adults

64
Q

Disease modifying Tx in SCD

A

HU
L-glutamine
Crizanlizumab (anti p-selectin)
Voxelotor (HbS polymerization inhibitor)

65
Q

Benefit of HU in SCD

A

Reduces pain episodes, acute chest syndrome, and
transfusions

66
Q

L-glutamine benefit in SCD

A

Reduces pain crisis in addition to HU

67
Q

HbE

A

Trait- Asymptomtic
Homozygotes- mild microcytic anemia

68
Q

HbC

A

HbCC or HbC beta-thalssemia have mild anemia with splenomegaly

69
Q

Gaucher disease clinical manifestations

A

Hepatosplenomegaly
Cytopenias
Bone deformation and pain

70
Q

Gaucher disease diagnosis

A

Decreased glucocerebrosidase activity
Genetic

71
Q

Gaucher disease Tx

A

Enzyme replacement therapy

72
Q

Incidence AIHA

A

2/100K

73
Q

Mortality AIHA

A

5%

74
Q

FP coombs causes

A

Allo Ab- 3 months from transfusion
Dara
IVIG
Sepsis- non specific Ab due to increased Ab in plasma

75
Q

Primary vs secondary AIHA %

A

50%-50%

76
Q

Which IgGs are clinically important for hemolysis

A

IgG1
IgG3

77
Q

Severe wAIHA necessitating rituximab

A

Hb <8
Transfusion
Evans
IgA

78
Q

Novel Tx in AIHA

A

Protesome inhibitors
CD38 i
Fosfamatinib
BTKi

79
Q

Nipocalimab

A

Anti FcRn
Prevention of fetal hemolysis
Novel Tx of wAIHA with combination with PEX

80
Q

Cold agglutination titer

A

1/64

81
Q

Monoclonal in CAD %

A

80%
IgMk

82
Q

Complement inhibition in CAD

A

Sutimlimab
C1 inhibitor
In acute hemolysis
Bridging Tx to rituximab

83
Q

Alpha globin chromosome

A

16

84
Q

Beta globin chromosome

A

11

85
Q

Hb goal in thalassemia

A

12

86
Q

Luspa SE

A

Bone pain
Extramedullary hematopoesis- consider imaging prior to Tx in thalassemia
May be teratogenic
Thrombosis
Fatigue
GI

87
Q

HbS goal of exchange transfusion in SCD

A

HbS> 40%

88
Q

Cold agglutinin titer for CAD

A

64

89
Q

1st line Tx of choice for CAD

A

BR X4
60% CR with long DoCR
Time to best response is long (6-7 months)

R alone is also an option

90
Q

Indications for BMB for mild anemia

A

Macrocytosis
Other cytopenias
FHx
Dysplasia on PB

91
Q

B12 deficiency definition

A

B12<200
or
B12 < 300 and MMA > ULN

92
Q

Target Ferritin in CKD pts

A

300-500

93
Q

HbSC / HbSβ+
vs
HbSS phenotype

A

Higher Hb with fewer complications
May be diagnosed in older age

94
Q

Tx of splenic squestraion in SCD

A

Transfusion

95
Q

Tx indication in CAD

A

Only if Sx
Cold avoidance is reccomended for all

96
Q

IV iron indications

A

CKD
CHF
Cancer
Poor abdorption
Urgent needs

97
Q

Common drugs causing drug induced AIHA

A

Cephalosporins, NSAIDs, PD1

Erez's HEM TEST notes 2024 (35 decks)

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