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Heme boards > hemolytic anemias > Flashcards

hemolytic anemias Flashcards

1
Q

target cell differential

A

Liver disease →
Splenectomy →
Thalassemia →
Other hemoglobinopathies →
Iron deficiency →

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2
Q

Differential for DAT positive hemolytic anemia

A

1) False positive
2) AIHA
3) Drug induced auto-immune hemolytic anemia → (antibiotics (cephalosporins, penicillins), CPIs, platinum-based chemo, NSAIDs) – table 1, acute (often within hours).
4) *Delayed hemolytic transfusion reaction → 1-2 weeks after transfusion of RBCs, although the interval can range from 24 hours to 28 days.
5) *Cold agglutinin disease

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3
Q

Therapies that can cause false positive result on DAT

A

IVIG
RHd
ATG
*daratumumab

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4
Q

Peripheral smear findings w/ AIHA

A

spherocytes
reticulocytosis
*review peripheral smear image online

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5
Q

Secondary causes of AIHA

A

Medications (penicillins, methyldopa, cephalosporins) →
CVID →
Lymphoproliferative disease (CLL) →
Connective tissue diseases (SLE)→
Viral infections (EBV, HEV, CMV, HIV) →
Autoimmune disorders (causal?) →
Allogeneic blood transfusion →
Chronic inflammatory disease (IBD) –>

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6
Q

Infectious causes of AIHA

A

EBV, Hep C, CMV, HIV, *brown recluse spiders, babesia

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7
Q

Initial management of AIHA

A

pred 1 mg/kg

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8
Q

Management of refractory AIHA

A
  • rituxan
  • Immunosuppressive agents, such as cytoxan, mycophenolate, azathioprine, or cyclosporine
  • splenectomy as last resort
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9
Q

What is a high titer in cold agluttinin disease?

A

(1:40) or >99th percentile

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10
Q

Infectious causes of cold agglutinin disease

A

1) mycoplasma pneumonia (atypical pneumonia)
2) EBV/mono

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11
Q

Secondary causes of cold agglutinin disease

A

1) Low grade lymphoproliferative disorders (B cell NHL, MGUS, LPL, SLL) ( clone producing monoclonal IgM antibody) (most commonly) → Etiology in most older people unless active infeciton
2) ***Infection (mycoplasma pneumonia (atypical pneumonia) + EBV/mono) →
3) Autoimmune disorder (SLE, RA)

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12
Q

Initial management of cold agglutinin disease

A
  • Cold avoidance (minimize cold rooms, cold water/liquids)
  • IF symptomatic critical anemia, see temporizing measures below
  • Initial period of observation with strict return precautions
  • Indications for treatment (Not all need treatment)
  • Symptomatic anemia, fatigue, cold-induced ischemic symptoms interfering with QOL
  • ***Not all hemolysis requires treatment (if stable and compensated can continue observation or see how they do after tapering steroids)
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13
Q

Definitive therapy of cold agglutinin disease

A

BR vs. single agent rituxan depending on burden of disease + toxicity profile
*steroids ineffective

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14
Q

Complement inhibitor approved for cold agglutinin disease 2) target

A

1) Sutimlimab 2) C1

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15
Q

PCH pathophys

A

polyclonal IgG developing following viral illness that leads to complement mediated hemolysis

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16
Q

PCH smear

A
  • spherocytes
  • *erythrophagocytosis
    *see picture online
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17
Q

DAT profile in paroxysmal cold hemoglobinuria

A

Positive for C3d

18
Q

Test specific for PCH

A

donald landsteiner antibody

19
Q

Differentiating cold agglutinin disease vs. PCH based on DAT

A

in PCH, C3 remains positive but IgG becomes negative at higher temperatures (in cold agglutinin IgG is always negative)

20
Q

PCH management

A

supportive care (self limited)

21
Q

PNH DAT

A

negative

22
Q

DAT negative hemolytic anemia differential

A

1) TMA’s → (TTP →
HUS + atypical HUS)
2) Drug-induced TMA → (table 5)
3) ***infections (clostridium perfringens, babesiosis, bartonella, leishmania) →
4) Platelet transfusions → Not ABO matched + low grade hemolysis
5) Liver disease →
6) Kidney disease →
7) Spherocytosis →
8) Thalassemia →
9) Sickle cell disease →
10) Delayed hemolytic transfusion reaction →
11) G6PD →
12) B12 deficiency →
13) hemodialysis →
14) hypersplenism
15) Paroxysmal cold hemoglobinuria

23
Q

Eculizumab SE’s

A
  • headache (improves after 1-2 doses)
  • back pain
  • nasopharyngitis
  • neisseria meningitis
24
Q

Pegcetacoplan mechanism

A

C3 monoclonal antibody
*inhibits both intra and extravascular hemolysis

25
Q

Newer drug approved for PNH

A

Pegcetacoplan

26
Q

spherocytes differential

A

HS → ALL RBCS are spherocytes
AIHA → ALL RBCs are spherocytes
All hemolytic anemias to some degree →
Warm autoimmune hemolytic anemia
Cold autoimmune hemolytic anemia/paroxysmal cold hemoglobinuria
Acute and delayed hemolytic transfusion reactions
ABO hemolytic diseases of newborn/Rh hemolytic disease of newborn

27
Q

HS confirmatory testing for diagnosis

A

Eosin-5-maleimide (EMA) binding, osmotic fragility, or osmotic gradient ektacytometry (OGE)

28
Q

dacrocytes significance

A

infiltrative processes

29
Q

DAT for AIHA

A

Anti-IgG and/or anti-C3

30
Q

First line for AIHA

A

steroids

31
Q

What can exacerbate AIHA?

A

Pregnancy

32
Q

What blood group is cold agglutinin directed at?

A

i antigen (capitalized though)

33
Q

Latest drug approved for CAD

A
  • sutimlimab
34
Q

Antibody and target in paroxysmal cold hemoglobinuria

A
  • complement fixing IgG directed against P antigen
35
Q

DAT during hemolytic episode in paroxysmal cold hemoglobinuria

A

Positive for C3d
Negative for IgG
(hemolysis is occuring once patient is warmed, binding occurs when patient is cold)

36
Q

PNH pathophys

A
  • complement driven hemolytic anemia resulting from clonal expansion of stem cells harboring a somatic PIG-A mutation
37
Q

PNH sequelae

A
  • VTE
  • bone marrow failure
  • pHTN from nitric oxide scavenging by free hemoglobin
  • smooth muscle complications: abdominal pain, erectile dysfunction, pHTN, diffficulty swallowing
38
Q

primary lab techniques for PNH diagnosis

A

1) flow for CD55, CD59
2) *FLAER (fluorescent labeled aerolysin)

39
Q

indications for starting treatment in PNH

A

1) essentially any symptoms affecting QOL
2) pregnancy

40
Q

Recent approval sfor PNH + benefit

A
  • these drugs target higher up in complement cascade
    pegcetacoplan (targets complement inhibition upstream so can reduce both intravascular and extravascular hemolysis so decreases breakthrough hemolysis)
    *iptacopan and Danicopan target alternative pathway
41
Q

Second line therapy for PNH

A

pegcetacoplan

42
Q
A

Heme boards (59 decks)

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