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LMCC > Heme > Flashcards

Heme Flashcards

1
Q

Haptoglobin

A

Protein that mops up free Hb allowing its clearance in the spleen
In hemolytic anemia, haptoglobin decreases b/c it is all consumed

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2
Q

Thalassemia genetic inheritance

A

Autosomal recessive

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3
Q

Hemochromatosis clinical features

A 
ABCDH
Arthralgia
Bronze skin 
Cardiomyopathy, liver cirrhosis 
Diabetes 
Hypogonadism (anterior pituitary damage)
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4
Q

Thalassemia

A

Defects in production of alpha (SEA and Africa) or beta (Mediterranean) chains of Hb resulting in ineffective erythropoiesis and hemolysis in spleen or BM

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5
Q

beta-Thalassemia peripheral blood smear

A

Microcytic
Teardrop
Target
Hypochromatic

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6
Q

Beta-thal treatment

A

Lifelong regular monthly transfusions to suppress endogenous erythropoiesis
Iron chelation (deferoxamine) to prevent iron overload in organs and formation of free radicals
Folic acid supplementation
Consider allogenieic bone marrow transplant or cord blood transplant as cure
Splenectomy

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7
Q

Alpha-thal

A

4 alpha globin genes
All 4 = Hb Barts, typically hydrops fetalis, incompatible with life
3/4 = HbH, presents in adulthood, decreased MCV, decreased Hb, splenomeg
1 or 2/4 = no tx required

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8
Q

Alpha-thal peripheral blood smear

A

Screen for HbH inclusion bodies with supravital stai

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9
Q

Sickle cell genetic inheritance

A

Autosomal recessive due to mutant beta globin gene (Glu –> Val substitution)

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10
Q

Sickle cell trait

A

Pt asymptomatic except during extreme hypoxia or infection

Increased risk of renal medullary carcinoma

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11
Q

Functional asplenism, increased susceptibility to encapsulated organisms

A

S. pneumoniae
N. meningitidis
H. influenza
Salmonella

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12
Q

Sickle cell treatment

A

Folic acid
Hydroxyurea to enhance production of HbF
Tx of vaso-occlusive crisis (O2, hydration, correct acidosis, analgesics)
Transfuse if Hb <50-60h/L

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13
Q

Sickle cell prevention

A 
Vaccination (pneumococcus, meningococcus, H. influenza) 
Prophylactic penicillin (3mo-5yo)
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14
Q

Warm autoimmune hemolytic anemia

A

IgG
Idiopathic
Secondary to lymphoproliferative d/o (ie. CLL)
Secondary to AI disease (ie. SLE)
Drug-induced
Spherocytes on blood film
Tx: corticosteroids, immunosuppression, splenectomy, folic acid, rituximab (2nd line to steroids)

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15
Q

Cold autoimmune hemolytic anemia

A

IgM
Idiopathic
Secondary to infection (ie. EBV)
Secondary to lymphoproliferative d/o (ie. CLL)
Agglutination of blood film
Tx: warm patient, rituximab regimens (1st line), plasma exchange (2nd line for high IgM levels), folic acid

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16
Q

Most common type of hereditary hemolytic anemia

A

Hereditary spherocytosis

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17
Q

G6PD

A

Defiency in glucose-6-phosphate dehydrogenase –> RBC sensitivity due to oxidative stress
X-linked recessive
Episodic hemolysis precipitated by oxidative stress, drugs, infection, fava beans
May present in neonates with prolonged jaundice

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18
Q

G6PD blood smear

A

Heinz bodies

Bite cells as they pass through spleen

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19
Q

G6PD tx

A

Folic acid
Stop offending drugs and avoid triggers
Transfusion in severe cases

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20
Q

Thrombocytopenia

A

Plt < 150 000/uL

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21
Q

Plt < 50 000

A

increased risk of procedural and surgical bleeding (<100,000 for neurosurgeries)

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22
Q

Plt < 20 000

A

increased risk of severe bleeding with fever and/or coagulopathy

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23
Q

Plt < 10 000

A

Increased risk of spontaneous bleeding (ie. ICH)

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24
Q

Heparin-associated thrombocytopenia

A

Plt >100 x 10^6, direct heparin mediated plt aggregation (non-immune)
Self limited
Continue with heparin therapy

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25
Q

Heparin-induced thrombocytopenia (HIT)

A

immune-mediated reaction following tx with heparin (typically within 5-10d) leading to thrombocytopenia and subsequent coag activation
Stop heparin, can never have again

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26
Q

4T score for heparin

A

Thrombocytopenia
Timing of plt count fall
Thrombosis or other sequelae
Other causes for thrombocytopenia

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27
Q

Thrombotic thrombocytopenic purpura pathophysiology

A

ADAMTS-13 protease activity decreased –> vWF not cleaved –> large multimer on endothelial surface –> plt adhere and aggregate –> diffuse small vessel clotting = MAHA

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28
Q

Pentad of TTP

A
  1. Thrombocytopenia*
  2. MAHA*
  3. Renal failure
  4. Fever
  5. Mental status change
  • = only ones required for dx
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29
Q

TTP treatment

A

PLEX (plasma exchange) +/- steroids
Improvement in neurological signs is initial indicator of response
Avoid platelet transfusion unless life-threatening bleed (“peeing into the ocean”)
MEDICAL EMERGENCY

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30
Q

HUS

A 
Predominantly in children/elderly 
90% secondary to Shiga toxin 
Similar to TTP (severe thrombocytopenia, MAHA, AKI, blood diarrhea) 
3 criteria: 
- Hemolytic anemia 
- AKI 
- Thrombocytopenia
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31
Q

HUS tx

A

Supportive (fluids, RBC transfusion)

Some evidence for PLEX

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32
Q

Most common inherited bleeding disorder

A

von Willebrand disease

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33
Q

vWD inheritance

A

Autosomal dominent

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34
Q

vWF function

A
  1. Platelet aggregation

2. Chaperone for Factor VIII

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35
Q

vWD treatment

A

Desmopressin (DDAVP) - causes release of vWF and Factor VIII from endothelial cells
TXA (stabilize clot formation)

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36
Q

Hemophilia genetic inheritance

A

X-linked recessive

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37
Q

Hemophilia A

A

Factor VIII deficiency

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38
Q

Hemophilia A tx

A

Desmopressin in mild form
Factor VIII concentrate for : prophylaxis or on demand
TXA

39
Q

Hemophilia B

A

Factor IX deficiency

40
Q

Hemophilia B tx

A

Factor IX concentrate

TXA

41
Q

Vitamin K dependent factors

A

1972

X, IX, VII, II, proteins C&S

42
Q

Vit K deficiency

A

INR/PT is elevated out of proportion to elevation of the aPTT

43
Q

Vit K deficiency tx

A

Vitamin K PO if no active bleeding
If bleeding –> Vit K 10mg IV
If life threatening bleeding, give prothrombin complex concentrate (PCC) or FP if C/I

44
Q

Factor deficiency in liver disease

A

Deficient synthesis of all factors except VIII

Order levels of Factor V, VII, VIII

45
Q

Etiologies of DIC

A 
OMITS
Obstetric complications
Malignancy
Infection
Trauma
Shock
46
Q

Coagulopathies a/w increased INR only

A 
Warfarin
Vit K deficiency 
Factor VII deficiency 
Factor VII inhibitors 
Liver disease
47
Q

Coagulopathies a/w increased PTT only

A 
Hemophilia A and B 
Heparin 
Antiphospholipid Ab 
Intrinsic factor inhibitors 
Factor XI and XII deficiency
48
Q

Extrinsic factor

A

Factor VII

49
Q

Intrinsic factor

A

Factors XII, XI, IX, VI

50
Q

Coagulopathies a/w increased INR and PTT

A 
Prothrombin deficiency 
Severe fibrinogen deficiency
Factor V and X deficiency 
Severe liver disease
Severe vitamin K deficiency
51
Q

Common pathway factors

A

Factor V
Factor X
Factor II (Prothrombin)
Factor I (Fibrinogen)

52
Q

Tear drop cells on blood smear

A

Myelofibrosis
Thal major
Megaloblastic anemia

53
Q

Poiilocytosis on blood smear

A

Fe def anemia

Myelofibrosis

54
Q

Hypersegmented neutrophils on blood smear

A

Megaloblastic anemia

55
Q

Coagulation studies in vWD

A

PT normal
PTT increased
Bleeding time increased

56
Q

PT measures

A

Extrinsic and common pathways
Factor I (fibrinogen)
Factor II (prothrombin)
Factors V, VII, X

57
Q

PTT measures

A

Intrinsic and common pathways

XII, XI, IX,VIII,X,V,II and I

58
Q

Chronic lymphocytic leukemia (CLL)

A

Proliferation of mature Bcells in pts who are generally >65yo
B-cells grow out of control and accumulate in bone marrow and blood –> eventually crowd out healthy blood cells

59
Q

CLL complications

A

Lymphadenopathy
Hepatosplenomegaly
Anemia
Infections

60
Q

CLL tx

A

Early stages: not treated

Late stages: Chemo and monoclonal ab (ritxuimab: monoclonal AB against CD20 cells)

61
Q

Hodgkin Lymphoma cells

A

Reed-sternberg

62
Q

Most common inhibitor in clinical practice

A

Lupus anticoagulant

63
Q

Most common factor-specific inhibitor

A

Factor VIII

64
Q

ITP treatment

A

Steroids
IVIG
Splenectomy
Rituximab

65
Q

3 common forms of MAHA

A

TTP
DIC
HUS

66
Q

TTP lab values

A 
Plts down 
Fibrinogen normal 
D-Dimer normal 
Schistocytes + 
PTT/PT normal
67
Q

DIC lab values

A

Plts down
Fibrinogen down
D-dimer higher
PTT/PT increased

68
Q

HIT dx

A

ELISA assay to detect AB
If +ve, serotonin assay
Serotonin = marker of plt activation

69
Q

Auer rod

A

Pathognomonic for AML

blast of myeloid lineage

70
Q

Acute lymphoid leukemia

A

More common in children
Most common malignancy in children
85% cure rates with intensive chemo
Harder to tx in adults

71
Q

Acute myeloid leukemia

A 
All malignant cells are blasts 
Myeloid malignancy arising in BM 
Results in pancytopenia 
More common in adults
Auer rods
72
Q

Clinical findings of multiple myeloma

A 
CRABi 
hyperCalcemia 
Renal failure 
Anemia 
Bone pain and pathological fractures 
Infections
73
Q

CML specific mutation

A

Philadelphia chromosome

74
Q

CML vs MDS

A

CML has HIGH blood counts due to malignancy of mature myeloid cells (–> splenomeg)
MDS has LOW blood counts due to abnormal maturation of myeloid cells (–> apoptosis)

75
Q

CLL

A

Older adults
Very slow progressive malignancy
Malignancy of mature lymphoid cells which often spreads to lymph nodes (BM and LN affected)
HIGH lymphocyte counts but cells look normal on smear
Need flow cytometry to prove cells are malignant

76
Q

Multiple myeloma

A

Malignancy of plasma cells (B lymphocytes that secrete antibodies)
Malignant plasma cells STAY in BM and wipe out healthy BM, send out clonal antibody that causes renal failure
Can detect clonal antibody via SPEP (single band in MM vs smear in healthy adult)

77
Q

Lab that marks the most advanced stage of Fe def and indicates imminent Fe deficiency anemia

A

High free erythrocyte protoporphyrin (heme synthesis requires presence of protoporphyrin and Fe; when there is not enough Fe, FEP levels rise and Hb levels fall)

78
Q

Stages of Fe-def anemia

A 
Low ferritin 
Low serum iron
Rise in serum transferrin
Increased TIBC 
Decreased TSat 
Free ertyhrocyte protorphyrin levels rise
79
Q

Massive PE treatment

A

Signalled by hemodynamic instability (ie. hypotension)

  1. stabilize pt (ie. NE to bring up BP)
  2. TPA
80
Q

Warfarin reversal

A

PCC/Octaplex (contains X, IX, VII, II)

Vitamin K

81
Q

Free erythrocyte porphyrin

A

Use when dx of beta thal minor is unclear
Normal in pts with beta thal trait
Elevated in pts with Fe deficiency or lead poisoning

82
Q

When to manage pt inpatient for DVT

A

Massive DVT
Symptomatic PE
High risk bleeding with anticoagulant therapy
Comorbid conditions or other factors that warrant in-hospital care

83
Q

Preferred tx for outpatient DVT management

A

LMWH
Warfarin
Compression stockings

84
Q

INR above which you should stop warfarin AND consider vitamin K

A

6

85
Q

Gallstones in a child should prompt you to think about…

A

Hereditary spherocytosis

Spectrin and/or ankyrin deficiency –> reduced flexibility of cell –> more prone to damage

86
Q

Test to confirm dx hereditary spherocytosis

A

Osmotic fragility test
+ smear: spherocytes without central pallor, howell jolly bodies
negative coombs test
++ family hx

87
Q

Most common hereditary thrombophilia

A

Factor V Leiden
Caused by mutation to Factor V which causes activated resistance to protein C –> increased Factor V availability to increase generation of thrombin –> increased coagulability
Dx: Activated Protein C resistance assay and genetic testing of factor V gene /mRNA

88
Q

Protein C function

A

Inactivates Factor V and VIII

89
Q

Protein S function

A

Cofactor to Protein C

90
Q

Length of tx for DVT

A

3mo for reversible cause
6mo for unknown cause
Indefinitely for recurrent DVT

91
Q

AIHA

A

Normocytic anemia
Evidence of hemolysis (LDH, jaundice, high bili, low haptoglobin)
Splenomegaly
Reticulocytosis
Smear: Spherocytes, reticulocytes, elliptocytes

92
Q

Acute hemolytic transfusion reaction

A

ABO incompatibility due to clerical error
Fever, chills, hemoglobinuria, FLANK PAIN, discomfort at infusion site –> –> renal failure and DIC
Dx: + Coombs test, hemoglobinuria, repeat type and cross match showing mismatch
Tx: Stop transfusion, supportive care

93
Q

Calcium and albumin correction

A

Every albumin drop by 10, calcium increases by 0.2

94
Q

vWd diagnosis

A

Ristocetin cofactor activity

ristocetin induces coagulation when vWF present, so if added to blood without vWF –> no coagulation

LMCC (22 decks)

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