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Senior - Heme-Onc > Lecture 4 - Normocytic Anemia > Flashcards

Lecture 4 - Normocytic Anemia Flashcards

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1
Q

DDX for normocytic anemai?

A 
Ancemia of chronic disease
Acute blood loss
Multifactorial anemia
Renal-failure associated anemia
Aplastic anemia
Hemolysis (hemolytic anemia)
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2
Q

2nd MC anemia?

A

Anemia of chronic disease (ACD)

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3
Q

Lab description of ACD?

A

Normochromic
Normocytic
Hypoproliferative

May be microcytic (late sign)

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4
Q

With ACD what determines the severity and characteristics of the anemia?

A

The underlying disease

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5
Q

What is the key pathophysiologic principle of ACD?

A

Disturbance in iron homeostasis mediated by chronic inflammation

Hepcidin released from liver in presence if IL-6

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6
Q

Why does the inflammation process cause anemia?

A

Inflammatory process leads to decreased iron uptake from GI tract and retention of iron w/in the macrophages as ferritin

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7
Q

Inflammatory processes cause a 2 pronged attack, what is it?

A
  1. Decreased iron availability in the bone marrow leads to reduced RBC production-hypoproliferation
  2. Reduced RBC survival (increased destruction)
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8
Q

How sever is the anemia with ACD?

A

Usually its mild but is often associated with other anemic processes

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9
Q

Lab findings for ACD?

A 
Normocytic: MCV 80-100
Low iron
Low transferrin
Low TIBC
HIGH ferritin
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10
Q

TX for ACD?

A

Treat the disease
Blood transfusion
EPO if symptomatic and Hb <10g/dL

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11
Q

Iron supplementation for ACD?

A

Not generally recommended if no iron deficiency

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12
Q

Charts on

A

Slides 18 and 19, maybe give them a look

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13
Q

What type of anemia is acute blood loss?

A

Normocytic anemia

- youre blood is fine you just dont have enough of it

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14
Q

With a hemorrhage pt you cannot trust?

A

CBC, they are getting IV fluids and blood products as part of their therapy and it will throw off their stuff

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15
Q

What is multifactorial anemia?

A

You have multiple causes for your anemia

Often Iron and B12 or iron and acd etc

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16
Q

Who gets renal failure associated anemia?

A

Prolonged renal failure:
- GFR < 30mL/min

It is a normocytic anemia

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17
Q

Erythropoietin, what is it good for?

A

Absolutely nothing!

Probably inaccurate however we cant count on it for diagnosis of renal failure associated anemia as it looks normal until Hb < 10

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18
Q

What is the tx for renal failure associated anemia?

A

Chronic synthetic erythropoietin agents (ESAs)

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19
Q

Hypoproliferative anemia is a prominent feature in?

A

Hematologic diseases described as bone marrow failure states

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20
Q

What is aplastic anemia?

A

It is a condition Bone marrow failure caused by:

Abnormal expression of stem cells leading to hypoplastic bone marrow.

Pancytopenia ensues

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21
Q

Primary and secondary aplastic anemia?

A

Primary:
-MC IgG autoantibody directed against the stem cell

Secondary:

  • inherited (fanconi’s)
  • acquired (chemo, radiation et)
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22
Q

Bone marrow disorders that cause pancytopenia?

A
  • Aplastic anemia
  • Myelodysplasia
  • Acute leukemia
  • Myelofibrosis
  • Infiltrative disease
    • (lymphoma, myeloma, carcinoma)
  • Megalobastic anemia
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23
Q

Non-marrow disorders that cause pancytopenia?

A
  • hypersplenism

- SLE

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24
Q

Clinical features of aplastic anemia?

A
  • Anemia: Weakness, fatigue, pallor
  • neutropenia - infection
  • thrombocytopenia - bleeding
  • P/E (pallor, purpura, petechiae)
  • bimodal distribution (15 -25 yo then 60+ yo)
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25
Labs for aplastic anemia?
Severe anemia Pancytopenia (hallmark) Decreased reticulocytes Normal RBC
26
How is the diagnosis for aplastic anemia made?
CBC - pancytopenia | Bone marrow bx - hypocellular bone marrow
27
What is fanconi’s anemia?
Inherited form of aplastic anemia Highly variable phenotypic expression of autosomal recessive trait or x-linked disorder
28
Fanconi’s anemia is characterized by?
Pancytopenia | Congenital malformations
29
Major causes of aplastic anemias
Slide 29, give it a gander
30
Tx for aplastic anemia?
Varies by age 20 y/o: bone marrow transplant (curative) > 45y/o: immunosuppression, let body fix itself 20-45y/o: shooter preference
31
Prognosis for aplastic anemia?
<50y/o: 70% 5 yr survival >70y/o: 50% 5 yr survival Severe unresponsive aplastic anemia have 1 yr untreated mortality rate of 70%
32
What are hemolytic anemias?
Condition in which RBCs are destroyed and removed from circulation - result is fewer mature RBCs which causes anemia Broad range of etiologies
33
Classifications for hemolytic anemias?
Intrinsic: defect in RBC (hereditary) Extrinsic: external to RBC
34
Lab descriptions of hemolytic anemias?
Peripheral Blood Smear - spherocytes - round, lack concavity and central pallor - schistocytosis - fragmented/destroyed rbc - helmet cells - target cells - reticulocytis
35
Labs for hemolytic anemias?
``` Haptoglobin : L Indirect bilirubin : H Hemoglobinuria : H LDH : H Reticulocytes : L/Normal ```
36
I am an acquired clonal stem cell D/o, what am I? My MC mutation is PIG-A gene
Paroxysmal nocturnal hemoglobinuria (PNH)
37
What is a PIG-A gene?
Gene responsible for some stuff... CD55 and CD59... Result is RBC are susceptible to complement-mediated hemolysis
38
Clinical manifestation of PNH?
Episodic dark cola-colored urine (wake up pee) Prone to thrombosis (veins > arteries) Stem cell disorder
39
What conditions are associated with PNH?
It is a stem cell d/o so: Aplastic anemia Myelodysplasia Acute myeloid leukemia
40
Hallmarks (Labs) for PNH?
LDH - H Urine hemosiderin - + Haptoglogin - L iron def
41
Best screening test for PNH?
Flow cytometry | - def of CD55 and CD59 on RBCs
42
Key words for PNH diagnosis?
- Episodic hemoglobinuria - Hemolytic anemia - Thrombosis (hypercoagulable state) - Flow cytometry (screening)
43
Tx of Paroxysmal Nocturnal Hemoglobinuria
Referral ``` Eculizumab - reduce hemolysis Anticoagulants Iron Bone marrow transplant Prednisone ```
44
MC hemolytic anemia to to RBC membrane defect?
Hereditary spherocytosis
45
What is going on with hereditary spherocytosis?
an abnormailty in spectrein or actin Basically the RBC membrane doesnt flex like it should so they are rigid and dont pass through small fenestrations in spleen Leads to badness (i mean hemolysis)
46
What type of anemia does hereditary spherocytosis cause?
Chronic hemolytic anemia
47
Clinical findings for hereditary spherocytosis?
Autosomal dominant disorder (fam hx) Dx’d in childhood Chronic hemolysis causes - jaundice, - splenomegaly - pigmented gallstones
48
Labs for hereditary spherocytosis?
Microcytosis or normocytic and increase MCHC Reticulocytis Spherocytes Neg coomgs (antibody test) Osmotic fragility test - increased
49
What is the only condition that has increased MCHC?
Hereditary spherocytois so if you see elevated MCHC pick this one!!!
50
Hereditary spherocytosis is intrinsic or extrinsic?
Intrinsic (its the RBC membrane that is fucked)
51
Tx for hereditary spherocytosis?
Supportive care Folic acid 1mg/d Splenectomy (that’ll teach the spleen)
52
This d/o causes acute hemolytic anemia with hemoglobinuria when taking the anti-malaria drug primaquine
G6PD deficiency
53
MC enzymatic d/o of RBC?
G6PD X-linked enzyme defect
54
What are heinz bodies?
Oxidized Hgb denatures to form precipitants called heinz bodies These damage RBC membrane and the spleen takes them out Seen with G6PD
55
Pathophysiology of G6PD?
Oxidative stress - cellular damage occurs from radical oxygen species that are not fully neutralized by anti-oxidants G6PD folks cant handle the stress
56
Drugs that affect G6PD?
Antimalarials Sulfonamides Nitrofuratonin And some misc stuff
57
Tx for G6PD?
Avoid that shit
58
Who gets G6PD deficiency?
10-15% of AA males
59
Labs for G6PD?
Normal between episodes During episodes - reticulocytosis - indirect bilirubin - bite cells - heinz bodies
60
What is sickle cell anemia?
Autosomal recessive disorder Characterized by point mutation of beta globin chain gene Sickling
61
What causes hemolytic crisis for sickle cells pts?
Splenic sequestration of sickled cells
62
Epidemiology of Sickle Cell Anemia?
8% of AA are carriers, 1 in 400 has Sickle Cell Disease
63
Clinical findings of Sickle cell anemia?
Usually Show up in 1st year of life Chronic hemolytic anemia Pain (acute vaso-occlusion from clusters) Acute chest syndrome
64
What is acute chest syndrome?
Fevers Chest pain Hypoxemia Pulmonary infiltrates
65
Howel-jolly bodies
Hallmarks of hyposplenism due to auto-infarct and vaso occlusion (sickle cell) (Nuclear remnants w/in RBCs that are usually removed by spleen and target cells) Thats all cool, it looks like some shit let on the RBC when you look at a microscope
66
Lab findings for sickle cell
Chronic hemolytic anemia howell-jolly bodies Chronic reticulocytosis
67
How is sickle cell confirmed?
Hb electrophoresis
68
Tx for sickle cell?
Supportive care Hydroxyurea Transfusion Pneumococcal vacc
69
Proposed sickle cell treatments?
L-glutamine Rivipansel Crizanlizumab See slide 60-62 if you care, it wont be a question b/c they are still in trials
70
Hospital therapy for sickle cell crisis?
``` IV DVT prophylaxis Diphenhydramine NSAIDs Opioid therapy ```
71
Autoimmune hemolytic aneima (AIHA)
Acquired autoantibodies to RBC It is an extrinsic defect
72
MC AIHA?
“Warm” AIHA IgG antibodies react at normal body temps and destroy RBCs
73
Cold AIHA?
Aka cold agglutinin disease IgM antibodies react at reduced body temps
74
Clinical feature of autoimmune hemolytic anemia?
- Episodic associated w physiologic stress - Rapid onset anemia - fatigue, angina, dypsnea, palpitations - pain w eating/drinking cold stuff - spherocytosis + COOMBS test
75
Why does AIHA have positive Coombs?
B/c its autoimmune and thats what Coombs is fore
76
Warm AIHA tx?
Refer to hematology Immunosuppression Splenectomy Transfusions (complicated)
77
Cold AIHA tx?
Refer to hematology Supportive Avoid cold Rituximab
78
What is microangiopathic hemolytic anemias (MAHA)?
Group of d/o | Intravascular hemolysis due to RBC fragmentation (traumatic destruction)
79
Hallmark of MAHA?
Fragmented cells on PBS - schistocytosis - helmet cells
80
Pts w hemolytic anemia are more prone to?
Infections liek parvovirus and strep Infections may trigger hymolysis due to “unique mechanisms” i.e. magic
81
Malaria
JK he just had a title slide of this, no info
82
They say blood is thicker than water
So what? So is toothpaste

Senior - Heme-Onc (6 decks)

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