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Heme Flashcards

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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
Heparin-induced thrombocytopenia (HIT)
immune-mediated reaction following tx with heparin (typically within 5-10d) leading to thrombocytopenia and subsequent coag activation Stop heparin, can never have again
26
4T score for heparin
Thrombocytopenia Timing of plt count fall Thrombosis or other sequelae Other causes for thrombocytopenia
27
Thrombotic thrombocytopenic purpura pathophysiology
ADAMTS-13 protease activity decreased --> vWF not cleaved --> large multimer on endothelial surface --> plt adhere and aggregate --> diffuse small vessel clotting = MAHA
28
Pentad of TTP
1. Thrombocytopenia* 2. MAHA* 3. Renal failure 4. Fever 5. Mental status change * = only ones required for dx
29
TTP treatment
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
30
HUS
``` Predominantly in children/elderly 90% secondary to Shiga toxin Similar to TTP (severe thrombocytopenia, MAHA, AKI, blood diarrhea) 3 criteria: - Hemolytic anemia - AKI - Thrombocytopenia ```
31
HUS tx
Supportive (fluids, RBC transfusion) | Some evidence for PLEX
32
Most common inherited bleeding disorder
von Willebrand disease
33
vWD inheritance
Autosomal dominent
34
vWF function
1. Platelet aggregation | 2. Chaperone for Factor VIII
35
vWD treatment
Desmopressin (DDAVP) - causes release of vWF and Factor VIII from endothelial cells TXA (stabilize clot formation)
36
Hemophilia genetic inheritance
X-linked recessive
37
Hemophilia A
Factor VIII deficiency
38
Hemophilia A tx
Desmopressin in mild form Factor VIII concentrate for : prophylaxis or on demand TXA
39
Hemophilia B
Factor IX deficiency
40
Hemophilia B tx
Factor IX concentrate | TXA
41
Vitamin K dependent factors
1972 | X, IX, VII, II, proteins C&S
42
Vit K deficiency
INR/PT is elevated out of proportion to elevation of the aPTT
43
Vit K deficiency tx
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
Factor deficiency in liver disease
Deficient synthesis of all factors except VIII | Order levels of Factor V, VII, VIII
45
Etiologies of DIC
``` OMITS Obstetric complications Malignancy Infection Trauma Shock ```
46
Coagulopathies a/w increased INR only
``` Warfarin Vit K deficiency Factor VII deficiency Factor VII inhibitors Liver disease ```
47
Coagulopathies a/w increased PTT only
``` Hemophilia A and B Heparin Antiphospholipid Ab Intrinsic factor inhibitors Factor XI and XII deficiency ```
48
Extrinsic factor
Factor VII
49
Intrinsic factor
Factors XII, XI, IX, VI
50
Coagulopathies a/w increased INR and PTT
``` Prothrombin deficiency Severe fibrinogen deficiency Factor V and X deficiency Severe liver disease Severe vitamin K deficiency ```
51
Common pathway factors
Factor V Factor X Factor II (Prothrombin) Factor I (Fibrinogen)
52
Tear drop cells on blood smear
Myelofibrosis Thal major Megaloblastic anemia
53
Poiilocytosis on blood smear
Fe def anemia | Myelofibrosis
54
Hypersegmented neutrophils on blood smear
Megaloblastic anemia
55
Coagulation studies in vWD
PT normal PTT increased Bleeding time increased
56
PT measures
Extrinsic and common pathways Factor I (fibrinogen) Factor II (prothrombin) Factors V, VII, X
57
PTT measures
Intrinsic and common pathways | XII, XI, IX,VIII,X,V,II and I
58
Chronic lymphocytic leukemia (CLL)
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
CLL complications
Lymphadenopathy Hepatosplenomegaly Anemia Infections
60
CLL tx
Early stages: not treated | Late stages: Chemo and monoclonal ab (ritxuimab: monoclonal AB against CD20 cells)
61
Hodgkin Lymphoma cells
Reed-sternberg
62
Most common inhibitor in clinical practice
Lupus anticoagulant
63
Most common factor-specific inhibitor
Factor VIII
64
ITP treatment
Steroids IVIG Splenectomy Rituximab
65
3 common forms of MAHA
TTP DIC HUS
66
TTP lab values
``` Plts down Fibrinogen normal D-Dimer normal Schistocytes + PTT/PT normal ```
67
DIC lab values
Plts down Fibrinogen down D-dimer higher PTT/PT increased
68
HIT dx
ELISA assay to detect AB If +ve, serotonin assay Serotonin = marker of plt activation
69
Auer rod
Pathognomonic for AML | blast of myeloid lineage
70
Acute lymphoid leukemia
More common in children Most common malignancy in children 85% cure rates with intensive chemo Harder to tx in adults
71
Acute myeloid leukemia
``` All malignant cells are blasts Myeloid malignancy arising in BM Results in pancytopenia More common in adults Auer rods ```
72
Clinical findings of multiple myeloma
``` CRABi hyperCalcemia Renal failure Anemia Bone pain and pathological fractures Infections ```
73
CML specific mutation
Philadelphia chromosome
74
CML vs MDS
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
CLL
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
Multiple myeloma
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
Lab that marks the most advanced stage of Fe def and indicates imminent Fe deficiency anemia
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
Stages of Fe-def anemia
``` Low ferritin Low serum iron Rise in serum transferrin Increased TIBC Decreased TSat Free ertyhrocyte protorphyrin levels rise ```
79
Massive PE treatment
Signalled by hemodynamic instability (ie. hypotension) 1. stabilize pt (ie. NE to bring up BP) 2. TPA
80
Warfarin reversal
PCC/Octaplex (contains X, IX, VII, II) | Vitamin K
81
Free erythrocyte porphyrin
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
When to manage pt inpatient for DVT
Massive DVT Symptomatic PE High risk bleeding with anticoagulant therapy Comorbid conditions or other factors that warrant in-hospital care
83
Preferred tx for outpatient DVT management
LMWH Warfarin Compression stockings
84
INR above which you should stop warfarin AND consider vitamin K
6
85
Gallstones in a child should prompt you to think about...
Hereditary spherocytosis | Spectrin and/or ankyrin deficiency --> reduced flexibility of cell --> more prone to damage
86
Test to confirm dx hereditary spherocytosis
Osmotic fragility test + smear: spherocytes without central pallor, howell jolly bodies negative coombs test ++ family hx
87
Most common hereditary thrombophilia
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
Protein C function
Inactivates Factor V and VIII
89
Protein S function
Cofactor to Protein C
90
Length of tx for DVT
3mo for reversible cause 6mo for unknown cause Indefinitely for recurrent DVT
91
AIHA
Normocytic anemia Evidence of hemolysis (LDH, jaundice, high bili, low haptoglobin) Splenomegaly Reticulocytosis Smear: Spherocytes, reticulocytes, elliptocytes
92
Acute hemolytic transfusion reaction
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
Calcium and albumin correction
Every albumin drop by 10, calcium increases by 0.2
94
vWd diagnosis
Ristocetin cofactor activity | ristocetin induces coagulation when vWF present, so if added to blood without vWF --> no coagulation

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